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COLLEGE  OF  PHYSICIANS 
AND   SURGEONS 


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PLATE  I 


The  Various  Neurones  Constituting   the  Nervous  System  and  their  Relations. 

The  association  neurones  are  shown  in  red.  A  sensory  impression  from  the  skin  reaches  the 
spinal  cord,  where  it  may  excite  the  motor  neurone  ;  or  the  Clarke  cell,  and  through  it  the  cerebellum; 
or  the  medulla,  optic  thalamus  and  cerebral  cortex,  causing  automatic  responses  and  conscious  per- 
ceptions. A  sensory  impression  from  the  semicircular  canals  may  reach  the  cerebellum,  causing  auto- 
matic acts  of  equilibrium.  Cerebellar  impulses  may  be  sent  to  the  motor  neurone  by  way  of  the  neu- 
rones of  Deiters  in  the  medulla,  producing  acts  of  balancing,  or  to  the  cerebrum  by  way  of  the  optic 
thalamus,  causing  perceptions  of  position.  Cerebral  impulses  may  be  sent  directly  to  the  motor  neu- 
rone or  indirectly  through  the  corpus  striatum,  producing  voluntary  or  automatic  acts.  The  motor 
neurone  sends  its  nerve  to  the  muscle. 


ORGANIC  AND  FUNCTIONAL 


NERVOUS  DISEASES 


A  TEXT-BOOK  OF  NEUROLOGY 


BY 

M.  ALLEN   STARR,  M.D.,  Ph.D.,  LL.D.,  Sc.D. 

PROFESSOR    OP   NEUROLOGY,  COLLEGE   OF   PHYSICIANS   AND    SURGEONS,    THE    MEDICAL    DEPARTMENT    OP 

COLUMBIA    UNIVERSITY   IN   THE   CITY   OF    NEW   YORK;    CONSULTING    NEUROLOGIST    TO    THE 

PRESBYTERIAN   HOSPITAL   AND    TO    ST.    MARY'S   FREE    HOSPITAL   FOR    CHILDREN 

EX-PRESIDENT  OF  THE  AMERICAN   NEUROLOGICAL  ASSOCIATION   AND  OF  THE  NEW  YORK  NEURO- 
LOGICAL SOCIETY  ;    CORRESPONDING  MEMBER  OP  THE  SOCI^T^  DE  NEUROLOGIE,  AND  OF 
THE  SOCIETE  DE  PSYCHIATRIE  DE  PARIS  ;   OF  THE  NEUROLOGICAL  SECTION  OF  THE 
EOYAL  SOCIETY  OF  MEDICINE,  LONDON,  AND  OF   THE  GESELLSCHAFT 
DEUTSCHER  NKRVENARZTE 
AUTHOR  OF  "FAMILIAR  FORMS  OF  NERVOUS  DISEASE,"   "BRAIN  SURGERY," 
AND  "ATLAS  OF  NERVE  CELLS" 


FOURTH  EDITION,    THOROUGHLY  REVISED 


ILLUSTEATED  WITH  323  ENGEAVINGS  IN  THE  TEXT  AND 
30  PLATES  IN  COLOES  AND  MONOCHEOME 


LEA   &    FEBIGER 

NEW   YOEK   AND   PHILADELPHIA 

1913 


Copyright,  1913 
By  Lea  &  Febiger 


Press  of 

The  New  era  Printing  Company 

Lancaster.  Pa. 


PEEFACE  TO  THE  FOUETH  EDITION. 


The  demand  for  another  edition  of  this  book  has  given  me  an  op- 
portunity for  a  careful  revision  of  its  contents,  for  many  additions,  and 
also  for  an  arrangement  which  should  make  it  of  greater  value  to  the 
student  and  practitioner. 

The  general  aspects  of  neurology,  the  method  of  examination  of  a 
patient,  and  the  principles  of  diagnosis  have  been  brought  together  in 
the  first  part.  The  various  symptoms  presented  by  nervous  affections 
are  there  described  and  analyzed ;  the  practical  anatomy  and  physi- 
ology of  the  nervous  system,  necessary  to  their  understanding,  are  con- 
cisely set  forth,  and  the  attempt  is  made  to  trace  each  symptom  to  its 
underlying  disturbance  of  function  in  a  definite  nerve  centre  or  nerve 
tract.  In  this  way  the  student  is  enabled  from  the  study  of  symptoms 
to  reach  a  conclusion  as  to  the  nature  and  location  of  the  lesion  pro- 
ducing it,  and  thus  to  make  a  diagnosis  of  the  disease  present. 

In  part  second  the  organic  diseases  are  taken  up.  Such  additions 
as  have  been  made  to  our  knowledge  in  the  past  three  years  have  been 
incorporated  in  this  part,  and  several  chapters,  especially  those  upon 
poliomyelitis,  and  syphilis  of  the  nervous  system,  have  been  rewritten. 
Much  new  material  has  been  added  to  the  chapter  on  tumors  of  the 
brain ;  and  pellagra  has  been  discussed. 

In  part  third  functional  diseases  are  fully  presented,  the  space  allotted 
to  this  department  having  been  more  than  doubled.  Fuller  discussion 
is  given  to  spasmodic  neuroses,  especially  to  the  various  forms  of  tic 
and  myoclonus.  Some  functional  paralyses,  myatonia  congenita,  myas- 
thenia gravis  and  family  periodic  paralysis  have  been  presented.  Psy- 
chasthenia  is  considered  apart  from  neurasthenia,  and  the  subjects  of 
the  conscious  and  subconscious  mental  activities,  of  suggestion  and  of 
psycho-analysis  and  of  hypnosis  are  fully  discussed.  In  the  chapter 
on  hysteria  the  various  theories  of  the  disease  are  put  before  the 
reader,  the  hypotheses  of  Babinski,  Janet  and  Freud  being  contrasted. 
Chapters  have  been  added  on  Headaches,  and  on  Disorders  of  Sleep. 

In  part  fourth  the  diseases  of  the  sympathetic  nervous  system  are 
discussed,  our  present  knowledge  of  vaso-motor  affections,  their  physi- 
ology and  pathology  being  summarized,  and  chapters  added  on  sym- 
metrical gangrene  and  angio-neurotic  oedema.  Trophic  symptoms 
occurring  in  nervous  disorders  are  also  presented. 

While  the  extensive  literature  of  neurology  has  been  carefully  sifted, 
and  references  have  been  given  to  important  articles  upon  the  various 
subjects  considered,  it  has  been  my  endeavor  to  utilize  my  personal 
observation  and  experience  in  the  presentation  of  each  sul)jcct ;  for  the 
mass  of  pathological  and  clinical  material  that  has  accunuilatcd  in  my 


iv  PREFACE. 

possession  in  the  past  thirty  years  in  the  practice  of  this  specialty  has 
repaid  analysis  and  has  led  me  to  many  definite  conclusions.  Due 
regard  has  been  given  to  theories  of  disease,  but  attention  has  been 
especially  directed  throughout  the  book  to  etiology,  diiferential  diag- 
nosis, and  treatment. 

Nervous  diseases  lie  on  the  borderland  between  medicine  and  sur- 
gery, and  in  the  consideration  of  treatment,  the -surgical  measures  for 
the  cure  of  many  of  them  are  fully  described.  For  the  surgical  suc- 
cesses are  among  the  most  brilliant  of  recent  achievements  in  neurology, 
and  my  own  experience,  working  with  McBurney,  McCosh,  Hartley, 
and  Harvey  Gushing,  has  been  extensive. 

It  is  my  hope  that  in  this  new  form  the  work  will  add  to  its  prac- 
tical service  as  a  text-book  for  students,  and  as  a  guide  for  physicians 
in  the  recognition  and  treatment  of  nervous  diseases. 

M.  Allen  Staer. 

Five  West  F^fty-fotjrth  Street, 
New  York. 


/■ 


CONTENTS 


PART  I. 

The  Structure  of  the  Nervous  System  and  the 
Diagnosis  of  Nervous  Diseases. 

CHAPTER  I. 
Introductoey.    The  General  Diagnosis  of  Nervous  Diseases       ,    17 

CHAPTER  II. 
The  Structure  op  the  Nervous  System 21 

CHAPTER  III. 
The  Peripheral  Nervous  System .36 

CHAPTER  IV. 
The  Spinal  Cord 47 

CHAPTER  V. 

The  Diagnosis  and  Localization  of  Brain  Diseases       .        .        .82 

PART  II. 
Organic  Nervous  Diseases. 

SECTION  I. 

Injuries  and  Diseases  of  the  Nerves. 

CHAPTER  VI. 
Injuries  of  Nerves.    Neuritis 159 

CHAPTER  VII. 

Injuries  of  the  Spinal  Nerves  and  Special  Forms  op  Neuritis.  174 

CHAPTER  VIII. 
Multiple  Neuritis 197 

CHAPTER  IX. 
The  Toxic  Forms  of  Multiple  Neuritis 212 

V 


vi  .  CONTENTS. 

CHAPTER  X. 

The  Infectious  and  Toxemic  Forms  of  Multiple  Neuritis  .        .  235 

CHAPTER  XL 
Epidemic  Multiple  Neuritis 245 

CHAPTER  XII. 

Multiple   Neuritis  Associated  with   Other  Diseased   States  of 
THE  Body 250 

SECTION    II. 
Diseases  of  the  Spinal  Cord. 

CHAPTER  XIII. 
The  Spinal  Bloodvessels 259 

CHAPTER  XIV. 
Poliomyelitis .  262 

CHAPTER  XV. 
Chronic  Atrophic  Paralysis.        ........  284 

CHAPTER  XVI. 
Muscular  Dystrophies 303 

CHAPTER  XVII. 
Syringomyelia  or  Gliosis  Spinalis 320 

CHAPTER  XVIII. 
Lateral  Sclerosis.    Spastic  Paraplegia 337 

CHAPTER  XIX. 
Locomotor  Ataxia.    Tabes  Dorsalis.    Posterior  Spinal  Sclerosis.  342 

CHAPTER  XX. 
Combined  Sclerosis 383 

CHAPTER  XXI. 

Myelitis  and  Myelomalacia         .        .        .        .        .        .        -        .  392 

CHAPTER  XXII. 
Injuries  of  the  Spinal  Cord.     H^ematomyelia 416 

CHAPTER  XXIII. 
Compression  of  the. Spinal  Cord 433 


CONTENTS.  vii 

CHAPTER  XXIV. 

Landry's  Paralysis.    Acute  Progressive  Paralysis.        .        .        .  454 

CHAPTER  XXV. 
Spinal  Meningitis 457 

SECTION   III. 
Diseases  of  the  Brain. 

CHAPTER  XXVI. 
The  Cerebral  Circulation 463 

CHAPTER  XXVII. 
The  Cerebral  Diseases  of  Vascular  Origin 467 

CHAPTER  XXVIII. 
The  Cerebral  Atrophies  of  Childhood 514 

CHAPTER  XXIX. 
Encephalitis ,  535 

CHAPTER  XXX. 
Meningo-Encephalitis.    Paresis.    Dementia  Paralytica         .        .  540 

CHAPTER  XXXI. 

Abscess  of  the  Brain 549 

CHAPTER  XXXII. 

Thrombosis  of  the  Venous  Sinuses 567 

CHAPTER  XXXIII. 
Tumors  of  the  Brain 571 

CHAPTER  XXXIV. 
Bulbar  Paralysis  .        .        .        .        .        .        .        .        .        .        .  614 

CHAPTER  XXXV. 
The  Cranial  Nerves  and  Their  Diseases 622 

SECTION   IV. 

General  Diseases  of  the  Nervous  System. 

CHAPTER  XXXVI. 
Multiple  Sclerosis 675 


Viii  CONTENTS. 

CHAPTER  XXXVII. 

Syphilis  of  the  Nervous  System 686    « 

CHAPTER  XXXVIII. 
Pellagra 698 . 

CHAPTER  XXXIX. 
Caisson  Disease .  701 

CHAPTER  XL. 
Pachymeningitis  and  Meningeal  Hemorrhage  ....  705 

CHAPTER  XLI. 
Cerebral  Meningitis 713 

PART  III. 

Functional  Diseases. 

CHAPTER  XLII. 
The  Painful  Neuroses 729 

CHAPTER  XLIII. 
Headache  and  Migraine 755 

CHAPTER  XLIV. 
Functional  Paralysis.  766 

CHAPTER  XLV. 

The  Spasmodic  Neuroses.    Myoclonia 778 

CHAPTER  XLVI. 
The  Spasmodic  Neuroses 78^ 

CHAPTER  XLVII. 
Tetany 804 

CHAPTER  XLVIII. 

Paralysis  Agitans  and  Tremor 806 

CHAPTER  XLIX. 
Epilepsy 813 

CHAPTER  L. 

Neurasthenia  and  the  Occupation  NEXfROSES 837 

CHAPTER  LI. 
Psychasthenia ,        ...  863 


CONTENTS.  IX 

CHAPTER  LII. 
Hysteria. 878 

CHAPTER  LIII. 
Vertigo 898 

CHAPTER  IJV. 
Disorders  of  Sleep 903 

CHAPTER  LV. 
Reflex  Neuroses 909 

PART  IV. 
The   Sympathetic  Nervous  System  and  its  Diseases. 

CHAPTER  LVI. 
Vaso-Motor  and  Trophic  Neuroses .,  915 

CHAPTER  LVII. 
Symmetrical  Gangrene .  932 

CHAPTER  LVIII. 
Angio-Neurotic  (Edema 938 

CHAPTER  LIX. 
Trophic  Neuroses 942 

Index 955 


PART  I. 

STfilJCTUEE  OF  THE  NERVOUS  SYSTEM 

AND 

DIAGNOSIS  OF  NERVOUS  DISEASES 


CHAPTER   I. 

INTEODUCTOEY.     THE  GENEEAL   DIAGNOSIS  OF  NEEVOUS 

DISEASES. 

THE  METHOD  OF  EXAMINATION  OF  A  PATIENT. 

The  multitude  and  variety  of  symptoms  which  are  present  in  ner- 
vous diseases  are  frequently  bewildering  and  render  it  difl&cult  for  the 
student  to  know  how  to  examine  a  patient  suffering  from  a  nervous 
disease.  Some  suggestions  regarding  methods  of  examination  are  neces- 
sary, and  when  each  symptom  is  elicited  by  this  examination  the  sig- 
nificance of  each  symptom  or  group  of  symptoms  will  be  discussed. 

It  is  important  in  every  case  to  obtain  a  complete  history  of  the 
patient  before  proceeding  to  the  physical  examination.  This  history 
should  elicit  the  hereditary  tendencies  of  the  patient,  for  the  family 
history  often  affords  an  important  clue  to  the  disease  present.  Alco- 
holism or  drug  addiction,  chronic  lead  or  other  poisons,  such  diseases 
as  gout,  tuberculosis  or  anaemia,  as  well  as  the  various  nervous  diseases 
in  parents  make  the  liability  to  nervous  affections  in  the  next  genera- 
tion greater,  and  are  often  the  cause  of  functional  affections,  such  as 
epilepsy,  neurasthenia,  psychasthenia  and  the  tics.  There  are  some 
diseases  which  run  through  several  generations  in  a  family,  and  which 
seem  to  imply  some  defective  capacity  of  development.  Thus  heredi- 
tary deafness  and  blindness ;  the  dystrophies  affecting  the  muscles ; 
the  forms  of  ataxia  seen  in  Friedreich's  disease  and  in  Marie's  cere- 
bellar atrophy,  and  juvenile  spastic  paraplegia  are  all  examples  of  what 
Gowers  has  termed  abiotrophy,  a  congenital  tendency  for  the  special 
organ  or  part  of  the  nervous  system  involved  to  wither  and  die  long 
before  the  life  of  the  individual  ends. 

The  personal  history  of  the  patient  should  be  carefully  studied,  for 
the  development  in  early  life  of  convulsions,  of  headache,  of  habit 
spasms,  of  fainting  fits,  of  mental  peculiarities,  or  mental  defects  will 
2  17 


18  THE  GENERAL  DIAGNOSIS  OF  NERVOUS  DISEASES. 

show  a  state  of  weakness  of  structure  or  error  of  function  in  the  brain 
which  may  be  a  cause  predisposing  to  the  occurrence  of  nervous  disease 
in  later  life.  There  are  many  nervous  aifections  directly  or  indirectly 
traceable  to  bad  habits,  to  alcoholism,  to  chronic  toxaemia  by  drugs  or 
metals  or  poisons,  to  the  infectious  diseases  and  to  syphilis.  That 
many  nervous  aifections  appear  as  a  sequel  of  infections  is  now  well 
known  ;  the  neurasthenia  and  psychasthcnia  following  the  grippe  being 
examjiles.  Sometimes  the  occupation  of  the  patient  will  at  once  lead 
to  the  diagnosis  ;  as  in  lead  palsy  or  in  writer's  or  telegrapher's  cramps, 
or  in  the  paraplegia  developing  in  those  who  work  in  caissons.  There 
are  not  a  few  cases  of  nervous  disease,  both  organic  and  functional, 
which  are  due  to  injury  or  to  its  attendant  mental  shock.  And  the 
influence  of  care,  anxiety,  and  long-continued  worry  and  overwork  of  a 
mental  kind  is  fully  appreciated  by  all  physicians  as  a  cause  of  nervous 
disease. 

The  history  of  the  mode  of  onset  of  the  affection  in  question  is 
equally  important.  The  sudden  appearance  of  symptoms,  trauma  being 
excluded,  suggests  some  primary  vascular  disease,  as  in  apoplexy ; 
or  some  acute  infection,  as  in  infantile  paralysis  or  chorea.  The 
gradual  onset  is  characteristic  of  the  more  slowly  advancing  sclerotic 
changes,  in  the  nervous  system  which  cause  so  many  forms  of  pro- 
gressive paralysis  ;  while  the  possibility  of  tumor  growth  should  never 
be  overlooked  as  a  cause  in  these  cases.  Lastly  it  is  not  to  be  for- 
gotten that  many  nervous  symptoms  are  wholly  secondary  to  and  the 
result  of  diseases  of  other  organs,  as  for  example  the  headaches  from 
eyestrain,  the  reflex  neuroses,  neurasthenia  of  pulmonary  or  gastric  or 
intestinal  origin,  hysteria  from  ovarian  disease. 

The  most  prominent  symptoms  related  by  the  patient  will  as  a  rule 
indicate  whether  the  affection  is  functional  or  organic.  The  fact  of 
occasional  complete  intermission  in  these  symptoms  is  itself  suggestive 
of  functional  disease,  as  in  neuralgia  as  contrasted  with  neuritis,  as  in 
epilepsy,  or  in  the  spasmodic  neuroses,  as  contrasted  with  the  perma- 
nence of  paralysis  or  of  contracture  or  of  ataxia.  The  extreme  varia- 
bility in  the  character  and  in  the  intensity  of  symptoms,  such  as  is  seen 
in  neuralgia,  iu  neurasthenia,  and  in  hysteria,  is  at  once  suggestive  of 
a  functional  disease  :  for  when  organic  changes  have  occurred  in  the 
brain  or  in  the  spinal  cord  the  symptoms  are  not  only  permanent  but 
are  invariable.  And  the  susceptibility  of  a  patient  to  various  physical 
or  psychical  impressions  or  remedies  is  also  an  indication  that  the 
symptoms  do  not  depend  upon  destruction  of  nerve  tissue.  Thus  the 
history  of  the  chief  complaints  and  of  their  mode  of  appearance  and 
constancy  will  usually  give  a  clue  to  the  form  of  disease  present. 

If  that  disease  is  clearly  organic  in  nature  there  is  rarely  any  difficulty 
in  assigning  it  to  the  part  of  the  nervous  system  primarily  involved. 
Lesions  of  the  nerves  produce  pain,  paralysis,  anaesthesia  and  trophic 
symptoms  wholly  different  in  their  exact  locality  and  distribution  in 
the  body  from  lesions  of  the  spinal  cord,  and  hence  by  the  grouping  of 
the  symjjtoms  rather  than  by  differences  in  their  kind  a  differentiation 


mn  QENEBAL  DIAGNOSIS  OF  NEBVOUS  DISEASES.       '      19 

between  nerve  and  cord  lesions  is  reached.  Lesions  of  the  spinal  cord 
produce"  symptoms  wholly  different  in  their  distribution  in  the  body 
from  lesions  in  the  brain,  one  being  as  a  rule  bilateral,  the  other  uni- 
lateral ;  one  being  attended  by  electrical  changes  in  the  muscles  and  a 
loss  of  reflex  action  in  the  muscles,  the  other  not.  Lesions  of  the  brain 
produce  many  changes  in  mental  activity,  in  consciousness,  memory 
and  temper,  in  the  powers  of  thought,  expression  and  action,  in  the  use 
of  the  cranial  nerves,  in  the  gait  and  balance  never  seen  in  disease  of 
the  nerves  or  of  the  spinal  cord.  Thus  a  cursory  examination  at  once 
directs  the  attention  of  the  examiner  to  the  portion  of  the  nervous 
system  chiefly  affected. 

The  objective  examination  to  determine  the  exact  character,  extent, 
and  location  of  each  symptom ;  and  to  ascertain  the  actual  degree  of 
incapacity  of  every  function  is  more  important  than  the  history.  During 
the  progress  of  such  an  examination  incidentally  the  character  and  tem- 
perament of  the  patient,  his  emotional  condition  as  shown  in  his  facial 
expression,  attitude,  and  manner  of  conversation,  his  degree  of  intelli- 
gence, his  power  of  understanding,  his  capacity  of  speech,  of  movement, 
or  of  balance,  can  easily  be  observed  without  direct  questions.  Dis- 
turbances in  the  state  of  the  mind,  in  the  character  and  acuteness  of 
the  emotional  control,  in  the  rapidity  of  mental  response  to  questions 
and  impressions,  and  in  memory  are  symptoms  which  are  to  be  elicited 
by  observation,  as. the  patient  may  be  unaware  of  them,  and  unable  to 
judge  of  his  own  condition.  Powers  of  expression,  the  capacity  to 
weigh  and  judge  matters,  the  prevailing  mood,  are  all  important  indi- 
cations of  the  mental  state  ;  and  can  sometimes  be  learned  better  from 
relatives  than  from  the  patient.  Hence  the  views  of  those  with  whom 
he  lives  are  always  to  be  obtained. 

In  a  case  of  shaking  palsy,  or  of  hemiplegia,  or  of  locomotor  ataxia, 
or  of  spastic  paraplegia,  mere  inspection  as  a  patient  walks  into  the 
office  is  sometimes  sufficient  to  warrant  a  diagnosis.  But  it  is  only  after 
a  thorough  investigation  of  the  powers  of  motion,  of  sensation,  of  reflex 
activity,  of  coordination,  of  gait,  of  speech,  of  mental  activity  and  of 
self-control  that  one  should  feel  sure  that  the  entire  ground  has  been 
covered  in  the  examination. 

The  various  symptoms  presented  by  the  various  forms  of  nervous 
disease  are  fully  discussed  in  the  following  chapters  of  this  part,  each 
symptom  in  affections  of  the  nerves,  of  the  spinal  cord  and  of  the 
brain  being  described,  the  manner  in  which  these  symptoms  may  be 
elicited  being  portrayed,  and  the  significance  of  each  symptom  being 
indicated.  The  attempt  is  made  to  trace  each  symptom  to  its  under- 
lying pathological  cause,  so  that  the  nature  and  situation  of  the  lesion 
incidentally  appears  from  the  study  of  the  symptoms.  But  as  this 
necessarily  implies  a  knowledge  of  the  anatomy  and  physiology  of  the 
nervous  system  tlie  essential  and  important  facts  of  applied  anatomy 
are  included  in  the;  chapters  upon  diagnosis. 

If  in  the  use  of  this  text-book  constant  comparison  is  made  between 
the  symptoms   described  in  each  djs^ease  and  the  discussioji  of  the  si^-- 


20  THE  GENEEAL  DIAGNOSIS  OF  NERVOUS  DISEASES. 

nificance  of  those  symptoms  in  the  chapters  upon  diagnosis,  a  much 
more  comjjlete  view  will  be  gained  of  the  disease  in  question,  and  it 
will  become  at  once  apj^arent  how  a  complete  study  of  a  symptom  in 
nervous  diseases  usually  leads  at  once  to  a  definite  conclusion  as  to 
both  the  character  and  the  situation  of  the  underlying  lesion  which 
causes  it. 

The  use  of  the  book  will  be  enhanced  by  a  constant  reference  to  the 
index  where,  incidentally,  the  classification  of  the  causes  of  the  majority 
of  symptoms  appears. 

As  a  working  scheme  for  such  a  neurological  examination  the  follow- 
ing is  oifered. 

Present  complaint,  with  history  of  its  onset  and  course. 

Previous  diseases,  and  habits,  and  injuries.     Heredity. 

Appearance,  station,  gait,  power  of  motion  or  ataxia. 

Investigation  of  each  cranial  nerve  in  order,  with  particular  atten- 
tion to  the  special  senses  and  ophthalmoscopic  examination  and  to  the 
motion  of  eyes,  face  and  tongue. 

Examination  for  paralysis  ;  including  state  of  reflexes  of  pupils  and 
superficial  and  deep  reflexes. 

Examination  for  disturbance  of  touch,  temperature,  pain  and  mus- 
cular senses ;  with  particular  care  in  the  location  of  these  or  of  all 
spontaneous  pain  or  numbness. 

Condition  of  speech,  and  intelligence  and  emotional  state. 

General  physical  examination  of  all  organs. 


CHAPTER   II. 

THE   STRUCTURE   OF   THE   NERVOUS   SYSTEM. 

I.  The  Cerebro-spinal  Nervous  System.  Neurones,  Central  and  Peripheral.  Den- 
drites. Axones.  Terminal  Tassels.  Varieties,  of  Neorones.  Nutrition  and 
Pathology  of  Neurones.     II.  The  Sympathetic  Nervous  System. 

The  nervous  system  is  divided  into  two  parts,  the  cerebro-spinal 
system  and  the  sympathetic  system. 

I.    THE  CEREBRO-SPINAL  NERVOUS  SYSTEM. 

The  cerebro-spinal  nervous  system  consists  of  two  large  cen- 
tral organs,  the  brain  and  spinal  cord,  and  of  a  very  extensive  and 
wide-spread  peripheral  system  of  nerves  which  bring  these  central 
organs  into  communication  with  every  part  of  the  body.  Inasmuch 
as  every  form  of  functional  activity  in  the  organism  requires  direc- 
tion in  accordance  with  the  needs  of  each  organ  and  also  of  the  entire 
body,  it  is  evident  that  a  central  regulating  station  in  perfect  connec- 
tion with  all  the  diiferent  parts  is  a  necessity.  The  cerebro-spinal 
nervous  system  supplies  this  need.  Its  function  consists  in  the  recep- 
tion of  impressions  and  the  response  to  these  impressions  by  actions 
that  are  performed  in  a  properly  ordered  manner  adapted  to  an  end. 
Incidentally  impressions  and  actions  are  registered,  so  that  recollection 
is  possible  and  repetition  is  easy.  These  acts  may  be  unconscious  and 
automatic,  or  conscious  and  voluntary,  according  to  the  parts  of  the 
nervous  system  which  are  called  into  play  by  the  impression.  But, 
whether  conscious  or  unconscious,  the  action  is  always  one  of  control 
of  the  lower  mechanisms  of  the  body ;  of  the  sensory  organs,  and  of 
the  voluntary  and  involuntary  muscles ;  of  the  circulatory  and  respi- 
ratory systems ;  of  the  secretory  glands,  and  of  the  digestive  and  re- 
productive systems.  Thus  the  nervous  system  may  be  considered  as 
the  governing  and  controlling  system  of  the  entire  organism. 

The  central  organs  of  the  nervous  system  are  made  up  of  gray  and 
white  matter,  whose  visible  difference  led  older  anatomists  to  attach 
much  importance  to  their  varying  arrangement.  The  gray  matter  was 
known  to  be  made  up  of  cells,  and  the  white  matter  of  nerve  fibres 
transmitting  impulses  to  and  from  the  cells.  But  recent  investigation 
has  demonstrated  that  gray  and  white  matter  are  merely  different  parts 
of  elementary  bodies,  termed  neurones,  which  make  up  the  nervous 
system,  and  that  the  study  of  these  neurones,  of  their  structure,  and 
of  their  connections,  whether  they  lie  singly  or  in  groups,  as  in  the 
spinal  cord,  or  in  masses,  as  in  the  basal  ganglia  of  the  brain,  or  spread 

21 


22  TEE  STHUCTUEE  OF  TUE  NEEVOVS  SYSTEM. 

out  in  layers,  as  in  the  cerebral  cortex,  is  a  matter  of  the  greatest 
importance  from  every  point  of  view.  It  is  found  that  the  white  mat- 
ter and  tlie  nerves  are  merely  the  distal  portions  of  these  neurones,  and 
are  not  to  be  studied  apart  from  the  central  part  or  body  which  forms 
the  gray  matter.  It  is  therefore  the  chief  object  of  the  anatomist 
to-day  to  establish  the  situation  of  the  neurone  body  and  the  course 
and  termination  of  the  neurone  branches,  their  mutual  relations,  and 
their  manner  of  action  and  interaction.      (See  Plate  I.,  frontispiece.) 

The  neurones  are  held  together  by  neuroglia  and  connective  tissue, 
and  are  amply  supplied  with  bloodvessels  and  lymphatics. 

Classes  of  Neurones.  —  There  are  two  classes  of  neurones  differ- 
ent in  their  origin  in  embryonal  life.  The  first  class  develops  in  the 
central  nervous  system.  The  second  class  develops  in  the  ganglia, 
which  lie  upon  the  nerves. 

I.  The  First  ot  Central  Class  of  Neurone.  —  The  neurone  consists 
of  a  cell  body  and  its  projecting  branches.  These  are  of  two  kinds,  den- 
drites and  axones. 

The  cell  body  has  various  shapes  in  different  parts  of  the  nervous  sys- 
tem, being  spherical,  pyramidal,  and.  polygonal,  as  shown  in  Fig,  1. 
The  actual  structure  of  the  body  is  shown  in  Plate  II,  A,  page  22,  which 
demonstrates  a  protoplasmic  mass  unstained,  a  nucleus  and  nucleolus 
and  a  large  number  of  granular  bodies  which  are  more  or  less  regularly 
arranged  in  the  protoplasm  about  the  nucleus.  These  bodies  are  stained 
by  basic  aniline  dyes  and  have  been  termed  Nissl  bodies,  chromophile 
bodies,  stainable  substance,  or  tigroid.  Nissl  bodies  are  made  up  of 
phospho-albumin,  and  it  is  supposed  that  this  exists  in  solution  in  the 
living  cell  among  the  other  ingredients  of  the  protoplasm,  but  is  pre- 
cipitated by  the  fixative  and  then  stained  by  the  dye.  The  cell  body 
varies  greatly  in  size  in  different  parts  of  the  nervous  system ;  it  is 
from  4  to  135  microns  in  diameter.  Through  the  cell  body  fine  fibrils 
have  been  shown  to  pass  in  many  directions  from  and  to  the  branching 
processes.^     (Figs.  2  and  3.) 

The  dendrites  are  branching  protoplasmic  processes  of  the  cell  body 
and  resemble  it  in  structure.  They  are  very  numerous  in  some  neu- 
rones. Near  the  cell  body  they  are  thick,  but  as  they  extend  away 
they  divide  and  subdivide,  resembling  the  roots  of  a  tree,  until  they 
end  in  fine  fibrils.  The  dendrites  vary  in  length,  some  neurones  having 
one  very  long  dendrite  and  many  short  ones.  These  variations  are 
shown  in  Fig.  1.  They  pursue  a  tortuous  course  and  are  very  irregu- 
lar in  their  size,  shape,  and  branching.  The  surface  of  the  dendrite 
is  rough  and  often  nodular,  and  appears  to  be  covered  with  small  gran- 
ules called  buds  or  gemmules.     Within  the  dendrite  fine  filaments  lie 

1  The  reader  is  referred  to  Barker' s  Nervous  System,  Appleton  &  Co.,  1898  ;  to  Ewing, 
Studies  on  Ganglion  Cells,  Archives  of  Neurology  and  Psychopathology,  1898,  vol.  i., 
p.  263 ;  to  Bailey,  Morphology  of  Ganglion  Cells,  Journal  of  Experimental  Medicine, 
1901,  vol.  V,  p.  550  ;  to  Nissl,  Die  Neuronenlehre,  Jena,  1903,  and  to  Lewandowsky^ 
Handbuch  der  Neurologic,  Bd.  I,  1910. 


/■ 


THE  CEREBROSPINAL  NERVOUS  SYSTEM. 
Fig.  1. 


23 


Neurones,     1,  anterior  horn  of  sjiinal  cord;  2,  optic  thaluiniis ;  3,  posterior  spinal  ganglion  (chick 
embryo);  4,  cerebellum  ;  5,  cerebral  cortex,  surface  layer. 


24  THE  STRUCTURE   OF  THE  NERVOUS  SYSTEM. 

Fig.  2. 


Large  pluripolar  ganglion  cell  of  the  ventral  paramedian  field  of  the  abdominal  cord  of  Lvmbriciis. 
A  primitive  fibril  is  seen  on  its  way  to  a  ganglion  cell  becoming  thinner,  owing  to  the  emission  of  the 
side  fibrils.  A  very  complicated  intracellular  reticulum  of  neuro-fibrils  is  to  be  made  out  in  the  forma- 
tion of  which  the  primitive  fibrils  of  all  the  processes  take  part,  kk,  nucleolus ;  k,  nucleus,  a  and  /3 
are  processes  containing  one  primitive  fibril  in  each,  which  arriving  in  the  cell  body  split  up  into 
several  bifurcating  neuro-fibrils.  The  primitive  fibril  in  a  can  be  followed  for  a  very  long  distance, 
being  thicker  at  a  distance  from  the  cell.  The  coarse  longitudinal  processes  y  and  &  contain  a  large 
number  of  primitive  fibrils  which,  as  far  as  they  can  be  followed,  do  not  unite  to  one  fibril.  (After  S. 
Apathy,  Mitth.  aus  der  zool.  St.  zu  Neapel,  1897,  Bd.  xii.,  Ht.  4,  Taf.  xxvii.,  Fig.  7.) 

Fig.  3. 


Cells  of  the  anterior  corpora  quadrigemina  of  the  cat,  to  show  neuro-fibrils.  A,  axone  ;  B,  plexus 
of  neuro-fibrils  made  up  of  fibrils  entering  the  cell  from  the  branches  ;  C,  perinucleur  plexus  of  fibrils 
in  a  cell  which  has  been  divided  longitudinally;  D,  fibrils  running  from  the  plexus  into  the  branches. 
(Ramon  y  Cajal,  Bibliog.  Anat,  1904,  p.  264.) 


THE  CEBEBEO-SPINAL  NEBVOUS  SYSTEM. 


25 


which  pass  into  the  cell  body,  and  through  it  into  other  dendrites  or 
into  the  axorie.^  These  filaments  are  called  neuro-fibrils.  They  were 
first  demonstrated  by  Apathy  in  worms  and  have  been  more  fully  des- 
cribed in  the  hmnau  neurone  by  Ramon  y  Cajal.  (See  Fig.  2.)  It 
is  supposed  that  the  dendrites  collect  and  transmit  nervous  impulses  to 
the  cell  body  ;  and  as  they  are  niunerous  and  long  it  is  evident  that 
impulses  from  many  sources  may  reach  any  one  cell  body. 

The  axone  or  axis-cylinder  process  of  the  neurone  is  a  single,  long 

Fig.  4. 


<^' 


^^ 


1 


-xt 


A,  Cell  dividpr'  ]  ".gitudinally  ;  B,  surface  of  cell  showing  the  "terminal  feet "  of  Held,  the  manner 
of  termination  of  the  fine  neuro-fibrils  upon  the  surface  of  the  cells.  These  fibrils  come  from  a  plexus  of 
fibrils  lying  about  the  cells.     (Ramon  y  Cajal,  Bibliog.  Anat.,  1904.) 

branch,  arising  from  a  cone-like  projection  of  the  cell  body.  This 
cone-like  projection,  as  well  as  its  base  within  the  cell  body,  con- 
tains no  chromophile  substance,  but  fine  fibrils  can  be  found  within  it 
by  acid  stains.  It  is  supposed  to  transmit  nervous  impulses  from 
the  cell.  It  is  straight  and  smooth  in  contour.  It  undergoes  a 
slight  contraction  soon  after  leaving  the  cell  body,  but  then  becomes 
larger  and  remains  uniform  in  diameter.  Though  in  its  course  within 
the  central  organs  it  gives  oif  very  fine  collateral  filaments  at  right 
angles  to  its  direction,  it  does  not  branch  in  the  nerve  until  it  reaches 
its  very  end,  some  distance  from  the  cell  body.  There  it  divides  into 
a  number  of  little  end  fibrils  resembling  a  tuft  or  tassel.  (Fig.  7.) 
In  the  central  organs  it  is  possible  that  these  fibrils  enter  the  dendrites 
of  other  neurones.     It  is  possible  that  they  end  in  free  terminations 


^  Van  Gehuchten. 
March,  1905. 


Le  Ndvraxe,  January,  1904.     Cajal,  Bibliographic  Anatomique, 


26 


TTtE  STBUCTUBE  OF  THE  NEHVOVS  SYSTEM. 


which  merely  touch  these  dcDcl rites.  It  is  also  possible  that  they  end 
on  the  surface  of  other  cell  bodies  in  what  are  known  as  the  plates 
of  Auerbach  or  the  end  feet  of  Held,  but  the  exact  connection  is  not 
yet  determined.  (See  Figs.  3  and  4.)  The  collaterals  terminate  in  the 
same  manner.  The  termination  of  the  axone  in  muscles  is  in  the 
so-called  muscle  plate.  In  the  other  organs  it  ends  in  a  fine  plexus 
of  nerve  filaments.  The  axone  is  made  up  of  many  fine  fibrils  packed 
together,  which  are  gathered  from  the  cell  body  and  from  its  dendrites. 


Fig.  5. 


Purkinje  neurone  of  cerebellum.    Many  dendrites.    One  axone  projecting  from  the  body. 

Fig.  8  shows  this  structure.  The  size  of  the  axone  at  its  exit  from 
the  cell  body  varies  in  different  neurones.  Some  axones  extend  long 
distances,  as  from  the  cortex  of  the  brain  to  the  lowest  part  of  the 
spinal  cord.     This  variety  is  known  as  Type  I.  of  Golgi.     (Fig.  9.) 

Some  axones  terminate  near  the  cell  body,  dividing  and  subdividing 
mto-a  sort  of  network.  This  variety  is  known  as  Type  II.  of  Golgi. 
(Fig.  10.)     Between  these  extremes  all  varieties  are  to  be  found. 

When  the  long  axones  leave  the  gray  matter  they  are  insulated  from 
each  other  by  receiving  a  medullary  sheath  or  fatty  coating,  held  in 
place  by  a  thin  membrane,  but  this  they  lose  at  their  termination.  In 
Fig.  9  this  is  shown.  Some  axones  on  nearing  their  termination  ap- 
pear to  split  in  two  in  a  Y-shaped  manner  and  each  division  has  its 
own  end  tassel.     If  the  nervous  impulse  passes  out  from  the  cell  body 


THE  CE-REBnO-SPINAL  NEnrOtJS  ST.'^fEM. 


S7 


along  the  ax(>ne  it  is  evident  that  it  may  reach  many  different  destina- 
tions, because  of  tlie  different  places  of  ending  of  tlie  collaterals  and  of 


Fig.  6. 


Neurones  of  the  cerebral  cortex,  second  layer.  The  long  dendrite  extends  to  the  surface  layer. 
Smaller  dendrites  go  out  from  the  body.  The  axons  passes  downward  from  the  body,  giving  off 
collaterals. 


28 


TEE  STBVCTVBE  OF  TEE  NERFOUS  SYSTEM. 


Fig.  7. 


Fig.  8. 


The  terminal  fibres  of  the  axones  ;  a,  axone  bifurcating  near  its  termination  ;  6,  c,  d,  terminal 
brushes.     (Eamon  y  Cajal,  Studien  iiber  die  Hirnrinde  des  Meuschen,  I.,  55.) 

the  terminal  filaments.     The  terminal  filaments  of  the  axones  are  never 
continuous  with  other  axones ;  but  impulses  are  probably  conveyed 
from  one    neurone  to  another    by  the   contact  of 
axones  with  adjacent  dendrites  or  with  other  cell 
bodies. 

The  nutrition  of  both  axones  and  dendrites  is 
controlled  by  the  cell  body,  for  they  atrophy  when 
separated  from  it.  Nerves  are  the  axones  of  neu- 
rones collected  into  bundles  and  held  together  by 
connective  tissue. 

II.  The  Second  or  Peripheral  Class  of  Neurone. — 
This  class  belongs  to  the  sensory  part  of  the  ner- 
vous system.  It  develops  originally  in  the  poste- 
rior spinal  ganglia  that  lie  outside  of  the  spinal 
cord  and  in  the  homologous  ganglia  that  are  in 
Axone  from  the  sciatic  couuection  witli  the  scusory  crauial  nerves  and  also 
nerve  of  a  frog,  showing  in  the  ganglia  of  the  sympathetic  system.  The 
fibrillary  structure  and  a  gensory    ueurouc   iu   au    early    stage    of  develop- 

node  of  Eanvier.     (Ver-  .'I'l  i  i  •  i       n  ii 

^orn )  ment  is  bipolar,  a  branch  coming  out  ol  each  end 

of  the  cell  body.  (Fig.  1,  3.)  Later  the  two 
branches  appear  to  be  fused  together  for  a  short  distance,  and  thus  in 
the  adult  the  sensory  neurone  is  pear-shaped  and  appears  to  send  out 
a  single  axone  which  divides  into  two  branches,  passing  in  opposite 
directions.     One  of  these  branches  finds  its  way  outward  to  the  periph- 


TBE  CEBEBEO-SPINAL  NEFvVOUS  SYSTEM. 


29 


ery  of  the  body,  forming  a  sensory  nerve,  and  terminates  in  a  fine 
brush-like  Expansion  of  filaments  in  the  skin  or  in  the  tactile  cor- 
puscles. The  other  branch  finds  its  way  inward  through  the  posterior 
nerve   into  the  spinal   cord  or  brain  axis,   where   it   bifurcates,   one 


Fig.  9. 


ft^s? 


Scheme  of  central  motor  neurone,  f I.  type  of  Golgi. )  The  motor  cell  body,  together  with  all  its 
protoplasmic  processes,  its  axis-cylinder  process,  side  fibrils,  or  collaterals,  and  end  ramifications, 
represent  parts  of  a  single  cell  or  neurone,  a.h.,  axone-hillock  devoid  of  Nissl  bodies,  and  showing 
fibrillation;  nx.,  axis  cylinder  or  axone.  This  process,  near  the  cell  body,  becomes  surrounded  by 
myelin,  m.,  and  a  cellular  sheath,  the  neurilemma,  the  latter  not  being  an  integral  part  of  the  neurone  ; 
c,  cytoplasm  showing  Nissl  bodies  and  lighter  ground  substance  ;  d.,  protoplasmic  processes  (dendrites) 
containing  Nissl  bodies;  ».,  nucleus;  n.',  nucleolus;  n.R.,  node  of  Ranvier ;  .v./.,  side  fibril;  n.  of  n., 
nucleus  of  neurilemma  sheath;  lei.,  motor  end  plate  or  telodendron;  m.',  striped  muscle  fibre;  s.L,, 
segmcntatioD  of  Lanterraann.    (Barker.) 


30  TBE  STEUCTUEE  OF  THE  NEEVOUS  SYSTEM. 

portion  passing  downward  and  the  other  portion  upward  in  the  pos- 
terior columns  of  the  spinal  cord,  or  in  the  formatio  reticularis  of  the 
brain  axis.  As  these  main  branches  pass  up  and  down  within  the 
cord  they  give  off  at  right  angles  little  collaterals,  and  these  collaterals, 


Golgi's  cell  of  Type  II.  from  the  dorsal  horn  of  the  gray  matter  of  the  spinal  cord  of  the  newborn 
mouse.  Even  in  such  a  neurone  the  axone  is  very  easily  distinguishable  from  the  dendrites.  The  lat^ 
ter  are  only  represented  in  part  in  the  illustration.     (After  von  Lenhossek.) 

together  with  the  main  branches,  terminate  in  brush-like  extremities 
within  the  gray  matter  of  the  posterior  horn,  either  near  their  entrance 
or  as  far  from  their  entrance  as  the  posterior  nuclei  of  the  medulla 
oblongata,  or  at  various  levels  in  the  brain  axis.  The  sensory  axone 
never  terminates  in  a  cell  in  the  spinal  cord  or  brain  axis.  It  ends  in 
brush-like  terminations  or  tassels. 

In  this  form  of  neurone  the  cell  body  is  situated  about  one-half  way 
between  the  terminal  extremities  of  its  two  great  branches,  and  this 
fact  of  the  interposition  of  the  body  in  the  course  of  a  nerve  tract, 
which,  from  the  nature  of  the  case,  must  be  a  continuous  tract,  sug- 


TEE  CEBEBBO-SPINAL  NEEVOUS  SYSTEM.  31 

gests  that  the  function  of  the  cell  body  is  a  trophic  one.  We  have 
proof  also  t)iat  an  axone  is  not  always  centrifugal  in  the  direction  of 
its  impulses. 

The  structure  of  the  cell  body  in  this  class  of  neurones  is  not  dif- 
ferent from  that  in  the  first  class ;  but  the  chromophile  granules  are 
arranged  about  the  nucleus  in  a  series  of  concentric  rings.  (See 
Plate  II.,  B.,  page  22.) 

Varieties  of  Neurones.  —  Neurones  have  been  classified  into  : 

1.  Primary  neurones,  whose  cell  bodies  lie  in  the  central  nervous 
system  or  ganglia  and  whose  axones  extend  thence  to  some  peripheral 
part  of  the  organism,  to  a  muscle,  or  to  a  gland,  or  to  the  skin,  or  to 
some  sensory  organ,  and 

2.  Secondary  neurones,  whose  cell  bodies  lie  in  the  central  nervous 
system  and  whose  axones  extend  to  some  other  part  of  the  central 
nervous  system,  to  terminate  about  a  primary  neurone  or  about  another 
secondary  neurone. 

Neurones  have  also  been  classified  according  to  their  function  into 

1.  Centrifugal :  (a)  motor,  (6)  secretory,  (c)  trophic. 

2.  Centripetal  or  sensory. 

3.  Intrinsic  or  association. 

The  diagram  (Plate  I.,  frontispiece)  illustrates  these  various  neurones 
and  their  relations  to  one  another. 

Reflex  acts  may  be  performed  by  the  primary  neurones,  but  all  con- 
scious sensations  or  voluntary  acts  involve  the  action  of  both  primary 
and  secondary  neurones  acting  together.  This  action  may  be  illus- 
trated by  the  analysis  of  a  simple  sensation,  like  the  impact  of  a  particle 
of  dust  in  the  eye,  which  causes  a  wink,  a  flow  of  tears,  a  conscious 
pain,  and  a  voluntary  effort  to  remove  the  particle.  The  sensation 
comes  in  from  the  eye  along  the  primary  sensory  neurone,  is  trans- 
mitted (a)  to  the  brain  axis,  where  it  reaches  the  primary  motor  neurone, 
setting  up  the  centrifugal  reflex  acts  of  a  wink  and  of  secretion  of  tears, 
and  (6)  to  the  secondary  sensory  neurone,  which  transmits  it  upward 
to  the  brain.  This  secondary  neurone  may  terminate  about  another 
secondary  or  association  neurone,  which  then  sends  the  impulse  on  to 
a  secondary  motor  neurone,  and  this  in  turn  sends  its  impulse  down  to 
the  primary  neurone,  which  transmits  it  to  the  muscle,  causing  the 
removal  by  the  hand  of  the  particle. 

There  are  many  more  complex  acts,  either  automatic,  like  the  act  of 
breathing  or  sneezing ;  or  voluntary,  like  the  act  of  walking  or  talk- 
ing, which  call  into  play  a  vast  number  of  neurones  —  centripetal, 
intrinsic,  centrifugal  —  which  cannot  be  so  easily  analyzed.  But  how- 
ever complex  the  act,  the  physical  basis  of  all  nervous  and  mental 
activity  is  this  series  of  neurones. 

The  arrangement  of  these  neurones  and  their  mutual  relations  will 
be  considered  in  connection  with  the  studies  of  diseases  of  the  various 
parts  of  tlic  nervous  system.  In  that  connection  also  the  vascular 
supply  of  the  nervous  system  will  be  described. 


32  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM. 

The  Nutrition  of  the  Neurone. — In  order  that  these  neurones 
may  do  their  work  they  must  have  a  normal  structure  and  a  normal 
capacity  lor  sustaining  their  nutrition  when  in  activity.  When  a  neu- 
rone is  made  to  work  it  undergoes  certain  manifest  changes.  Hodge  ^ 
was  the  first  to  describe  alterations  in  the  form  and  appearance  of  the 
cell  and  of  its  nucleus  consequent  upon  its  activity.  There  is  a  gen- 
eral diminution  in  the  size  of  the  cell,  a  lessened  power  to  absorb  stain- 
ing substances,  which  may  be  taken  as  a  proof  of  the  using  up  of  its 
own  substance  and  also  changes  in  the  appearance  of  the  nucleus, 
which  is  decreased  in  size,  and  changes  from  a  smooth  and  rounded 
outline  to  a  jagged  and  irregular  one.  As  the  cell  becomes  changed 
in  its  structure  by  constant  work,  it  becomes  more  and  more  exhausted, 
so  that  finally  there  comes  a  time  when  it  is  no  longer  capable  of  send- 
ing out  impulses,  and  requires  a  period  of  rest  to  make  up  what  it  has 
lost  of  form  and  to  regain  a  store  of  energy.  Vas "  and  Mann  ^  have 
described  these  physiological  changes  more  exactly.  Vas  showed  that 
mild  stimulation  of  a  cell  caused  a  swelling  of  its  body  and  of  the 
nuclei  and  a  clearing  up  of  the  central  part  of  the  cell  by  an  apparent 
movement  of  the  chromatin  bodies  to  its  periphery.  Mann  showed 
that  functional  activity  of  the  cell  is  accompanied  by  an  increase  in 
the  size  due  to  imbibition  of  the  lymph  lying  in  the  cavity  about  the 
cell,  so  that  the  cell  at  work  fills  up  the  cavity  in  which  it  lies.  When 
activity  goes  on  to  the  point  of  fatigue,  then  a  shrivelling  of  the  cell 
begins,  first  in  the  nucleus,  then  in  the  body.  At  the  same  time 
changes  go  on  in  the  chromatin.  During  the  period  of  activity  the 
chromatin  material  is  used  up,  so  that  a  fatigued  cell  does  not  absorb 
staining  material  as  does  a  cell  at  rest.  In  an  exhausted  cell  the  only 
stain  is  in  and  about  the  nucleus.  In  Plate  II.  these  changes  are 
shown.  These  results  have  been  reached  by  stimulating  cells  to  work 
in  living  animals  either  by  electricity  or  by  keeping  up  movements, 
such  as  running,  or  by  exposing  one  eye  to  the  light  while  the  other 
was  kept  dark,  and  then  contrasting  the  appearance  of  the  cells  made 
to  work  with  those  that  were  kept  at  rest.  When  a  stimulated  cell  is 
allowed  to  rest  it  gradually  resumes  its  original  appearance ;  but  the 
period  of  rest  must  be  adequate.  During  the  period  of  reconstruction 
the  chemical  activities  going  on  in  the  cell  are  numerous,  and  its 
power  of  assimilation  of  material  furnished  to  it  by  the  blood  must  be 
increased.  If,  however,  anything  interferes  with  this  increased  nutri- 
tion, such  as  an  imperfect  supply  of  blood  or  a  deleterious  substance 
of  any  kind  in  the  blood,  then  the  process  of  building  up  fails  to  take 
place,  and  the  neurone  cannot  resume  its  function. 

The  Pathology  of  the  Neurone. — The  fundamental  fact  at  the  basis 
of  all  forms  of  nervous  disease  is  some  change  in  the  power  of  the  neu- 
rone to  continue  its  normal  functions.  This  may  be  of  a  temporary 
character,  whose  nature  is  as  yet  not  understood,  and  not  attended  by 

*  Hodge.     American  Journal  of  Psychology,  vol.  ii. ,  p.  39. 

^Vas.  Ueber  den  Bau  des  Chromatins  in  den  lymphatischen  Ganglien,  Archiv, 
fiir  Mikroscopische  Anatoraie,  1892,  Heft  3,  p.  375. 

3  Gustav  Mann.     Journal  of  Anatomy  and  Physiology,  October,  1894. 


THE  CEREBROSPINAL  NERVOUS  SYSTEM.  33 

visible  alterations  of  form ;  and  may  be  succeeded  by  a  resumption  of 
natural  action^     This  is  the  hypothetical  basis  of  functional  nervous 
diseases.     But  the  loss  of  power  in  the  neurone  may  also  be  due  to 
damage  more  or  less  grave  to  its  structure,  visible  through  the  micro- 
scope, and  often  incapable  of  repair.     This  is  the  basis  of  organic  dis- 
eases of  the  nervous  system.     The  varieties  will  be  carefully  considered 
in  connection  with  the  various  diseases  studied.     It  is  always  to  be 
remembered  that  the  pathological  changes  which  go  on  in  the  neurone 
are  in  no  sense  distinct  from  those  occurring  in  the  cells  of  other 
organs ;  but  the  exact  appearances  are  determined  by  the  structure  of 
the  neurone  as  diiferent  from  that  of  other  cells.     We  are  at  present 
able  to  see  changes  in  the  size  and  shape  of  the  cell  body  and  of  its 
nucleus  and  nucleolus.      We  can   distinguish,  by  Nissl's  method  of 
staining,  changes  in  the  chromophile  bodies  within  the  cell  which  con- 
sist in  a  gradually  progressing  disintegration  of  these  bodies  until  they 
are  resolved  into  a  fine  dust,  and  then  entirely  disappear,  leaving  the 
cell  body  a  homogeneous,  unstained  mass.     The  protoplasmic  substance 
in  which  these  chromophile  bodies  lie  also   undergoes  degenerative 
changes  in  disease ;  but  our  staining  methods  are  not  adequate  to  show 
this.     We  can  see  a  displacement  of  the  nucleus  of  the  cell  body  to 
one  side  as  the  chromophile  bodies  disappear,  which  indicates  either  a 
solution  of  the  substance  about  the  nucleus,  and  its  consequent  sinking 
by  gravitation  from  lack  of  support,  or  an  active  migration.      We  can 
see  the  development  of  vacuoles  within  the  cell  body,  indicating  an 
absorption  of  both  protoplasmic  and  chromophile  debris.     And  we  can 
see  a  gradual  shrivelling  of  the  cell  body  until  its  place  is  taken  by  a 
granular  scar.     The  dendrites  and  the  axone  share  in  the  pathological 
processes  within  the  cell  body,  showing  similar  evidences  of  disinte- 
gration and  destruction,  or  the  axone  alone  may  undergo  these  changes 
in  lighter  grades  of  atrophy.    All  forms  of  neurones  appear  to  undergo 
similar  pathological  alterations.     These  may  be  classed  together,  no 
matter  what  their  cause,  as  parenchymatous  inflammatory  processes  or 
as  parenchymatous  degeneration.     There  are  certain  chemical  changes 
which  attend  degeneration  in  the  neurones  which  have  recently  been 
determined  by  Mott.^     He  has  ascertained  that  when  nervous  tissue 
degenerates,  protagon  or  lecithin  breaks  up  into  choline,  glycero-phos- 
phoric  acid,  and  stearic  acid.     The  protagon  is  converted  into  a  fat  of  a 
different  composition,  the  phosphoretted  fat  being  changed  into  a  non- 
phosphoretted  neutral  fat.      This  is  a  process  of  hydration,  and  is 
expressed  by  the  following  equation  : 

C44H90NPO  +  SH^O  =  2C,8H3A  +  C3H9PO6  +  CsHisNOa 
(lecithin)         (water)    (stearic  acid)      (glycero-         (choline) 

phos.  acid) 

Parenchymatous  degeneration  may  be  attended  by  or  may  be  due  to 
inflammation  in  the  connective  tissue  or  in  the  neuroglia  which  every- 
where surrounds  and    supports  the  neurones.     Such  inflammation  is 

'Arch,  of  Neurology  of  the  London  County  Asylums,  1899,  vol.  i.,  p.  185. 
3 


34  THE  STRUCTURE  OF  THE  NERVOUS  SYSTEM. 

termed  interstitial,  and  the  mechanical  compression  exerted  upon  the 
neurones  by  an  increase  in  the  supporting  substance  may  cause  serious 
degeneration  in  the  neurone.  Again,  in  those  forms  of  inflanmiation 
in  which  we  have  an  emigration  of  leucocytes,  we  can  often  see  these 
bodies  invading  the  neurone  body  and  apparently  destroying  its  tissue. 
It  has  also  been  shown  that  bacteria  may  find  lodgment  within  the 
neurone  body  or  in  its  branches,  causing  irritation  and  destruction ;  and 
it  is  not  unlikely  that  in  some  cases  the  invasion  of  the  neurone  by 
leucocytes  is  really  an  attempt  to  remove  these  bacteria  by  phagocytosis. 
In  Plate  III.  these  changes  are  shown. 

All  these  pathological  processes  will  be  more  fully  studied  in  connec- 
tion with  the  various  diseases  to  be  considered. 

II.    THE  SYMPATHETIC  NERVOUS  SYSTEM. 

The  sympathetic  nervous  system  is  the  second  division  of  the  ner- 
vous system.  Though  closely  connected  with  the  cerebro-spinal  sys- 
tem by  the  nerves,  it  appears  to  differ  from  it  in  its  structure  and  to 
be  independent  of  it  in  its  function.  It  consists  of  a  series  of  masses 
of  gray  matter  enclosed  in  capsules  termed  ganglia,  made  up  of  spheri- 
cal cell  bodies  and  joined  to  each  other  and  to  the  so-called  involuntary 
organs  of  the  body — the  heart,  bloodvessels,  and  lymphatics ;  the 
lungs ;  the  digestive,  hematogenetic,  secretory,  and  reproductive  sys- 
tems— by  nerves  of  a  peculiar  type  whose  axones  lack  insulating 
material  or  medullary  sheaths,  and  hence  are  gray  instead  of  white. 

The  sympathetic  system  is  subdivided  into  : 

1.  Two  great  cords  containing  ganglia,  which  lie  on  the  sides  of  the 
vertebral  column  and  are  connected  with  the  spinal  cord  on  one  side 
and  with  the  plexuses  on  the  other. 

2.  Three  prevertebral  plexuses,  the  cardiac,  solar,  and  hypogastric, 
which  are  masses  of  ganglia  connected  with  the  viscera. 

3.  Many  peripheral  plexuses  connected  with  individual  organs. 

4.  Terminal  monocellular  ganglia  scattered  through  the  viscera. 

5.  Sympathetic  nerve  fibres  which  join  these  plexuses  and  ganglia 
together  and  also  establish  their  connection  with  the  spinal  cord  and 
brain. 

Many  groups  of  cells  lying  in  the  central  region  of  the  spinal  cord  and 
in  the  lateral  part  of  the  central  gray  matter  receive  and  give  off  nerve 
fibres  which  pass  by  way  of  the  nerve  roots  to  the  spinal  nerves,  and 
thence  to  the  sympathetic  cords  and  ganglia.^  These  nerves  are  both 
sensory  and  motor ;  hence  it  is  probable  that  some  part  of  the  activity 
of  the  sympathetic  system  is  controlled  by  the  spinal  cord. 

The  connection  of  the  sympathetic  system  with  the  brain  is  made 
by  the  great  vagus  or  pneumogastric  nerve. 

The  action  of  the  sympathetic  system  in  regulating  the  vegetative 
functions  and  the  acts  of  the  heart,  bloodvessels,  and  lungs  is  usually 

^Onuf  and  Clollins.  Archives  of  Neurology  and  Psychopathology,  1900,  vol.  iii., 
No.  1. 


/' 


..« 


,■     .■■(llllllM-fc.l 


THE  SYMPATHETIC  NERVOUS  SYSTEM.  35 

automatic  and  unconscious ;  but  this  activity  may  be  reflected  upon  con- 
sciousness id"  an  indefinite  manner  and  cause  changes  in  the  emotional 
state  and  in  the  general  feeling  of  comfort.  Head,  who  has  made  a 
careful  study  of  this  subject/  affirms  that  exaltation  or  depression  of 
spirits,  hallucinations,  a  state  of  suspicion,  and  a  change  of  character 
may  be  induced  by  the  reflected  pains  of  visceral  disease.  He  ascribes 
many  of  the  symptoms  which  we  term  hysterical  to  such  sources.  It 
is  also  a  fact  that  unusual  states  of  consciousness,  expectant  attention, 
and  conditions  of  the  mind  and  brain  induced  by  hypnotic  suggestion 
may  in  turn  produce  physical  changes  in  the  vascular  and  vegetative 
organs.  These  can  be  explained  only  by  admitting  a  control  by  the 
brain  of  these  organs  through  sympathetic  channels,  but  in  an  uncon- 
scious and  involuntary  manner. 

^The  Gulstonian  Lectures  for  1901.     Brain,  1901,  vol.  xxiv.,  p.  345. 


CHAPTER  III. 

THE  PERIPHERAL  NERVOUS  SYSTEM. 

The  peripheral  nervous  system  is  made  up  of  the  twelve  cranial 
and  thirty-one  spinal  pairs  of  nerves  which  connect  every  part  of  the 
body  with  the  central  nervous  system ;  viz.,  the  cerebral  axis  and  the 
spinal  cord ;  and  also  of  the  sympathetic  nervous  system  with  its 
various  ganglia.  The  nerves,  as  already  shown,  are  merely  prolonga- 
tions of  the  neurones ;  motor  nerves  being  the  axones  of  the  motor 
neurones  situated  in  the  anterior  gray  horns  of  the  spinal  cord  and  in 
the  gray  matter  of  the  medulla,  pons,  and  crura  cerebri ;  sensory 
nerves  being  the  axones  of  sensory  neurones  situated  in  the  posterior 
spinal  ganglia  and  in  the  ganglia  of  the  cranial  nerve  roots ;  sympa- 
thetic nerves  being  the  axones  of  the  various  sympathetic  neurones 
situated  in  their  ganglia. 

THE  HISTOLOGY  OF  A  NERVE  FIBRE. 

When  a  nerve  trunk  is  dissected,  the  connective-tissue  sheath  or 
perineurium  enclosing  its  fibres  torn  away,  and  the  individual  fibres  set 
free  by  tearing  the  finer  connective-tissue  strands  or  endoneurium 
which  bind  them  together,  it  is  possible  to  distinguish  certain  parts  by 
means  of  appropriate  methods  of  staining. 

There  is,  first,  the  axone  or  axis  cylinder.  (Fig.  11.)  This  is  made 
up  of  a  number  of  primitive  fibrils  longitudinally  arranged  and  con- 
tinuous throughout  the  length  of  the  nerve.  The  fibrils  are  cemented 
together  by  a  substance  which  appears  finely  granular.  Each  axone 
comes  from  a  single  nerve  cell ;  the  individual  fibrils  come  from  the 
cell  body  and  from  its  dendrites.  The  fibrils  pass  together  in  the  axone 
to  the  periphery  and  there  terminate,  the  individual  fibrils  branching 
out  in  various  directions,  and  joining  with  other  fibrils  from  other 
axones  to  form  a  fine  plexus  within  the  organ  to  which  the  nerve  as  a 
whole  has  gone.  It  has  been  thought  possible  to  trace  individual 
fibrils  of  the  plexus  into  individual  epithelial  cells  in  various  internal 
organs  and  in  the  skin.  Other  axones  can  be  traced  directly  to  ter- 
minal organs,  such  as  the  terminal  plates  upon  the  muscle  and  the  ter- 
minal bulbs  and  corpuscles  in  the  skin.  In  these  no  division  or 
branching  of  the  fibrillary  constituents  of  the  axis  cylinder  has  been 
discovered.  Their  termination  in  each  of  the  organs  of  special  sense 
is  still  different.  The  diameter  of  an  axis  cylinder  varies  from  2.6 /z 
to  7.5  /;!.  It  is  no  longer  believed  that  the  longest  fibres  have  the  great- 
est diameter. 

There  is,  secondly,  the  myelin  sheath  surrounding  the  axis  cylinder. 

36 


TEE  EI8T0L0GY  OF  A  NEBVE  FIBRE. 


37 


(Fig.  11.)  ,^This  is  not  a  continuous  tube,  but  consists  of  a  series  of 
short  tubes  or  interannular  segments  placed  end  to  end.  These  seg- 
ments vary  in  length  from  0.1  mm.  to  1  mm.  and  are  longer  on  thick 
fibres.  The  point  of  junction  of  two  adjacent  segments  is  indicated 
by  a  constriction  in  the  contour  of  the  nerve  fibre,  the  node  of  Ranvier, 
and  if  the  fibre  be  stained  with  osmic  acid  the  myelin  will  be  seen  to 
be  deficient  at  these  nodes.  (Fig.  8.)  If,  by  any  means,  the  nerve 
fibre  be  broken  the  myelin  in  any  segment  will  run  out  and  collect  in 
drops,  showing  that  it  is  a  semifluid  substance  of  fatty  nature. 


Node  of  Ranvier. 


'"Neurofibrils 
'  Cement  stibstance 


"Axis  cylinder 


^Line  of  Schmidt- 
Lantermann 


' — Schwann's  corpuscle 


Schwann's  sheath~i 


•Medullary  sheath 


Mauthner's  sheath 


.Schwann's  sheath 


Axis  cylinder~-i 


Diagram  of  the  structure  of  a  medullated  nerve  fibre,  showing  two  different  views  Concerning  the 
relations  of  the  sheaths  of  Mauthner  and  of  Schwann.    Compare  the  right  and  left  sides.    (Symonowicz. ) 


The  myelin  sheath  is  not  a  necessary  constituent  of  all  nerve  fibres, 
for  the  majority  of  the  nerves  of  the  sympathetic  system  are  devoid  of 
such  sheaths.  Nor  does  the  myelin  sheath  of  the  nerves  of  the  cerebro- 
spinal system  extend  from  end  to  end  of  the  axone.  For  the  axone 
first  receives  its  sheath  at  some  little  distance  from  the  cell  from  which 
it  issues ;  and  at  its  termination,  where  it  breaks  up  into  branching 


38 


TEE  PEBIPHEEAL  NEHVOUS  SYSTEM. 


fibrils,  the  myelin  envelope  ceases.  Throughout  the  course  of  the 
nerve,  however,  the  myelin  sheath  is  present  in  the  cerebro-spinal 
nerves,  forming  a  protecting  envelope  and  probably  acting  as  an  insu- 


lating and  nutrient  substance  as  well. 


There  is,  thirdly,  the  sheath  of  Schwann  —  a  connective-tissue  mem- 
brane surrounding  the  myelin  sheath.  (Fig.  9.)  This,  like  the  last, 
develops  in  segments  which  become  cemented  to  one  another  at  the 
points  of  constriction  of  the  fibre ;  but  finally  the  continuity  between 
adjacent  segments  becomes  complete,  so  that  in  an  adult  fibre  the 


Fig.  12. 


Cross-section  of  a  nerve,  showing  five  bundles  of  nerve  fibres. 

sheath  is  continuous  over  the  node  of  Ranvier,  and  here  the  sheath  of 
Schwann  is  the  only  covering  of  the  axis  cylinder.  On  the  inner  side 
of  this  sheath,  and  half-way  between  two  nodes,  a  nucleus  is  found. 
The  sheath  of  Schwann  extends  almost  to  the  terminal  filaments  of  the 
axone,  covering  them  for  a  little  distance  after  they  have  lost  the 
myelin  sheath.  By  appropriate  staining  fine  lines  may  be  shown  pass- 
ing between  the  outer  and  inner  layers  of  protoplasm  through  the 
myelin  sheath  ;  the  so-called  incisures  of  Schmitt-Lantermann.  These 
have  been  considered  little  trabeculse  of  the  protoplasm  within  the  seg- 
ment, in  the  midst  of  which  the  myelin  lies.  Eecent  staining  methods 
seem  to  indicate  that  they  belong  to  the  sheath  of  Schwann  and  are 
connective  tissue.  The  layer  of  protoplasm  lying  against  the  axis 
cylinder  is  the  layer  which  was  formerly  described  as  the  sheath  of 
Mauthner.  Some  authorities  consider  that  a  layer  of  connective  tissue 
similar  to  the  sheath  of  Schwann  surrounds  the  axis  cylinder,  but  this 
is  still  uncertain.     Two  views  of  its  relations  are  shown  in  Fig.  11. 

Thus  the  nerve  fibre  consists  of  a  central  conducting  strand  sur- 
rounded and  insulated  by  a  series  of  cylinders  placed  end  to  end  and 
joined  to  one  another. 


SYMPTOMS  OF  DISEASES  OF  THE  NEBVES.  89 

Nerves.  ^  Individual  nerve  fibres  are  associated  in  bundles  held 
together  hf  fine  connective-tissue  cells  whose  nuclei  in  a  carmine  or 
fuchsin-stained  preparation  can  be  seen  lying  always  adjacent  to,  but 
outside  of,  the  sheath  of  Schwann.  This  has  been  called  the  endo- 
neurium,  while  the  connective-tissue  sheath  surrounding  the  entire 
bundle  is  named  the  perineurium.  The  connective  tissue  holding 
numerous  bundles  together  is  called  the  epineurium,  and  this  forms  the 
firm,  glistening  sheath  of  the  nerve.  Capillary  vessels  with  free  anasto- 
moses run  within  the  nerve,  their  walls  lying  adjacent  to  the  individual 
fibres,  and  thus  affording  a  perfect  supply  of  nutriment.  Lymph  spaces 
also  have  been  demonstrated  within  the  nerve  sheath,  but  not  among 
the  fibres.  That  the  interfibrillary  spaces  of  the  endoneurium,  however, 
open  into  these  perivascular  lymph  spaces  is  probable  from  the  fact 
that  they  do  so  in  other  organs.  While  it  is  evident  that  the  nutri- 
ment of  the  axis  cylinder  is  derived  from  the  circulatory  fluids,  it  is 
probable  that  it  is  only  at  the  nodes  of  Ranvier  that  the  absorption 
takes  place,  since  elsewhere  the  myelin  sheath  interferes  with  osmosis. 
Thus,  if  the  nerve  be  put  in  nitrate  of  silver,  it  is  only  opposite  the 
nodes  that  the  axis  cylinder  becomes  stained.  There  is  no  histological 
difference  between  motor  and  sensory  nerve  fibres. 

SYMPTOMS  OF  DISEASES  OF  THE  NERVES. 

Parsesthesia.  —  A  very  slight  pressure  upon  a  nerve,  of  short  dura- 
tion, may  cause  disagreeable  tingling  and  numbness  of  the  skin,  which 
is  felt  in  the  distribution  of  the  sensory  filaments  of  the  nerve.  This 
is  an  experience  common  to  everyone  when  from  pressure  on  the  sciatic 
nerve  the  foot  is  said  to  be  asleep.  Such  sensations  of  numbness  often 
occur  in  the  arm  and  hand,  or  in  the  foot  and  leg  at  night  during  sleep 
and  wake  the  patient,  who  may  be  alarmed,  and  from  a  recurrence  of 
the  symptoms,  anticipate  paralysis,  especially  if  the  numb  sensation 
is  temporarily  attended  by  awkward  movements  or  by  a  feeling  of 
weakness.  Inasmuch  as  the  relief  of  the  pressure  is  followed  by  a 
rapid  recovery  from  the  symptoms  such  sensations  are  of  no  importance. 
They  are  due  to  a  slight  impairment  of  nutrition  in  the  nerve  probably 
consequent  upon  a  venous  congestion,  for  a  similar  set  of  sensations  can 
be  caused  by  tying  a  string  tightly  about  the  finger  or  wrist  and  caus- 
ing a  stasis  of  venous  blood.  Some  persons  seem  to  be  especially  sub- 
ject to  such  symptoms.  Such  a  condition  does  not  ordinarily  amount 
to  a  neuritis,  but  if  it  is  long  continued  it  may  produce  it,  as  is  seen  in 
cases  of  musculospiral  palsy  from  sleeping  on  the  arm  m  a  state  of 
intoxication. 

A  slight  general  numbness  of  all  the  fingers  in  both  hands,  and  even 
of  the  hands  and  wrists  af5  well,  occasionally  occurs  in  attacks  of  short 
duration  or  may  become  permanent.  This  is  noticed  chiefly  in  women 
whose  hands  are  constantly  immersed  in  hot  water  or  who  use  the 
hands  continuously  for  hours  in  any  employment.  It  has  been  called 
acroparcesthesia.^     It  is  due  to  a  slight  impairment  of  nutrition  in  the 

^Dana.  New  York  Medical  Kecord,  January  20,  1891.  Collins,  New  York  Med- 
ical Record,  December  'S,  1901. 


40  TEH  PEBIPEEEAL  NEBVOUS  SYSTEM. 

nerves,  and  if  the  motor  as  well  as  the  sensory  nerves  are  aifected  it  is 
attended  by  a  sense  of  weakness  and  lack  of  skill.  Disturbances  of 
circulation  are  sometimes  the  cause,  as  in  numbness  from  pressure. 
These  symptoms  are  variable  in  duration  and  may  continue  if  the  cause 
is  not  removed  ;  but  they  never  go  on  to  any  serious  disease,  and  hence 
are  of  little  importance.  Rest  from  work  and  massage  are  the  best 
remedies. 

Pain.  —  The  effect  of  an  injury  or  wound  of  a  nerve  or  plexus  of 
nerves  is  immediate  pain  at  the  point  of  injury,  pain  or  disagreeable  sen- 
sations, such  as  numbness  or  prickling,  referred  to  the  regions  from  which 
the  sensory  filaments  in  the  injured  nerve  come,  anaesthesia  in  this 
region  to  all  forms  of  sensation,  of  temporary  or  permanent  duration, 
occasionally  attended  by  trophic  disturbances,  and  paralysis  of  the 
muscles  to  which  the  motor  filaments  in  the  injured  nerve  pass.  The 
paralysis  is  soon  followed  by  a  loss  of  faradic  contractility  in  the  muscle 
and  by  a  loss  of  its  mechanical  excitability  to  percussion,  and  later  by 
a  progressive  atrophy  of  the  muscle. 

At  the  point  of  injury  to  the  nerve  the  symptoms  vary.  They  differ 
when  the  skin  is  unbroken,  when  the  nerve  is  exposed  to  the  air,  and 
when  the  wound  is  infected.  In  the  first  case,  while  there  may  be 
slight  tenderness  to  pressure  there  may  be  no  spontaneous  pain.  Thus 
in  a  case  of  bilateral  ulnar  paralysis  from  pressure  exerted  on  the 
nerves  of  the  arms  during  a  long  surgical  operation,  where  the  arms 
hung  over  the  sides  of  the  operating  table  for  an  hour  and  their  weight 
against  the  sharp  edge  caused  the  injury,  the  patient  had  no  pain  at 
all  and  no  tenderness  at  the  point  of  injury.  In  the  common  form  of 
injury  to  the  musculospiral  nerve  from  pressure  on  the  arm  by  the 
head  during  the  heavy  sleep  of  intoxication,  the  patient  wakes  with 
wrist-drop,  but  has  no  pain.  In  facial  palsy  there  is  rarely  pain 
behind  the  ear.  Thus  where  the  injury  is  produced  by  long-continued 
slow  pressure  sufficient  to  destroy  the  nutrition  of  a  nerve  there  may 
be  no  pain.  On  the  other  hand,  pressure  on  a  nerve  by  a  tumor  or 
pressure  by  a  pregnant  uterus  on  the  lumbar  and  sacral  plexus  may 
give  rise  to  severe  pain,  both  local  and  distal.  If  the  nerve  is  lacer- 
ated suddenly,  as  by  a  strain  or  a  blow  or  by  a  fracture  of  a  bone  tear- 
ing it,  there  is  always  severe  pain  at  the  point  of  injury,  and  this  pain 
is  usually  much  increased  if  the  nerve  is  exposed  to  the  air  in  an  open 
wound.  In  these  cases  also  the  subsequent  connective-tissue  growth 
and  thickening  about  the  wounded  ends  is  tender  and  may  cause  great 
pain,  the  finer  filaments  becoming  entangled  in  the  scar  tissue. 

The  pain  after  injury  is  often  felt  not  only  at  the  point  of  injury, 
but  distally  in  the  entire  peripheral  distribution  of  the  nerve.  Thus  in 
a  case  of  ulnar  nerve  injury  occurring  with  a  fracture  at  the  elbow 
there  was  pain  of  an  agonizing  kmd  in  the  inner  condyle  of  the 
humerus  and  also  severe  shooting  pains  down  the  inner  side  of  the 
forearm  and  into  the  hand  and  little  and  ring  fingers ;  these  pains  grew 
worse  for  several  days  and  continued  for  many  weeks,  with  intense 
tenderness  in  the  entire  ulnar  nerve  distribution,  any  touch  in  this 


PLATE  IV 


The  Distribution  of  Sensory  Nerves  in  the  Skin.     (After  Flower.) 

The  areas  of  the  skin  supplied  by  the  cutaneous  nerves  are  shown  in  finely  dotted 
outline.  The  circles  on  the  trunk  show  areas  occasionally  anaesthetic  in  hysteria.  The 
lines  across  the  limbs  at  ankle,  knee  and  thigh,  wrist,  elbow  and  shoulder  show  the  upper 
linnits  of  anaesthesia  in  multiple  neuritis  of  varying  degrees  of  severity. 


SYMPTOMS  OF  DISEASES  OF   THE  NEBVES.  41 

region  causing  agony.  Ulcerations  of  the  skin  of  the  fingers  and  hand 
occurred  later  and  the  skin  became  glossy.  This  condition  remained 
stationary  until  by  operation  at  the  elbow  the  nerve  was  released  from 
a  mass  of  callus,  and  thus  was  freed  from  pressure,  when  all  the  symp- 
toms gradually  subsided  and  recovery  ensued. 

The  pain  and  tenderness  caused  by  an  injury  of  a  nerve  are  not 
always  limited  to  the  distribution  of  that  nerve,  as  one  would  expect. 
Thus  in  a  case  where  a  perforating  wound  of  the  sole  of  the  foot 
had  left  a  small  piece  of  rubber  shoe  embedded  in  the  flesh  the  orig- 
inal injury  was  slight,  the  wound  healed,  and  for  a  long  time  no 
inconvenience  was  felt;  but  four  years  later  pain  and  tenderness 
began  again  in  the  scar  and  extended  up  the  leg  and  thigh  until  the 
entire  limb  became  painful,  hypersensitive,  and  useless  from  the 
intense  tenderness,  and  even  the  lower  spine  was  sensitive  and  pain- 
ful. These  symptoms  all  subsided  rapidly  when,  by  operation,  the 
piece  of  rubber  was  removed.  It  was  found  to  be  surrounded  by  a 
little  mesh  of  fine  nerve  filaments  holding  it  like  a  basket.  The  rapid 
recovery  showed  that  there  was  no  true  ascending  neuritis,  as  had  been 
supposed.  In  such  cases  the  continued  local  pain  causes  a  hypersen- 
sitive state  of  the  central  organ  by  a  summation  of  impressions,  and 
this  shows  itself  by  a  general  pain.  In  many  cases  of  neuritis  of  one 
cord  of  the  brachial  plexus  the  pain  in  the  entire  arm  renders  it  use- 
less. The  pain  in  neuritis  is  increased  by  pressure  upon  the  affected 
nerve.  It  is  also  intensified  by  motion  of  the  limb,  especially  if  this 
causes  a  stretching  of  the  nerve. 

Anaesthesia.  —  The  anaesthesia  following  injury  of  nerves  is  more 
pronounced  and  extensive  immediately  after  the  injury  than  it  is  later. 
There  appear  to  be  fine  anastomoses  of  the  sensory  filaments  of  nerves 
in  the  skin,  and  thus  sensations  ordinarily  conveyed  by  one  nerve  may, 
if  that  nerve  be  injured,  pass  by  adjacent  nerves.  Such  anastomoses 
seem  to  be  more  complete  in  some  individuals  than  in  others,  hence 
in  two  patients  with  similar  injuries  the  anaesthesia  may  be  different 
in  extent.  Thus  after  injury  of  the  musculospiral  nerve  it  is  very 
•  common  to  find  sensation  in  the  back  of  the  hand  near  the  thumb 
perfectly  restored  within  a  week,  while  the  wrist-drop  may  last  some 
months.  After  a  division  of  the  median  nerve  above  the  wrist  I  have 
seen  the  anaesthesia  progressively  diminish  in  the  hand  long  before  the 
union  of  the  ends  of  the  severed  nerve  had  restored  the  muscular  power 
in  the  flexors  of  the  fingers.  After  excision  of  one  branch  of  the  tri- 
geminal nerve  for  neuralgia,  sensation  returns  in  the  face  in  a  consider- 
able part  of  the  region  which  is  anaesthetic  immediately  after  the  oper- 
ation ;  but  a  small  area  of  anaesthesia  is  usually  left  as  a  permanent 
result  of  injury  to  any  sensory  nerve,  and  this  includes  analgesia  and 
thermo-anaesthesia.  A  loss  of  muscular  sense  rarely,  if  ever,  follows  a 
nerve  injury.  This  is  because  many  different  sensations  combine  to 
produce  this  sense,  deep  and  superficial  nerves  from  muscles,  liga- 
ments, and  articular  surfaces  all  conveyiiig  impressions  which  are 
coordinated  in  tlie  spinal  cord  before  being  sent  up  to  tlie  brain  and 
appreciated  as  muscular  sense. 


4^  TEE  PEBIPHEEAL  NEBYOVS  SYSTEM. 

Head  ^  explains  this  in  an  interesting  manner.  He  believes  that  nerves 
contain  different  systems  of  fibres,  some  transmitting  sensations  of  pain 
and  of  extremes  of  temperature  which  he  terms  protopathic  sensations, 
others  transmitting  sensations  of  touch  and  of  slight  temperature  which 
he  terms  epicritic  sensations.  He  has  shown  that  after  an  injury  of  a 
nerve  the  distribution  of  these  different  forms  of  anaesthesia  varies 
somewhat  and  that  during  recovery  protopathic  sensations  return  before 
epicritic  sensations.  The  unit  of  protopathic  supply  lies  in  the  pos- 
terior roots,  but  of  epicritic  supply  in  the  peripheral  nerves.  The 
more  nearly  a  peripheral  nerve  represents  the  supply  of  one  posterior 
nerve  root  the  more  definite  will  be  the  border  of  the  analgesia;  the 
more  the  representation  of  posterior  nerve  roots  in  a  nerve  the  less 
definite  will  be  the  protopathic  symptoms.  He  believes  that  the  sensi- 
bility of  deep  parts,  of  joints  and  muscles  is  transmitted  by  the  sensory 
filaments  in  motor  nerves. 

As  regeneration  occurs  in  the  injured  nerve  sensations  gradually 
return  in  the  previously  insensitive  region,  but  at  first  all  sensations 
are  attended  by  an  abnormal  sense  of  tingling  or  numbness  which  is 
disagreeable.  This  tingling  or  numbness  is  often  perceived  during  the 
entire  course  of  the  disease.  It  is  then  due  to  irritation  in  the  central 
end  of  the  injured  nerve,  the  pathological  irritation  being  referred 
erroneously  to  the  region  whence  the  irritated  filaments  come.  Such 
numbness  may  be  attended  by  pain  of  a  sharp,  shooting  character,  and 
in  some  sensory  nerves,  notably  the  trigeminal  and  sciatic,  this  pain 
may  be  the  most  important  symptom  of  neuritis  from  pressure.  Thus 
in  a  case  of  osteosarcoma  of  the  pelvis,  sciatic  neuralgia,  supposedly 
functional,  preceded  for  seven  months  all  other  symptoms.  In  a  case 
of  osteoma  of  the  skull  near  the  optic  chiasm  trigeminal  neuralgia 
preceded  by  one  year  the  optic  atrophy  and  other  symptoms.  When 
septic  infection  occurs  in  the  injured  nerve  this  pain  is  more  intense 
than  under  other  circumstances,  and  it  is  then  always  attended  by  a 
state  of  hyperalgesia  in  the  affected  area.  Such  a  state  is  known  as 
anaesthesia  dolorosa,  for  pain  is  caused  by  any  touch  in  the  sensitive 
region,  even  though  the  touch  itself  is  not  perceived. 

Trophic  Disturbances  in  the  anaesthetic  region  occur  more  commonly 
in  septic  cases,  but  they  may  develop  in  any  case.  They  are  usually 
produced  by  neglected  injuries  of  the  insensitive  region,  which  become 
infected,  a  slight  pin  prick  or  a  scratch  being  capable  of  originating  a 
serious  trouble  if  not  disinfected  at  once.  Pain  is  nature's  means  of 
protection  in  health,  giving  warning  of  injuries  or  diseases  and  leading 
to  their  care ;  but  from  insensitive  regions  no  warnings  can  come,  and 
no  protection  is  therefore  given.  But  this  is  not  a  sufficient  explana- 
tion for  the  occurrence  of  such  trophic  symptoms  as  arise  in  neuritis, 
either  traumatic  or  spontaneous.  We  see  peculiar  bullous  eruptions, 
herpes,  acne  or  eczema,  extraordinary  vasomotor  conditions,  with  alter- 
nate flushing  and  pallor  of  the  skin  attended  by  intense  heat  and  cold, 
a  dryness  and  thinning  of  the  skin,  with  a  peculiar,  smooth,  shining 

1  Brain,  Part  II.,  1905.     Also  No.  121,  1908. 


Neck  in  usclea 


SYMPTOMS  OF  DISEASES  OF  TSE  NEBVFS.  4S 

appearance  termed  glossy  skin,  unusual  sweating,  which  may  be  acid 
and  fetid,  or 'hardening  and  thickening  of  the  skin,  or  abnormal  growth 
of  hair,  or  falling  of  the  hair ;  a  defective  or  irregular  growth  of  the 
nails,  which  may  be  ridged,  curved,  and  marked  with  white  plaques  in 
the  region  supplied  by  the  affected  nerve.  Very  rarely  acute  gangrene 
has  been  observed,  chiefly  in  old  persons.  The  occurrence  of  these 
symptoms  has  given  rise  to  the  hypothesis  of  trophic  nerves,  whose 
function  is  to  regulate  nutrition  and  repair  and  whose  injury  results  in 
processes  of  disease  in  the  derma  and  its  adnexa.  Such  trophic  symp- 
toms develop  only  when  the  nerve  injured  is  a  sensory  nerve.  The 
trophic  disturbances  seen  in  cases  of  locomotor  ataxia  and  syringo- 
myelia are  evidence  that  central  as  well  as  peripheral  lesions  may  be 
attended  by  such  symptoms ;  but  even  in  these  diseases  it  is  the  sen- 
sory portion  of  the  nervous  system  to  which  the  pathological  change  is 
limited.  The  true  explanation  of  trophic  changes  is  to  be  found  not 
in  the  hypothesis  of  an  injury  to  trophic  nerves,  but  in  the  fact  that 
ordinary  sensory  impressions  are  interrupted  or  perverted,  and  nature, 
lacking  its  accustomed  guide  to  repair  and  misled  by  abnormal  impres- 
sions, produces  effects  which  are  needless  under  the  circumstances  or 
fails  to  produce  those  which  ordinarily  would  be  required.  Thus,  an 
increased  secretion  of  sweat  may  be  a  natural  thing  under  a  certain 
condition  —  e.  (/.,  heat  —  when  unnatural  it  may  result  from  a  series 
of  abnormal  sensations  of  pathological  origin,  being  received  by  the 
central  organs  which  are  incapable  of  distinguishing  natural  from  arti- 
ficial impulses.  A  bed-sore  is  usually  produced  by  pressure  or  irrita- 
tion of  the  skin,  not  perceived,  and  so  not  removed  or  repaired.  If, 
by  extraneous  care,  such  pressure  and  irritation  are  avoided,  as  in 
health  would  occur  by  change  of  position  and  care  of  the  skin,  bed- 
sores may  be  entirely  prevented.  In  a  patient  of  my  own,  suffering 
from  transverse  myelitis  with  pronounced  tendency  to  bed-sores,  the 
simple  expedient  of  turning  him  every  half  hour  day  and  night  and 
wiping  off  the  skin  lightly  each  time  that  he  was  turned  prevented  for 
four  years  the  development  of  any  skin  abrasion.  I  have  never  seen 
trophic  skin  affections  occur  in  the  course  of  neuritis  when  the  parts 
were  properly  protected  from  injury,  and  slight  injuries  were  antisepti- 
cally  treated. 

It  must  be  stated,  however,  that  other  theories  than  the  one  here 
offered  have  been  advanced  to  account  for  the  trophic  symptoms  in 
neuritis.     They  are  ascribed  to  vasomotor  disturbance. 

Vasomotor  Symptoms.  —  Some  authors  believe  that  in  the  course 
of  neuritis  the  vasomotor  nerves  are  diseased  and  that  their  lesion 
results  in  an  irregularity  of  blood  supply  to  the  part  concerned.  This 
irregularity  consists  of  an  active  hypersemia,  as  is  proven  by  experi- 
mental section  of  the  sympathetic  nerve  to  a  rabbit's  ear,  which 
causes  extreme  dilatation  of  the  vessels  and  a  rise  of  temperature 
in  the  part ;  but  it  has  never  been  demonstrated  that  a  mere  condi- 
tion of  congestion  goes  on  to  a  condition  of  inflammation  unless 
other  causes,  chiefly  septic  or  microbic,  enter  as  a  factor.     Further- 


44  TEE  PEBIPEEBAL  NEBVOVS  SYSTEM. 

more,  it  is  well  known  that  the  bloodvessels  are  under  the  control 
of  vasomotor  ganglia  in  their  walls,  which  regulate  their  calibre  even 
after  division  of  the  fibres  which  connect  these  ganglia  with  the  spinal 
cord.  Thus,  after  division  of  the  sympathetic  in  the  ear  of  a  rabbit,  the 
congestion  gradually  subsides  in  the  ear  and  the  temperature  becomes 
normal.  Another  argument  against  the  vasomotor  origin  of  trophic 
disturbances  is  derived  from  the  study  of  cases  of  erythromelalgia^ 
This  is  an  affection  first  described  by  Weir  Mitchell,  characterized  by 
a  dilatation  of  the  arteries  in  the  extremities  resulting  in  an  extreme 
condition  of  redness  and  sensation  of  heat  and  pain.  It  is  an  affec- 
tion of  the  hands  and  feet  and  occasionally  involves  the  arms  to  the 
elbows,  and  the  legs  to  the  knees.  After  hours  of  extreme  heat  and 
flushing  the  extremities  may  suddenly  become  cold,  pale,  or  blue  and 
shrivelled  up,  presenting  the  appearance  that  the  hands  present  after 
long  soaking  in  hot  water,  and  this  alternation  of  distention  and  con- 
traction of  the  bloodvessels  constitutes  the  symptomatology  of  the  dis- 
ease. It  appears  to  be  a  pure  vasomotor  neurosis,  but  it  is  rarely,  if 
ever,  attended  by  any  trophic  disturbances  of  the  skin  or  of  the  nails, 
such  as  occur  in  neuritis,  and  no  pathological  observations  are  at  hand 
to  establish  the  hypothesis  that  there  is  a  true  neuritis  of  the  vaso- 
motor nerves.  For  this  reason  the  theory  of  the  vasomotor  origin  of 
trophic  affections  appears  to  be  incredible.  Head  affirms  that  trophic 
disturbances  occur  only  when  the  protopathic  system  of  nerves  is  in- 
volved and  this  accords  with  the  theory  I  have  already  advanced. 

Paralysis,  —  The  paralysis  which  results  from  traumatic  neuritis  is 
limited  to  the  muscles  supplied  by  the  injured  nerve,  and  is  total  only 
in  those  muscles  which  have  no  collateral  nerve  supply.  It  is  a  flaccid 
palsy,  and  if  it  results  in  any  stiffness  of  joints  or  limbs  or  deformity 
soon  after  the  injury,  this  is  due  to  contraction  in  the  healthy  unopposed 
muscles  and  not  to  contracture  in  the  paralyzed  ones.  After  a  long- 
continued  paralysis,  however,  attended  by  atrophy,  a  shortening  of  the 
weak  muscle  may  occur.  Thus,  in  cases  of  facial  palsy  which  do  not 
recover,  a  contracture  is  not  very  rare,  causing  stiffness  of  the  face. 
In  such  paralyzed  muscles  no  mechanical  or  reflex  motion  is  possible. 
Percussion  of  the  muscle  or  of  its  tendon,  therefore,  fails  to  produce 
any  response. 

Electrical  Changes.  —  Electrical  changes  soon  develop  in  the  mus- 
cles paralyzed,  usually  within  four  or  five  days  of  the  time  of  injury. 
These  are  of  several  kinds  and  are  known  as  the  i-eaction  of  degenera- 
tion, partial  or  complete.  In  health  a  muscle  may  be  made  to  contract 
by  sending  a  galvanic  or  faradic  current  through  its  nerve,  or  by 
applying  either  current  directly  to  the  muscle.  In  neuritis  the  nerve 
becomes  inexcitable  to  any  form  of  electricity  and  the  muscle  shows 
changes  in  its  excitability.  A  number  of  different  conditions  have 
been  observed.  Sometimes  there  is  a  simple  diminution  of  excita- 
bility, and  then  a  very  strong  faradic  or  galvanic  current  is  needed  to 
produce  contractions.  Frequently  all  faradic  excitability  is  lost,  and 
then  the  muscles  react  to  a  galvanic  current  only.     They  may  be  at 


Muscles  of  hacK' 


-»   c;     fy 


SYMPTOMS  OF  DISEASES  OF   THE  NEBVES.  45 

first  hypersensitive  to  galvanic  stimuli,  but  later  it  may  require  a  very 
strong  galvaiiic  current  to  produce  contraction.  The  contraction  is 
rarely  quick,  as  in  health.  It  is  sluggish  and  vermiform.  In  some 
cases  the  normal  polar  reaction  is  found,  and  the  closure  of  the  nega- 
tive pole  applied  to  the  muscle  produces  stronger  contractions  than 
the  closure  of  the  positive  pole.  This  is  termed  a  partial  reaction 
of  degeneration.  In  many  cases  the  contraction  of  the  muscle  when 
stimulated  with  the  positive  pole  is  greater  than  when  stimulated 
with  the  negative  pole.  This  is  termed  a  complete  reaction  of 
degeneration.  A  loss  of  faradic  irritability  and  a  marked  decrease 
in  the  galvanic  irritability  of  the  muscle  are,  therefore,  important 
symptoms  of  neuritis.  As  the  disease  goes  on  to  recovery  a  gradual 
increase  in  the  galvanic  irritability  occurs  —  a  fact  which  is  often 
of  much  aid  in  prognosis  if  careful  measurements  of  the  strength 
of  the  current  used  be  made  by  the  galvanometer.  It  is  possi- 
ble to  record  such  measurements  upon  charts,  and  thus  to  obtain  an 
electric  curve  for  each  muscle  which  is  paralyzed.^  These  curves 
enable  one  to  judge  of  the  progress  of  the  case  very  accurately,  and 
when  the  line  is  advancing  steadily  toward  the  normal  point,  after  a 
great  deflection  or  after  a  stationary  level,  the  prognosis  is  favorable. 

It  is  to  be  remembered,  however,  that  voluntary  power  always  re- 
turns some  time  before  electric  reactions  become  normal. 

The  extent  of  the  paralysis  in  any  case  is  determined  by  the  distri- 
bution of  the  nerve  aifected.  Thus  in  facial  palsy  all  the  muscles  of 
the  face  except  the  orbicularis  oris  are  paralyzed.  In  musculospiral 
paralysis  the  extensors  of  the  wrist  and  fingers  are  totally  paralyzed 
and  the  supinator  longus  is  partially  paralyzed.  In  peroneal  paralysis 
the  muscles  which  abduct  and  lift  the  foot  are  useless.  The  muscles 
supplied  by  the  various  nerves  of  the  brachial,  lumbar  and  sacral 
plexuses  are  shown  in  Plates  V.  and  VI.,  on  pages  42  and  44. 
From  an  examination  of  any  patient  it  is  easy  to  determine  what 
muscles  are  paralyzed.  Comparison  of  the  facts  elicited  with  these 
plates  will  enable  one  to  reach  a  conclusion  as  to  which  nerve  or  nerves 
or  plexus  is  affected.  But  if  the  extent  of  the  paralysis  cannot  be  ex- 
plained by  the  lesion  of  one  or  more  nerves,  it  is  probable  that  the 
paralysis  is  of  spinal  rather  than  nerve  origin. 

The  duration  of  the  paralysis  depends  wholly  on  the  question  of  the 
possibility  and  time  of  regeneration  of  the  nerve.  In  case  a  nerve  is 
divided  and  at  once  reunited  this  paralysis  may  disappear  in  a  couple 
of  weeks.  In  an  ordinary  traumatic  case  or  after  inflammation,  as  in 
facial  palsy,  six  weeks  may  be  the  limit.  In  other  cases  a  long  time, 
six  or  eight  months,  elapses  before  the  paralysis  passes  off.  The  further 
the  injury  from  the  end  of  the  nerve,  the  longer  the  time  to  recovery  — 
a  fact  which  gives  support  to  the  hypothesis  of  Ranvier  that  the  new 
nerve  must  grow  out  from  the  central  end  at  the  point  of  injury  and 
find  its  way  down  the  old  sheath  to  the  muscle.  In  musculospiral 
paralysis  from  pressure  over  the  humerus  seven  months  is  the  average 
*  Starr,  Journal  of  Nervous  and  Mental  Disease,  February,  1887,  vol.  xiv. 


46  TEE  PEEIPHEBAL  NERVOUS  SYSTEM. 

duration  to  recovery.  In  case,  however,  some  obstruction  to  repair 
exists  the  paralysis  is  permanent.  Hence  scar  tissue  which  prevents 
the  new  filaments  from  developing  must  often  be  removed  in  order  to 
effect  a  cure. 

Spasm  of  the  muscles  supplied  by  the  injured  nerve  occasionally 
occurs,  but  is  a  rare  symptom.  It  is  usually  a  reflex  phenomenon  due 
to  sensory  irritation  and  not  to  any  pressure  on  the  motor  nerve.  In 
a  gunshot  injury  of  the  ulnar  nerve  cramps  in  the  hand  and  extensors 
of  the  wrist  have  been  noticed  (Raymond).  In  the  so-called  occupa- 
tion neuroses  which  some  authors  ascribe  to  neuritis,  such  cramps  are 
common,  but  in  ordinary  cases  of  neuritis  they  do  not  occur. 

While  the  statements  of  the  symptoms  occurring  in  neuritis  thus  far 
made  are  generally  true,  it  is  found  that  neuritis  in  different  nerves 
produces  diverse  symptoms,  hence  a  special  consideration  of  neuritis  of 
the  different  nerves  is  necessary.     See  Chapter  VII. 

Diagnosis.  —  The  diagnosis  of  neuritis  usually  presents  no  diffi- 
culty. The  limitation  of  the  pain,  anaesthesia,  and  paralysis  to  the 
distribution  of  a  single  nerve,  and  the  tenderness  at  some  point  along 
its  course  are  characteristic  of  the  affection  at  the  onset,  and  within  a 
week  of  the  beginning  the  development  of  the  reaction  of  degeneration 
in  the  muscles  supplied  by  the  nerve  affected  makes  the  diagnosis  com- 
plete. In  Plate  IV.  the  area  of  the  skin  related  to  each  nerve  is 
shown.  If  a  neuritis  is  present  the  pain  parsesthesia  and  anaesthesia 
are  limited  to  that  part  of  the  skin  which  corresponds  to  the  affected 
nerve.  If  Plate  IV.  be  compared  with  Plate  VII.  it  will  be  evident 
that  the  area  of  skin  related  to  each  nerve  is  very  different  from 
the  area  of  skin  related  to  each  spinal  segment.  If  in  any  case 
of  pain,  parsesthesia  or  anaesthesia  the  distribution  of  these  in  the  skin 
is  rather  that  of  spinal  than  of  nerve  relation,  the  diagnosis  will  be  of 
spinal  rather  than  of  nerve  disease. 

On  page  55  the  grouping  of  the  paralyzed  muscles  in  spinal  lesions 
is  given.  This  may  be  contrasted  with  the  grouping  indicated  as 
characteristic  of  nerve  lesions  in  Plates  V.  and  VI.  on  pages  42  and 
44.  The  exact  determination  of  the  extent  of  tlie  paralysis  will 
enable  one  to  reach  a  conclusion  as  to  whether  it  is  due  to  nerve  or  to 
spinal  lesion.  Thus  if  all  the  small  muscles  of  the  hand  are  paralyzed 
and  atrophied  it  will  be  much  more  likely  that  the  lesion  is  in  the  first 
dorsal  segment  of  the  cord  than  in  the  ulnar  and  median  nerves ;  and 
if  the  long  flexors  of  the  fingers  escape  it  will  be  evident  that  the 
median  nerve  is  not  affected ;  hence  the  lesion  must  be  spinal. 


DESCRIPTION  OF  PLATE  VII. 
This  diagram  has  been  constructed  by  combining  the  results  of  a  large  number  of  cases  of  transverse 
lesion  of  the  cord  at  different  IcTcls  studied  or  reported  during  the  past  twelve  years.    The  dorsal  areas 
are  taken  from  Head. 


PLATE  VII 


Areas  of  Anassthesia  upon  the  Body  after  Lesions  in  the  Various 
Segments  of  the  Spinal  Cord. 

The  segments  of  the  cord  are  numbered  :  C  1  to  VIII,  D  I  to  XII,  L  i  to  V,  S  I  to  5, 
and  these  numbers  are  placed  on  the  region  of  the  skin  supplied  by  the  sensory  nerves  of 
the  corresponding  segment. 


CHAPTER  IV. 

THE   SPINAL  CORD. 

THE    DIAGNOSIS    AND    LOCALIZATION    OF    SPINAL-CORD 

DISEASES. 

The  spinal  cord  is  a  long  cylindrical  organ  extending  from  the 
medulla  oblongata  at  the  base  of  the  brain  downward  through  the 
vertebrae  to  the  level  of  the  second  lumbar  spine.  It  is  surrounded 
by  two  membranes  —  the  pia  mater,  which  contains  its  numerous 
bloodvessels,  and  the  dura  mater,  which  acts  as  a  thick  protecting 
sheath,  and  which  lies  against  the  bony  walls  without  being  closely 
adherent  to  them.  From  the  sides  of  the  spinal  cord  the  spinal  nerves 
come  out  in  pairs,  each  nerve  having  two  roots — an  anterior  root, 
which  issues  from  the  antero-lateral  groove,  and  a  posterior  root, 
which  enters  the  postero-lateral  groove.  On  each  posterior  root  lies 
an  oval  body — the  posterior  spinal  ganglion  —  which  contains  the 
sensory  neurone  bodies.  The  cord  has  two  enlargements,  the  cervical 
and  lumbar,  opposite  to  the  exit  of  the  nerves  to  the  extremities.  It 
is  made  up  of  thirty-one  segments,  each  segment  consisting  of  a  mass 
of  gray  matter  connected  with  a  pair  of  spinal  nerves  which  pass  to  a 
definite  region  of  the  body ;  and  each  segment  is  joined  to  the  others 
and  also  to  the  braiu  by  means  of  nerve  tracts  running  through  the 
various  columns  which  surround  the  gray  matter  of  the  segment. 

The  spinal  cord  is  an  organ  that  has  two  distinct  functions  :  namely, 
(1)  the  function  of  controlling  directly  the  various  parts  of  the  body 
with  which  it  is  joined  by  means  of  its  pairs  of  nerves ;  and  (2)  the 
function  of  conducting  impulses  to  and  from  the  brain.  In  consider- 
ing, therefore,  the  functions  of  the  cord  and  the  symptoms  that  arise 
from  disturbance  of  these  functions,  it  is  necessary  to  understand  not 
only  the  function  of  each  spinal  segment  so  far  as  its  motor  mechan- 
isms, its  sensory  connections,  its  vasomotor  and  trophic  functions,  and 
the  distribution  of  its  particular  pair  of  spinal  nerves,  are  concerned, 
but  also  the  functions  of  the  columns  of  the  cord  that  pass  through 
the  particular  segment  concerned,  and  that  transmit  motor  impulses 
downward  from  the  brain  and  sensory  impulses  upward  toward  the 
brain. 

In  this  chapter  each  symptom  that  may  present  itself  in  any  case 
of  spinal-cord  disease  will  be  considered,  and  these  symptoms,  one  by 
one,  will  be  referred  to  the  anatomical  structure  whose  function  is  im- 
paired, and  thus  it  will  be  possible  to  determine  what  the  pathological 
import  of  each  symptom  may  be.     This  will  demonstrate  how  readily 

47 


48 


THE  SPINAL  COED. 


Fig.  13. 


the  localization  of  spinal  affections 
may  be  determined,  and  from  this 
localization  and  the  combination  of 
the  symptoms  it  will  be  easily  pos- 
sible to  arrive  at  a  diagnosis  of  any 
form  of  spinal-cord  disease. 

Symptoms.  —  The  symptoms  of 
spinal-cord  disease  are  paralysis, 
changes  in  reflex  activity,  disturbance 
in  the  control  of  the  sphincters,  alter- 
ations of  gait  and  of  posture,  defects 
of  sensation,  ataxia,  pain,  and  trophic 
disorders. 

Paralysis  is  a  condition  of  weak- 
ness or  total  loss  of  power  in  a 
muscle.  It  may  be  limited  to  a 
single  muscle,  it  may  affect  a  group 
of  muscles,  or  it  may  affect  a  limb 
in  its  entire  muscular  apparatus. 
Paralysis  may  be  due  to  an  interfer- 
ence with  the  transmission  of  volun- 
tary impulses  from  the  motor  centres 
of  the  brain  to  the  motor  cells  (neu- 
rones) of  the  spinal  cord,  which  im- 
j)ulses  pass  in  the  motor  tracts  of  the 
spinal  cord  —  viz.,  in  the  lateral  pyra- 
midal and  anterior  median  columns. 
Paralysis  may  also  be  caused  by  a 
destruction  of  the  motor  neurones 
situated  in  the  anterior  gray  horns  of 
the  spinal  cord,  whose  axis-cylinder 
processes  (axones)  pass  directly  to  the 
muscles  through  the  anterior  nerve 
roots  and  the  motor  nerves  of  the 
body.  There  are,  therefore,  two  forms 
of  spinal  paralysis,  quite  clearly  dis- 


Diagram  of  the  spinal  cord,  showing  the  motor 
mechanisms.  P»/,  lateral  pyramidal  column ;  A3f, 
anterior  median  column  transmitting  voluntary  im- 
pulses from  the  right  pyramid  of  the  medulla  to  the 
motor  cells  of  the  anterior  horns  of  the  cord,  whence 
motor  nerves  issue  in  the  motor  root  {m)  ;  S,  sensory 
nerve  sending  its  fibres  into  the  posterior  horn  and 
into  the  root  zone  of  the  column  of  Burdach  (B), 
whence  fibres  pass  forward  to  reach  the  commissural 
cells  (c)  and  the  association  cells  (o),  and  the  motor 
cells  (to).  These  form  the  motor  mechanism  of  reflex 
and  automatic  acts.  L,  fibres  of  limiting  layer,  consist- 
ing of  association  neurones  between  various  levels  of 
the  cord,  being  branches  of  the  cells  («). 


PLATE   VIII 


The  Spinal  Cord,  its  Nerves  and  Arteries.     ( Adamkiewicz.) 

fi.  Basilar.      V.  Vortobral.      I'r.  Vcrtcbro-cerebellar.  Vna.  Vcrlpbro-spiniilis  anterior.     Sa.  Spinalis 

anterior.     M.  Magna-spinalis.     Ic.  V'lrf.t   ccrvifal    nerve.  Vsp.  Vertebro-.spinali.s  posterior.     Sp.  Spinalis 
posterior,     fif/.   Fourth  ventricle. 


tbt:  diagnosis  of  spinal-coed  diseases.  49 

tinct  from  one  another  according  as  the  lesion  affects  the  first  (cortico- 
spinal) or  th9»  second  (spinomuscular)  parts  of  the  motor  mechanism. 

The  diagram  (Fig.  13)  shows  these  two  parts  of  the  motor  mechan- 
ism. The  motor  neurones  of  the  brain  send  their  axones  downward 
through  the  pyramids  of  the  medulla,  where  a  partial  decussation 
takes  place,  the  majority  of  the  axones  from  one  pyramid  crossing  into 
the  opposite  lateral  pyramidal  tract  of  the  cord  (Fig.  13,  Py),  and  the 
remainder  passing  directly  into  the  anterior  median  column.  (Fig. 
13,  AM.)  As  these  axones  pass  downward  they  terminate  at  various 
levels  in  the  anterior  gray  horns  of  the  spinal  cord,  their  terminations 
taking  the  form  of  fine  brush-like  expansions  which  surround  the 
motor  neurones  of  the  cord  (see  page  24),  each  filament  coming  into 
contact  with  the  protoplasmic  processes  (dendrites)  of  those  neurones, 
but  not  being  continuous  with  those  dendrites.  As  these  motor  axones 
pass  downward  to  their  termination  they  give  off  small  fine  branches 
(collaterals)  at  right  angles  to  their  course,  and  these  collaterals  ter- 
minate in  the  same  manner  in  brushes  around  the  motor  neurones  of 
the  cord.  Thus  a  voluntary  impulse  starting  from  a  motor  neurone 
of  the  brain  may  reach  several  motor  neurones  of  the  cord.  As  the 
motor  tract  passes  downward  through  the  cord  it  becomes  smaller  and 
smaller,  but  few  fibres  remaining  in  the  anterior  median  column  below 
the  dorsal  region,  while  some  fibres  of  the  lateral  pyramidal  tract 
extend  to  the  very  last  segment  of  the  cord. 

This  motor  tract  is  much  more  highly  developed  in  those  animals 
which  make  use  of  the  digits  of  their  extremities.  Thus  in  elephants 
the  pyramidal  tract  is  small,  while  in  man  it  is  most  highly  developed 
and  large.  Hence,  it  is  evident  that  this  tract  transmits  those  volun- 
tary impulses  which  are  concerned  in  the  finely  adjusted  movements 
of  volition. 

Each  axone  in  this  tract  being  a  process  of  a  motor  neurone  of  the 
cerebral  cortex,  is  dependent  upon  that  neurone  for  its  nutrition.  If 
the  cortical  neurones  are  destroyed  or  if  the  axone  is  separated  at  any 
point  in  its  course  from  its  neurone,  it  degenerates  to  its  terminal 
brush.  Hence  a  degeneration  of  the  motor  tracts  of  the  cord  may  be 
due  to  disease  in  the  brain,  as  in  hemiplegia,  as  may  be  due  to  any 
transverse  lesion  of  the  cord  which  separates  the  axones  from  their 
neurones.  The  following  figures  (14  to  17)  of  specimens  demonstrate 
descending  degeneration  of  the  motor  tracts  in  the  cord.  Figs.  14 
and  15  show  descending  degeneration  consequent  upon  hemiplegia  of 
slight  and  of  severe  type,  the  anterior  median  column  on  the  side 
of  the  brain  lesion  and  the  lateral  pyramidal  tract  upon  the  other  side 
being  degenerated.  Figs.  16  and  17  show  descending  degeneration  in 
both  lateral  pyramidal  and  anterior  median  columns  consequent  upon 
a  transverse  myelitis  located  at  some  distance  above  the  level  from 
which  the  section  is  taken. 

Paralysis   of   the   cortico-spinal    type,   which   is    always   due   to  a 
disease  in  this  first  element  of  the  motor  mechanism,  has  the  follow- 
ing  characteristics*     The   muscles  paralyzed   are    partially  and   not 
4 


Fig.  14. 


Section  of  the  siiinal  cord,  at  the  sixth  cervical  segment,  showing  descendiug  degeneration  in  the 
left  lateral  pyramidal  and  the  right  anterior  median  columns,  after  a  small  lesion  in  the  motor  tract  of 
the  right  cerebral  hemisphere. 


Fig 


Section  of  the  spina!  cord,  at  the  fifth  cervical  segment,  showing  descending  degeneration  in  the 
left  anterior  median  and  right  lateral  pyramidal  tract,  after  extensive  lesions  in  the  left  cerebral  hemi- 
sphere.   There  is  slight  degeneration  in  the  left  pyramidal  tract  and  in  the  posterior  columns. 


Fig.  16. 


Section  of  the  spinal  cord  (somewliat  distorted)  in  lower  cervical  region,  showing  bilateral  descend- 
ing degeneration  in  both  anterior  median  and  lateral  pyramidal  tracts.  This  section  demonstrates  the 
unequal  size  of  corresponding  columns  on  the  two  sides  of  the  spinal  cord.  When  the  anterior  median 
column  is  large  in  extent  the  opposite  pyramidal  tract  is  correspondingly  small.  Integrity  of  the 
direct  cerebellar  columns  is  evident. 

Fig.  17. 


Section  of  the  spinal  cord  at  the  third  Imnbar  segment,  sliowiiig  descending  degeneration  in  botb 
lateral  pyramidal  tracts.  A  few  librcs  are  dcgonfw.ted  in 'be  a;,iterior  mc(?iau  , columns.  Atihislevc,! 
there  are  no  direct  cerebellar  columns. 


52  THE  SPINAL  COBV. 

absolutely  paralyzed.  All  the  muscles  of  the  limb  affected  are 
about  equally  involved;  the  limb  is  stiff,  its  joints  being  moved 
with  difficulty  and  slowly  on  account  of  the  rigicl  condition  of  the 
muscles.  The  muscles  are  thus  in  a  state  of  slight  tonic  contraction 
and  are  hypersensitive  to  mechanical  irritation,  so  that  tapping  a 
muscle  produces  a  quick  contraction,  and  tapping  its  tendon  causes 
a  prompt  response.  The  muscles  show  no  tendency  to  atrophy, 
though  from  disuse  they  may  gradually  become  somewhat  thin. 
They  are  never  flabby;  their  nutrition  is  good;  there  is  no  change 
in  their  electric  excitability.  In  this  form  of  paralysis  the  circu- 
lation of  the  limb  is  sometimes  impaired.  There  is  slight  blueness 
and  coldness  in  the  extremity  and  a  tendency  to  oedema.  Sensory 
disturbances  do  not  necessarily  attend  this  form  of  paralysis,  and, 
if  they  are  present,  are  an  indication  of  disease  elsewhere  than  in  the 
motor  tract. 

This  type  of  paralysis  is  seen  after  all  forms  of  cerebral  disease,  and 
then  usually  affects  the  arm  and  leg  upon  one  side  (hemiplegia).  It  is 
also  seen  in  both  lower  limbs  after  a  transverse  lesion  of  the  cord,  such 
as  may  be  produced  by  Pott's  disease,  by  transverse  myelitis,  by  soften- 
ing from  thrombosis,  by  hemorrhage,  or  by  tumors  of  the  spinal  cord. 
If  this  transverse  lesion  is  in  the  cervical  region,  the  arms  are  also  in- 
volved. It  is  also  seen  in  primary  lateral  sclerosis  and  in  syphilitic 
paraplegia,  in  which  conditions  it  is  limited  to  the  legs. 

The  second  or  spinomuscular  type  of  paralysis  has  an  entirely  dif- 
ferent set  of  characteristics.  The  muscles  affected  are  usually  entirely 
paralyzed  at  the  outset,  and  if  they  recover  at  all,  recover  slowly  and 
imperfectly.  While  all  the  muscles  of  the  limb  may  be  affected,  as  a 
rule  they  are  not ;  a  few  muscles  here  and  there  upon  the  limb  being 
greatly  paralyzed,  while  others  retain  their  power ;  or  if  all  the  mus- 
cles are  paralyzed  at  first,  some  will  recover  to  a  greater  extent  than 
others.  The  limb  is  never  stiff,  but  hangs  helpless,  yielding  to  the 
force  of  gravitation,  its  joints  being  relaxed  and  the  articular  surfaces 
no  longer  being  held  in  close  approximation  by  the  tonic  contraction 
of  the  muscles ;  hence  these  joints  are  all  more  freely  movable  than  in 
health.  The  muscles  are  relaxed  and  flabby.  They  do  not  respond 
to  mechanical  irritation  by  tapping  with  a  hammer,  and  tapping  their 
tendons  does  not  cause  contraction ;  the  so-called  tendon  reflex  is  lost. 
The  muscles  atrophy  very  soon  after  they  are  first  affected,  and  this 
atrophy  may  go  on  rapidly  until  but  little  of  the  muscle  is  left.  There 
is  an  early  appearance  of  the  reaction  of  degeneration  in  the  muscle ;  that 
is,  it  no  longer  responds  when  either  faradic  or  galvanic  excitation  is 
passed  through  its  nerve,  and  it  no  longer  responds  when  faradism  is 
applied  directly  to  it.  The  circulation  in  the  limb  is  always  impaired 
in  this  form  of  paralysis.  The  vessels  are  relaxed,  the  blood  pressure 
is  decreased,  there  is  a  slow  capillary  circulation,  the  limb  is  blue  and 
cold,  and  all  the  chemical  processes  seem  to  be  delayed ;  hence  the 
surface  temperature  is  much  colder  than  in  the  first  type  of  paralysis. 
Soon  after  the  onset  the  limb  is  likely  to  be  covered  with  ciammy  per- 


PLATE   IX 


XIII 


The  Cervical  and  Sacral  Enlargements  of  the  Spinal  Cord  in  Cross-section. 

(Starr.) 

Showing  tlie  various  neurons  in  the  gray  matter,  the  direction  of  their  axons,  and  the 
varieties  of  fibers  in  the  different  columns  of  the  cord.  Blue,  motor  neurons  ;  red,  sensory- 
neurons  ;  purple,  association  neurons  and  axons. 

I.  Ant.  median  column.  II.  Anterolateral  column.  III.  Gowers'  anterolateral  ascending  column. 
IV.  Marginal  column.  V.  Lateral  pyramidal  column.  VI.  Direct  cerebellar  column.  VII.  Lissauer's 
tract.  VIII.  Kxt.  portion  of  column  of  Burdach.  IX.  Root  zone  of  the  column  of  Burdach.  X. 
Descending  comma-shaped  bundle  of  Schultze.  XI.  Post,  co-nmissural  tract.  XII.  Column  of  GoU, 
XIII.   ScijtomarKinal  tract. 


TEF.    DIJ  GNOSIS   OF   SPINAL-COIiD   DISEASES. 


53 


spiration,  and  later  on,  when  this  is  no  longer  present,  it  is  very  diffi- 
cult to  heat  it  up  to  the  temperature  of  the  other  side.  Sensory  dis- 
turbances do  not  necessarily  attend  this  form  of  paralysis. 

This  type  of  paralysis  is  due  to  a  lesion  of  motor  cells  in  the  anterior 
gray  horns  of  the  cord  (Fig.  13,  m)  or  to  an  affection  of  the  nerve 
trunks  containing  the  axones  of  these  motor  neurones.  It  occurs  in 
infantile  spinal  paralysis  or  anterior  poliomyelitis,  acute  or  chronic  ;  in 
amyotrophic  lateral  sclerosis  ;  in  myelitis,  either  localized  in  a  few  seg- 
ments or  extensive  throughout  the  cord  ;  in  syringomyelia  when  the 
lesion  invades  the  anterior  horns ;  in  tumors  and  hemorrhages  within 
the  cord ;  and  in  softening  of  the  cord  due  to  embolism  or  thrombosis. 
This  type  is  also  present  in  neuritis. 

The  reason  for  these  characteristics  of  this  second  type  of  paralysis 
will  be  better  understood  if  the  anatomical  structure  of  the  second  part 
of  the  motor  tract  is  considered.  The  motor  neurones  of  the  cord,  as 
already  stated,  lie  in  the  anterior  part  of  the  gray  matter.  These 
motor  neurones  are  not  scattered  irregularly  through  its  gray  matter, 

Fia.  18. 


j:^ 


■y^4f^'^ , 


I  he  groups  of  cells  in  the  spiml  cord  at  the  fifth  ccrvic  il  ■-e^iiK  ut 

but  are  collected  into  groups,  the  larger  number  of  groups  lying  in  the 
cervical  and  lumbar  enlargements.  The  number  of  these  groups  varies 
greatly  in  different  segments  of  the  cord,  as  is  shown  in  Figs.  18  to 
20,  and  the  groups  have  a  varying  extent  longitudinally,  so  that  while 
some  groups  are  limited  to  a  single  segment,  others  extend  through 


54 


TEJS  SPINAL  COSD. 


several  segments.  Each  group  of  neurones  controls  a  single  muscle  or 
group  of  muscles  which  act  simultaneously  and  in  harmony.  The 
fundamental  movements  of  flexion  and  extension  of  the  larger  muscles 
of  the  limbs  are  represented  in  the  large  groups  of  the  median,  anterior, 
and  lateral  parts  of  the  anterior  horn.  The  accessory  movements  of 
the  fingers  and  toes  which  are  peculiar  to  monkeys  and  to  man  are 
represented  in  the  smaller  groups  lying  nearer  the  central  portion  of 

Fig.  19. 


The  groups  of  cells  in  the  anterior  horn  of  the  spinal  cord  at  the  seventh  cervical  segment. 

the  gray  matter.  In  Figs.  18  to  22  groups  of  cells  at  different  levels 
of  the  cord  are  shown  and  it  will  be  seen  that  they  differ  in  their  situ- 
ation and  extent  at  different  levels.  A  careful  study  of  comparative 
anatomy  and  of  the  lesions  occurring  in  anterior  poliomyelitis,  in  which 
disease  single  groups  of  cells  are  affected,  has  enabled  us  to  determine 
the  exact  level  in  the  cord  of  the  various  groups  of  cells  representing 
the  various  muscles  of  the  body.  These  are  shown  in  Table  I.,  which 
gives  each  segment  of  the  cervical,  lumbar,  and  sacral  regions,  with  a 
list  of  the  muscles  represented  in  each  segment.  It  will  be  noticed 
that  some  muscles  are  represented  in  two  or  even  three  segments,  while 
other  muscles  are  represented  in  but  one.  It  is  evident,  therefore, 
that  if  the  lesion  in  the  cord  is  limited  to  one  segment,  it  will  paralyze 
completely  two  or  three  muscles  which  are  represented  by  motor  neu- 
rones in  that  segment  only,  and  it  will  paralyze  partially  other  muscles 
which  aTe  represented  not  only  in  that  segment,  but  also  in  adjacent 
segments.  Hence  the  apparent  irregular  distribution  and  degree  of 
the  paralysis  in  various  muscles  upon  the  limb  in  cases  of  infantile 
paralysis. 


TTIT?  DIAGNOSIS  OF  SPINAL-COBD  DISEASES. 


55 


Table  I. — Showing  the  Muscles  Represented  in  Groups  of  Cells  in  the  Various 


/ 

Segments  of  the  Spinal  Cord. 

n.,  HL 

IV. 

V. 

VL 

VII. 

VIII. 

L 

Cervical. 

'  Cervical. 

Cervical. 

Cervical. 

Cervical. 

Cervical. 

Dorsal. 

Diaphragm. 

Diaphragm. 

Stern 0- 

Lev.  ang.  soap. 

mastoid. 

Rhomboid. 

Rhomboid. 

Trapezius. 

Supra-  and 

Supra-  and 

Scalenus. 

infraspin. 
Deltoid. 
Supin.  long. 
Biceps. 

infraspin. 
Deltoid. 
Supin.  long. 
Biceps. 
Supin.  brev. 
Serratus  mag. 
Pect.  (clav. ). 
Teres  minor. 

Biceps. 

Serratus  mag. 
Pect.  (clav.). 
Pronators. 
Triceps. 
Brach.  ant. 
Long  exten- 
sors of  wrist. 

Pronators. 

Triceps. 

Brach.  ant. 

Long  exten- 
sors of  wrist 
and  fingers. 

Pect.  (costal). 

Latis.  dorsi. 

Teres  major. 

Long  flexors 
of  wrist  and 
fingers. 

Long  flexors 

of  wrist  and 

fingers. 
Extensor  of 

thumb. 
Intrinsic 

muscles  of 

Extensor  of 

thumb. 
Intrinsic 

muscles  of 

hands. 

hands. 

I.    Lumbar. 

II.    Lumbar. 

III.    Lumbar. 

IV.     Lumbar. 

V.    Lumbar. 

Quadr.  iumb. 

Obliqui. 

Transversalis. 

Psoas. 

Psoas. 

Iliacus. 

Iliacus. 
Sartorius. 
Quad.  ext.  cruris. 

Quad.  ext.  cruris. 

Obturator. 

Adductores. 

Obturator. 

Adductores. 

Glutei. 

Glutei. 

Biceps  femoris. 

Semi-tend. 

Popliteus. 

I.    Sacral. 

II.    Saci-al. 

III.    Sacral. 

IV.  and  V.  Sacral. 

Biceps  femor. 
Semi-memb. 
Ext.  long.  dig. 
Gastroc. 
Tibialis  post. 

Gastroc. 

Tibialis  post. 

Tibialis  auticus. 

Peronei. 

Intrinsic  muscles  of  foot. 

Peronei. 

Intrinsic  muscles  of  foot. 

Sphincter  ani  et  vesicae. 
Perineal  muscles. 

Certain  special  forms  of  the  second  type  of  paralysis  are  quite  com- 
monly recognized.  Thus  we  have  in  anterior  poliomyelitis  the  upper- 
arm  type  of  paralysis,  in  which  the  deltoid,  biceps,  supinator  longus, 
and  muscles  about  the  shoulder-blade  are  affected  together,  the  muscles 
moving  the  wrist  and  fingers  escaping.  Reference  to  the  table  will 
show  that  tliis  form  is  due  to  a  lesion  in  the  upper  part  of  the  cervical 
enlargement.     We  also  have  a  lower-arm  type  of  paralysis,  in  which 


56 


THE  SPINAL  COED. 


the  muscles  wliicli  move  the  fingers  and  wrist  are  alone  invaded,  the 
supinator  lougus,  which  lies  among  them,  escaping  entirely.  Refer- 
ence to  tlie  table  will  show  that  this  is  due  to  a  lesion  of  the  lower  cer- 
vical enlargement.     The  same  distinction  can  be  made  in  paralysis  of 


w 


Fig.  20. 


The  groups  of  cells  in  the  third  liuubar  segment  of  the  spinal  cord. 

the  leg,  there  being  a  thigh  type,  a  leg  type,  and  a  foot  type,  according 
as  the  lesion  is  in  the  upper  lumbar,  midlumbar,  and  sacral  segments. 
By  reference  to  the  table,  therefore,  it  will  be  possible  in  any  case  of 
paralysis  of  the  second  type  to  arrive  at  a  localization  of  the  lesion  or  at 
an  estimate  of  its  extent  in  the  cord  when  the  muscles  paralyzed  are 
exactly  determined.  The  electrical  examination  will  assist  in  this  de- 
termination, inasmuch  as  the  muscles  whose  groups  of  cells  are  de- 
stroyed will  have  lost  their  faradic  excitability,  while  the  muscles 
whose  groups  of  cells  are  intact  will  show  no  change  in  electrical 
reactions. 

That  the  motor  neurones  of  the  cord  have  a  distinct  influence  upon 
the  nutrition  of  the  muscle  and  upon  the  circulation  in  it,  is  shown 
by  the  rapid  atrophy  and  the  vasomotor  disturbances  in  the  muscle 
which  also  attend  this  type  of  paralysis,  and  to  which  allusion  has 
already  been  made. 

It  has  already  been  stated  that  this  type  of  paraijciis  may  be  due 


THE   DIAGNOSIS   OF   SPINAL-COED   DISEASES. 


57 


Fig.  21. 


t:    Sl^^ 


g  f.i 


\m 


XII  D 


Mi 


IV  c 


Diagram  of  the  lon'-"udinal  appearance  of  tho  cervical  and  lumbar  enlargements  of  the  spinal  cord, 
showing  the  groups  or  c  Jumns  of  cells  ;  made  up  from  drawings  of  cross-sections  of  the  cord  in  a  series. 
The  figures  show  tho  segment.     (Chr.  Jacob,  Das  Menschonhirn,  Fig.  4,  p.  7.) 


58  TEE  SPINAL  COED. 

to  a  lesion  in  the  axones  arising  from  the  motor  neurones  of  the  cord. 
It  is  to  be  remembered,  however,  that  after  their  exit  from  the  cord 
these  axones  are  divided  up  into  nerves,  being  distributed  in  various 
directions  through  the  branchial  and  lumbar  and  sacral  plexuses.  A 
diifereutial  diagnosis  can  always  be  made  between  lesions  of  the  spinal 
cord  and  lesions  of  the  nerve  trunks  by  the  distribution  of  the  par- 
alysis ;  for  the  muscles  which  are  paralyzed  together  from  a  lesion  of 
a  single  nerve  are  different  from  the  muscles  paralyzed  together  from 
a  lesion  of  the  spinal  cord.  Thus  the  deltoid  is  often  paralyzed  from 
a  lesion  of  the  circumflex  nerve  alone,  but  is  never  paralyzed  alone 
from  a  lesion  of  the  cord.  Thus  the  extensors  of  the  wrist,  together 
with  the  supinator  longus,  are  paralyzed  in  lesions  of  the  musculo- 
sjnral  nerve  ;  but,  as  already  stated,  these  muscles  are  never  paralyzed 
together  in  small  lesions  of  the  spinal  cord,  their  groups  of  cells  lying 
far  apart.  And  what  is  true  of  the  nerves  of  the  brachial  plexus  is 
also  true  of  the  nerves  going  to  the  lower  extremity.  Hence,  while 
the  characteristics  of  paralysis  in  lesions  of  the  spinal  cord  and  of  the 
motor  nerve  trunks  are  the  same,  the  association  of  paralyzed  muscles 
with  one  another  in  the  two  conditions  differs.  A  further  point  of 
differentiation  is  also  found  in  the  distribution  of  anaesthesia  occurring 
with  lesions  of  the  nerve  trunks,  anaesthesia  being  frequently  present 
in  lesions  of  the  nerve  trunks  and  not  necessarily  present  in  lesions  of 
the  motor  neurones  of  the  cord.  Furthermore,  when  it  is  present 
from  a  lesion  of  the  cord,  as  will  be  shown  later,  the  distribution  of 
the  anaesthesia  in  the  skin  differs  entirely  from  the  distribution  of 
anaesthesia  after  a  lesion  of  the  nerve. 

It  is  thus  possible,  from  a  study  of  paralysis  and  of  its  essential 
characteristics  and  distribution  in  any  case,  to  reach  a  diagnosis  and  to 
localize  the  lesion  and  to  differentiate  between  lesions  of  the  motor 
tracts  of  the  cord  and  of  the  motor  neurones  of  the  cord  and  of  the  nerve 
trunks. 

There  are  cases,  however,  of  general  disease  of  the  cord,  such  as  a 
general  myelitis,  involving  both  the  white  tracts  and  the  gray  matter, 
in  which  a  third  type  of  paralysis  is  observed.  This  presents  some 
of  the  characteristics  of  both  the  former  types,  and,  were  the  diagnosis 
to  rest  upon  the  examination  of  the  paralysis  alone  it  might  present 
certain  difficulties.  Fortunately,  there  are  other  symptoms  always 
present  to  guide  us  in  the  diagnosis ;  for  in  a  general  inflammation  of 
the  cord  the  changes  in  reflex  action  and  in  the  control  of  the  bladder 
and  rectum,  and  the  tendency  to  very  severe  trophic  disturbances,  are 
so  marked  as  to  leave  no  doubt  regarding  the  actual  condition.  In 
this  third  type  of  spinal  paralysis  the  paralysis  resembles  more  closely 
the  second  type  than  it  does  the  first,  there  being  the  same  total  par- 
alysis, the  same  atrophy,  and  the  same  reaction  of  degeneration  in  the 
paralyzed  muscles  ;  but  the  distribution  of  the  paralysis  is  more  exten- 
sive—  is,  in  fact,  frequently  total  in  both  legs  and  almost  complete  in 
both  arms,  and,  although  there  is  no  rigidity  of  the  limbs,  there  is 
sometimes  an  increase  in  the  reflex  activity  and  in  the  mechanical  ex- 


TEE  DIAGNOSIS  OF  SPINAL-COUD  DISEASES.  69 

citability  of  the  muscles  in  the  early  stage  though  later  they  are  lost. 
This  is  particularly  noticed  in  the  disease  amyotrophic  lateral  sclerosis 
in  which  both  the  lateral  tracts  and  the  anterior  horns  are  simultane- 
ously progressively  destroyed.^  In  this  disease  the  first  type  of  par- 
alysis is  present  in  the  legs  for  several  months,  and  the  second  type  is 
present  in  the  arms,  associated  with  an  increased  mechanical  excita- 
bility of  the  muscles,  but  finally  this  is  lost,  and  the  arms  present  the 
typical  second  type,  which  gradually  and  finally  extends  also  to  the  legs. 

In  cases  of  iujury  of  the  spinal  cord  by  fractures  and  dislocations  of 
the  vertebrae,  the  spinal  cord  may  be  seriously  bruised  or  it  may  be 
absolutely  disintegrated.  The  symptoms  of  paralysis  appear  to  differ 
somewhat  in  these  two  conditions,  as  has  been  shown  by  Thorburn  ^ 
and  by  Kocher.^  When  the  spinal  cord  is  injured,  but  not  destroyed, 
there  is  total  paralysis  below  the  level  of  the  injury,  with  a  condition 
of  rigidity  of  the  limbs  and  an  increase  of  tendon  reflexes.  There  is 
also  a  loss  of  control  of  the  bladder,  which  may  take  the  form  either 
of  retention  of  urine  or  of  spontaneous  evacuation  of  the  bladder. 
There  is  likely  to  be  some  distention  of  the  abdomen  by  gas,  due  to  a 
paralysis  of  the  intestinal  wall. 

When  the  spinal  cord  is  absolutely  divided  or  destroyed  at  any  level, 
there  is  total  paralysis  below  this  level,  the  limbs  being  completely  re- 
laxed and  not  in  a  state  of  rigidity.  The  paralysis  is  symmetrical 
upon  the  two  sides.  The  tendon  reflexes  are  absolutely  lost.  There 
is  always  a  retention  of  urine,  which  has  to  be  relieved  by  catheter ; 
there  is  tympanites  with  distention  of  the  abdomen ;  there  is  a  paral- 
ysis of  the  vasoconstrictors  leading  to  a  dilatation  of  the  subcutaneous 
veins,  and  consequently  to  an  increased  temperature,  and  to  priapism. 
There  is  an  increase  in  the  genital  reflex,  obtained  by  pinching  the 
testicle.  There  is  a  loss  of  sensibility  to  pain  and  temperature,  and 
usually  to  touch  also,  at  a  definite  level  of  the  surface,  as  shown  in 
Plate  VII.  (See  page  46.)  In  any  case  in  which  tliese  characteristics 
are  absent  after  an  injury  of  the  spine,  it  is  certain  that  the  cord  has 
not  been  completely  destroyed  at  the  level  of  the  lesion. 

Spasmodic  Contractions  of  the  Muscles  of  an  involuntary  kind  are 
associated  with  paralysis  ia  many  forms  of  spinal-cord  disease.  Like 
the  paralysis,  they  can  be  divided  into  two  categories.  When  the 
condition  present  is  spastic  paralysis  and  the  muscles  are  rigid  and  re- 
flexes exaggerated,  patients  often  notice  a  spontaneous  trembling  of 
the  entire  limb,  due  to  alternate  contraction  of  the  two  sets  of  opposing 
muscles.  This  is  usually  attended  by  an  extensor  spasm,  so  that  the 
legs  are  stiffened  and  shake  more  or  less  violently.  The  condition  is 
occasionally  so  extreme  as  to  warrant  the  terra  "  spinal  epilepsy,"  first 

'  The  view  of  Lapinsky  (Deut.  Zeitschr.  f.  Nervenh.,  July,  1904),  that  each  group  of 
spinal  cells  repreHcnts  a  functional  coordinated  action  and  not  a  special  muscle  or  group 
of  muscles,  is  contradicted  by  the  phenomena  of  infantile  spinal  paralysis. 

^  A  Contribution  to  the  Surgery  of  the  Spinal  Cord,  Philadelphia,  1893. 

^"Die  Verletzungen  der  Wirbelsaule,  zugleich  als  Beitrag  zur  Physiologie  des 
Menschlichen  Ruckenmarl<es,"  Mittheilungen  aus  den  Grenzgebieten  der  Medizin  und 
Chirurgie,  Jena,  1890,  vol.  i.,  pp.  401-4()0. 


60  THE  SPINAL  COM). 

applied  to  it  by  Brown-S6quard,  although  this  term  is  misleading  and 
should  be  discarded.  This  symptom  is  seen  in  lateral  sclerosis  from 
any  cause,  and  is  indicative  of  a  lesion  in  the  lateral  columns  of  the 
cord.  When  it  is  present  the  lesion  does  not  involve  the  spinomuscu- 
lar  element  of  the  motor  tract. 

Fibrillary  twitchings  of  the  muscles  are  much  less  painful  than 
spasm  of  the  entire  muscle,  but  give  considerable  discomfort.  Such 
fibrillary  twitchings  are  present  in  diseases  of  the  anterior  horns  of  the 
cord,  especially  in  chronic  anterior  poliomyelitis  and  in  syringomyelia. 
Individual  fibres  of  the  muscles  alternately  contract,  producing  a  little 
wave-like  movement  which  goes  on  in  the  muscle  itself.  This  does 
not  involve  a  sufficient  number  of  the  muscle  fibres  to  produce  any 
contraction  of  the  muscle  as  a  whole.  Such  fibrillary  twitchings  can 
be  elicited  by  percussion  of  the  muscle  or  by  exposure  of  the  limb  to 
cold.  They  always  indicate  a  disease  in  the  motor  cells  controlling 
the  muscle,  as  they  are  absent  in  all  forms  of  muscular  dystrophy  in 
which  the  disease  is  exclusively  a  muscular  affection  ;  hence  such  fibril- 
lary twitchings  are  always  associated  with  the  second  type  of  paralysis. 
They  occasionally  accompany  the  third  type  of  paralysis,  in  which  the 
motor  cells  are  also  affected. 

Disturbance  of  Reflex  Action  is  a  symptom  of  great  importance  in 
spinal-cord  diseases,  the  increase  in  reflex  activity  or  the  suspension 
of  reflex  activity  being  both  of  great  significance.  The  diagram  (page 
48)  shows  the  anatomical  basis  of  a  simple  reflex  act.  Whatever 
theory  may  be  held  with  regard  to  the  exact  nature  of  the  tendon  re- 
flexes, whether  they  are  due  to  pure  mechanical  irritation  of  the  fibres 
of  the  muscle,  whose  tone  is  maintained  by  spinal  impulses,  or  whether 
they  are  due  to  a  transmission  of  impulses  through  the  spinal  cord,  the 
facts  here  stated  hold  true. 

A  reflex  act  is  the  immediate  result  of  a  sensory  impression  received 
in  the  spinal  cord,  and  it  takes  place  without  necessarily  producing  any 
conscious  perception  and  without  any  voluntary  guidance.  The  sen- 
sory nerve  enters  the  spinal  cord  through  the  posterior  nerve  root  and 
divides  into  two  parts,  which  separate  in  a  Y-shaped  division,  one 
branch  passing  upward  and  the  other  downward  in  the  root  zone  or 
column  of  Burdach  (see  Fig.  13,  B).  As  these  branches  pass  up  and 
down  they  give  off  at  right  angles  to  their  course  small  twigs  (collat- 
erals), and  these  collaterals,  as  well  as  the  terminal  filaments  of  the 
branches,  terminate  in  brush-like  expansions  in  the  gray  matter  of  the 
cord  at  various  levels.  Thus  a  sensory  impulse  entering  in  a  posterior 
nerve  is  distributed  to  a  considerable  extent  of  the  gray  matter  of  the 
cord.  Some  of  the  terminal  filaments  pass  forward  to  end  about  the 
motor  neurones  of  the  anterior  horn  of  the  same  side  upon  which  they 
enter  ;  others  pass  forward  and  cross  through  the  posterior  commissure 
to  terminate  about  the  motor  neurones  in  the  anterior  horn  of  the  oppo- 
site side ;  these  subserve  reflex  motor  acts.  Others  terminate  about 
the  large  cells  in  the  median  gray  matter,  which  are  the  intrinsic  or 
association  cells  of  the  cord,  and  transmit  the  impulses  to  other  levels 


TEE   DIAGNOSIS   OF   SPINAL-CORD   DISEASES.  61 

where  they  reach  other  motor  cells  (Fig.  13,  a);  others  end  about  cells 
whose  functien  is  to  control  vasomotor  and  trophic  reflex  acts.  Thus 
a  single  sensory  impulse  entering  the  cord  may  be  widely  distributed 
and  awaken  many  reflex  acts.  Impulses  entering  the  cord  through 
the  sensory  nerve,  and  thus  reaching  various  mechanisms  of  the  cord, 
set  up  an  activity  in  the  various  cells  presiding  over  these  mechanisms, 
and  hence  motor  impulses  pass  outward  to  the  muscles  or  to  the  viscera 
and  result  in  their  contraction,  and  hence  in  motion.  The  number  of 
these  reflex  acts  constantly  going  on  in  the  body  is  enormous.  In  fact, 
in  many  of  the  lower  animals  the  spinal  cord  is  so  much  more  developed 
than  the  brain,  that  it  is  evident  that  almost  the  entire  nervous  mechan- 
ism acts  without  conscious  perception  or  voluntary  control.  The 
whole  regulation  of  nutrition,  of  circulation,  "of  digestion,  of  reproduc- 
tion, and  of  excretion  is  under  the  control  of  the  spinal  cord  inde- 
pendently of  the  brain.  This  is  proven  by  the  fact  that  in  man,  when 
consciousness  is  suspended  in  sleep,  in  coma,  or  by  extensive  injuries  of 
the  brain  cortex,  as  in  paresis  and  senile  dementia,  these  vegetative  func- 
tions go  on  in  a  normal  manner.  And  it  is  well  known  that  infants 
born  with  defective  brains,  or  with  almost  no  brain  at  all,  may  live 
for  several  months. 

While  the  majority  of  these  reflex  acts  are  known  to  have  mechan- 
isms in  the  cord,  there  are  only  a  few  reflexes  that  are  recognized  and 
that  can  be  tested  in  health  and  disease.  These  reflex  acts  are,  first, 
the  tendon  reflexes ;  second,  the  skin  reflexes ;  third,  the  automatic 
functions  of  the  bladder  and  rectum. 

(a)  Tendon  reflexes  are  produced  by  tapping  the  tendon  of  a  muscle 
near  its  insertion  and  thus  producing  a  sudden  contraction  of  the 
muscle.  Thus  the  tendons  about  the  wrist  and  elbow,  the  patella 
tendon  at  the  knee,  and  the  Achilles  tendon  at  the  ankle  can  be 
excited  in  a  state  of  health.  We  now  know  the  exact  level  in  the 
spinal  cord  that  contains  the  reflex  mechanism  necessary  to  the  exist- 
ence of  these  various  reflex  acts.  lu  Table  II.  the  various  spinal 
muscular  reflexes  are  given,  the  method  of  producing  the  reflex,  and 
the  level  of  the  segment  controlling  each. 

Table  II. — Localization  of  Muscular  Reflex  Acts  in  the  Spinal  Cord. 

Reflex  acts.  Localization  in  segment. 

Pupillary  reflex  through  the  sympathetic  :  Dil-     Fourth  cervical  to  first  dorsal. 

atation  of  the  pupil  produced  by  irritation  of 

the  neck. 
Scapular  reflex  :  Irritation  of  the  skin  over  the     Fifth  cervical  to  first  dorsal. 

scapula  produces  contraction  of  the  scapular 

muscles. 
Biceps  and   supinator   longus  :    Tapping  their    Fifth  and  sixth  cervical. 

tendons  produces  flexion  of  the  forearm. 
Triceps  reflex  :   Tapping  tendon  produces  ex-     Sixth  cervical. 

tension  of  forearm. 
Scapulohumeral  reflex  :  Tapping  the  inner  lower    Seventh  cervical. 

edge  of  the  scapula  causes  adduction  of  the 

arm. 


62 


THE  SPINAL  COHD. 


Table  II.  —  Localization  of  3f oscular  Reflex,  Acts  in  the  Spinal  Cord.  —  Continued. 
Reflex  acts.  Localization  in  sej^ment. 

Tapping  extensor  tendons  at  the  wrist  causes  ex-     Sixth  to  eighth  cervical. 

tension  of  the  hand. 
Tapping  flexor  tendons  at  the  wrist  causes  flexion     Seventh  to  eightli  cervical. 

of  the  hand. 
Palmar  reflex  :  Stroking  palm  causes  closure  of     Eighth  cervical  to  first  dorsal. 

fingers  ;  finger  clonus. 
Abdominal  reflex  :   Stroking  side  of  abdomen     Ninth  to  twelfth  dorsal. 

causes  retraction. 
Genital  reflex  :   Squeezing   the   testicle   causes     First  to  third  lumbar. 

contraction  of  the  abdominal  muscles. 
Patella  tendon  :  Striking  tendon  at  knee  causes    Second  and  third  lumbar. 

extension  of  the  leg  ;  "knee-jerk.'* 
Achilles  tendon  reflex  :   Tapping  the  Achilles     First  to  third  sacral. 

tendon  causes  flexion  of  ankle. 
Foot   clonus  :    Extension    of    Achilles   tendon     First  to  third  sacral. 

causes  flexion  of  the  ankle. 
Plantar   reflex  :   Tickling  sole   of  foot  causes    First  to  tliird  sacral. 

flexion  of  the  toes. 
Babinski's  reflex  :  Scratching  sole  of  foot  causes     First  to  third  sacral. 

extension   of    great  toe  and    flexion  of    the 

others. 
Mendel's  reflex  :  Tapping  the  tendons   of   the     First  to  third  sacral. 

toes  causes  flexion  or  extension  of  the  toes. 
Gordon's  reflex  :  Deep  pressure  on  muscles  of     First  to  third  sacral. 

calf  of  leg  causes  extension  of  the  toes. 
Oppenheim's  reflex  :  Stroking  the  outer  side  of     First  to  third  sacral. 

the  leg  near  the  tibia  causes  retraction  of  the 

toes  and  contraction  of  the  tibialis  anticus. 
Spasm  of  anus  on  irritation.  Fourth  and  fifth  sacral. 

In  the  process  of  disease  these  reflex  acts  may  be  lost  or  they  may 
be  exaggerated. 

Loss  of  Tendon  Reflexes.  —  A  loss  of  the  reflex  implies  a  lesion  in 
the  reflex  mechanism,  either  in  the  sensory  nerve  which  would  neces- 
sarily result  in  a  coincident  condition  of  ansesthesia,  or  in  the  sensory 
reflex  fibres  within  the  cord,  which  might  give  rise  to  this  symptom 
alone,  or  in  a  destruction  of  the  motor  mechanism,  which  would  give 
rise  to  a  coincident  condition  of  paralysis.  If,  therefore,  by  the  dis- 
tribution of  the  anaesthesia  or  of  the  paralysis  it  is  possible  to  exclude 
in  any  case  a  lesion  of  the  nerve  trunk,  the  loss  of  reflex  necessarily 
implies  a  lesion  of  the  spinal  cord.  This  lesion  will  necessarily  be 
limited  to  the  segment  controlling  a  reflex  tested.  Therefore,  in  the 
examination  of  any  case  of  spinal  disease,  it  is  incumbent  to  try  each 
of  the  reflexes  in  turn,  and,  if  any  one  of  them  is  lost,  to  direct  partic- 
ular care  to  the  examination  of  the  other  functions  of  the  segment  of 
the  cord  in  which  the  lesion  is  indicated.  The  diseases  in  which  the 
reflex  activities  are  suspended  are  locomotor  ataxia  from  the  very 
earliest  stage,  and  also  those  cases  of  general  paresis  in  which  there  is 
an  early  complicating  sclerosis  of  the  posterior  columns  of  the  cord ; 
syringomyelia,  anterior  poliomyelitis,  general  myelitis  in  the  later 
stages,  transverse  myelitis  at  the  level  of  the  lesion,  disseminated 
sclerosis  when  the  patch  of  sclerotic  tissue  happens  to  He  in  the  reflex 


TEE  DIAGNOSIS  OF  SFINAL-COED   DISEASES.  63 

arc,  tumors  and  hemorrhage  in  the  cord  at  the  site  of  the  lesion.  It 
is  evident  t^t  a  loss  of  tendon  reflexes  is  a  common  accompaniment 
of  the  second  type  of  paralysis. 

An  Exaggeration  of  the  Tendon  Reflexes  is  also  frequently  observed 
as  a  symptom  of  spinal  disease.  This  implies  a  suspension  of  the  inhib- 
itory impulses  coming  from  the  brain  in  a  state  of  health,  which  im- 
pulses pass  downward  through  the  lateral  columns  in  the  motor  tracts. 
The  inhibition  being  removed,  the  spinal  cord  reacts  more  quickly  and 
intensely  to  sensory  impulses  coming  into  it  from  without.  In  this 
condition  a  phenomenon  known  as  clonus  may  be  elicited  in  almost  any 
one  of  the  tendons  of  the  longer  muscles.  Clonus  consists  of  a  rapidly 
repeated  series  of  contractions  in  a  muscle  set  up  by  a  sudden  over- 
extension of  the  tendon  of  that  muscle.  Thus  if  the  patient's  leg  be 
allowed  to  rest  upon  the  left  hand  of  the  examiner,  and  the  right  hand 
grasping  the  foot  presses  it  suddenly  backward,  thus  extending  the 
Achilles  tendon,  if  ankle  clonus  is  present  a  series  of  vibrations  of  the 
foot  will  be  produced,  due  to  a  repeated  contraction  of  the  muscles  of 
the  calf  of  the  leg.  A  similar  clonus  may  be  elicited  in  the  quadriceps 
femoris  by  a  forcible  pressure  downward  upon  the  patella  tendon  when 
the  limb  is  relaxed  and  extended.  A  similar  clonus  may  occasionally 
be  elicited  in  the  tendons  of  the  elbow,  of  the  wrist,  and  of  the  fingers. 

A  symptom  known  as  Babinski's  reflex  is  usually  to  be  elicited 
when  the  tendon  reflexes  are  exaggerated,  and  is  a  positive  sign  of  some 
disease  in  the  lateral  column  of  the  cord  or  in  the  motor  tract.  This 
is  a  sudden  extension  of  the  great  toe,  produced  by  scratching  the  sole 
of  the  foot,  due  to  a  contraction  of  the  extensor  muscle,  which  stands 
out  like  a  cord.  It  does  not  occur  in  hysteria,  in  which  other  reflexes 
may  be  increased ;  hence  it  is  a  valuable  diagnostic  symptom. 

Deep  pressure  of  the  calf  muscles  will  produce  extension  of  the  toes 
in  cases  where  the  tendon  reflexes  are  exaggerated.  This  has  been 
called  the  Gordon  reflex  after  its  discoverer.^ 

Stroking  hard  the  outer  side  of  the  leg  near  the  tibia  sometimes 
causes  a  contraction  of  the  tibialis  anticus  muscle  and  retraction  of  the 
toe.  This  has  been  termed  Oppenheim's  reflex.  It  can  be  found 
where  the  tendon  reflexes  are  exaggerated. 

The  existence  of  clonus,  like  that  of  exaggerated  reflexes,  is  an  indi- 
cation of  disturbance  of  function  in  the  lateral  columns  of  the  spinal 
cord,  and  is,  therefore,  commonly  associated  with  the  first  type  of 
spinal  paralysis.  It  is  also  an  early  indication  of  any  pathological 
process  which  interferes  with  the  transmission  of  motor  impulses  from 
the  brain  to  the  cord.  Thus  in  the  early  stage  of  Pott's  disease,  when 
slight  pressure  is  made  upon  the  cord  or  some  disturbance  of  the  nutri- 
tion of  the  cord  occurs  opposite  the  caries,  the  reflexes  are  commonly 
exaggerated  in  the  segments  below.  Exaggeration  of  the  reflexes  is 
present  in  lateral  sclerosis,  in  descending  degeneration  of  the  lateral 
columns  l^clow  the  level  of  the  lesion,  in  syphilitic  paraplegia,  and  in 
the  early  stage  of  acute  myelitis  when  the  pathological  process  irritates 
Vour.  Nerv,  and  Ment.  Dis.,  Feb.,  1905, 


64  TBE  SPINAL  COHD. 

the  motor  neurones  before  they  are  destroyed.  It  is  particularly 
noticeable  in  the  arms  in  amyotrophic  lateral  sclerosis  in  the  early  stage 
of  the  disease,  but  as  the  process  advances  reflexes  are  lost  in  the  arms, 
while  they  continue  to  be  exaggerated  in  the  legs.  An  increase  of 
tendon  reflexes  is  not  infrequently  observed  in  hysteria. 

While  it  is  true  that  a  compression  of  the  spinal  cord  of  slight  or 
intense  degree,  or  a  partial  destruction  of  the  spinal  cord  in  its  upper 
region,  always  produces  an  increase  of  the  tendon  reflexes  in  the  parts 
below  the  lesion,  it  has  been  observed  by  Miles  and  Bastian  that  in 
some  cases,  where,  by  injury  (fracture  or  dislocation  of  the  vertebrae), 
the  spinal  cord  has  been  entirely  divided  or  so  completely  crushed  that 
all  its  elements  have  been  destroyed,  the  tendon  reflexes  below  the 
point  of  destruction  have  been  abolished.  This  is  not  thought  to  be 
due  to  the  concussion  of  the  cord  consequent  upon  the  injury,  as  it  may 
persist  for  many  weeks.  It  has  been  ascribed  to  a  setting  up  of  ab- 
normal inhibitory  impulses  from  the  point  of  destruction  downward, 
due  to  the  irritation  of  the  lesion.  This,  however,  is  not  accepted  by 
Bastian  and  no  satisfactory  explanation  of  the  condition  can  be  oifered. 
In  any  case  of  localized  crush  of  the  cord  from  an  injury  the  absolute  loss 
of  tendon  reflexes  is  thought  to  indicate  a  total  destruction  of  the  cord, 
and  in  such  a  case  any  operation  at  the  site  of  injury  would  be  futile. 

(6)  The  skin  reflexes  are  not  as  well  understood  as  the  tendon  re- 
flexes. When  certain  areas  of  the  skin  are  slightly  stroked  or  tickled, 
there  occurs  a  slight  movement  of  the  skin  at  a  part  not  under  the 
point  of  irritation,  but  near  to  it,  which  seems  to  be  due  to  the  con- 
traction of  involuntary  muscular  fibers  lying  under  the  skin.  These 
skin  reflexes  are  best  seen  in  animals,  especially  horses  and  cows,  for 
the  sting  of  a  fly  is  capable  of  setting  up  a  little  fibrillary  twitching  in 
the  skin  of  their  bodies  at  almost  any  part.  The  following  table  (III-) 
gives  a  list  of  the  skin  reflexes  found  in  man,  the  method  of  their  pro- 
duction, and  the  level  of  the  spinal  cord  that  is  supposed  to  control 
them,  though  it  is  not  certain  that  they  are  under  the  control  of  a  direct 
spinal  mechanism : 

Table  III.  —  Localization  of  Skin  Reflexes  in  the  Spinal  Cord. 

Reflex  acts.  Localization  in  segment. 

Epigastric  reflex  :  Stroking  breast  causes  dim-     Seventh  to  ninth  dorsal. 

pling  of  the  epigastrium. 
Cremasteric  reflex  :  Stroking  inner  side  of  thigh     First  and  second  lumbar. 

causes  retraction  of  testicle. 
Gluteal  reflex  :   Stroking  buttock  causes  dim-     Foui'th  to  fifth  lumbar. 

pling  in  the  fold. 

The  skin  reflexes  are  usually  lost  in  those  diseases  in  which  the  ten- 
don reflexes  are  exaggerated.  They  are  also  lost  in  brain  diseases,  but 
never  in  hysteria.     They  are  never  exaggerated. 

The  Reflex  Mechanisms  Controlling  the  Bladder  and  Rectum  are 
complex  and  are  located  in  the  fourth  and  fifth  sacral  segments  of  the 


TBI:  DIAGNOSIS  OF  SPINAL-COED   DISEASES.  65 

spinal  cord.^  The  sensory  impulses  setting  up  this  mechanism  come 
from  the  muQOus  membrane  of  the  bladder  or  of  the  rectum,  and,  pass- 
ing inward  to  the  cord,  produce  two  separate  eifects  :  one  is  the  active 
motor  impulse  of  contraction  in  the  muscles  which  empty  these  organs  ; 
the  other  is  an  inhibitory  impulse  arresting  the  action  of  those  muscles 
which  normally  exert  a  constriction  at  the  opening  of  these  organs. 
Such  evacuation  of  their  contents  by  a  reflex  act  may  occur  without 
the  knowledge  of  the  individual  or  without  his  control  when  disease 
cuts  off  the  lower  part  of  the  cord  from  its  communication  with  the 
brain,  as  in  a  transverse  myelitis  of  the  dorsal  region.  We  then  have 
a  condition  known  as  active  incontinence,  in  which  the  organs  are 
emptied  spontaneously  at  intervals  in  a  normal  manner.  The  mech- 
anism itself,  however,  may  be  destroyed  by  any  lesion  of  the  sacral 
region  of  the  spinal  cord.  Under  these  circumstances,  the  reflex  arc 
being  broken  and  the  motor  cells  controlling  muscular  action  being 
destroyed,  the  organs  are  no  longer  evacuated  by  reflex  impulses,  and 
their  evacuation  has  to  be  attained  by  outside  aid.  In  some  indi- 
viduals a  distention  of  the  bladder  finally  overcomes  a  constrictive 
action  of  the  sphincters,  and  then  there  is  a  constant  leakage,  produc- 
ing a  passive  incontinence  of  urine,  but  the  rectum  shows  no  tendency 
to  empty  itself.  In  some  individuals  the  constrictive  action  of  the 
sphincters  seems  to  be  unusually  strong,  and  occasionally  a  distention 
will  lead  to  a  rupture  of  the  bladder  rather  than  to  its  evacuation  by 
water  pressure.  Sometimes  there  is  a  permanent  weakness  of  the 
sphincter  and  a  constant  dribbling  of  urine  without  any  distention  of 
the  bladder. 

Disturbances  in  the  action  of  the  bladder  and  rectum  constitute  the 
chief  sources  of  danger  in  spinal-cord  disease,  for  a  retention  of  urine 
is  liable  to  set  up  catarrhal  conditions,  and,  finally  cystitis,  and  the 
evacuation  by  means  of  a  catheter  is  liable  to  lead  to  the  infection  of 
the  bladder  by  germs  unless  the  catheter  is  absolutely  aseptic.  Such 
a  cystitis  when  once  set  up  very  often  leads  to  pyelitis,  nephritis,  and 
death ;  hence  the  greatest  care  is  to  be  taken  of  the  bladder  in  spinal- 
cord  disease.  The  evacuation  of  the  rectum  must  also  be  carefully 
attended  to,  even  though  this  involves  its  emptying  by  digital  manipu- 
lation, as  is  commonly  the  case.  Disturbance  in  the  action  of  the 
bladder  and  rectum  occurs  in  almost  all  forms  of  spinal-cord  disease, 
and,  therefore,  is  not  diagnostic  of  any  one  special  pathological  lesion 
or  of  its  location.  It  rarely  occurs  in  hysteria,  though  retention  is 
occasionally  present. 

The  Gait  in  Spinal  Disease.  —  In  various  forms  of  spinal-cord  dis- 
eases the  position  and  gait  assumed  involuntarily  by  the  patient  in 
walking  are  abnormal.  In  diseases  which  produce  the  first  type  of 
paralysis  —  e.  g.,  lateral  sclerosis  — the  gait  is  a  stiff  one  ;  the  hips  and 
knees  are  held  rigid  and  adducted,  so  that  in  walking  the  knees  ap- 
pear to  touch  or  even  to  overlap,  and  the  feet  are  not  lifted  from  the 
floor,  and   hence  are  dragged   or  are   pulled   forward  with  effort,  the 

'  Van  Geliuclitcn.     Le  Nevraxe,  1902,  vol.  iv.,  p.  119. 
5 


66  THE  SPINAL  COHB. 

great  toe  scraping  the  floor.  At  the  same  time  the  tendency  to  ankle 
clonus  frequently  leads  to  trepidation  of  the  entire  body  from  the 
shaking  of  the  foot.  These  patients  cannot  step  out  freely,  and  hence 
their  steps  are  very  short,  but  quite  regular  in  length.  They  rise  from 
a  chair  or  sit  down  slowly,  there  being  an  apparent  resistance  to  the 
flexion  and  extension  of  the  joints.  Their  motions  are  not  awkward 
and  can  be  guided  accurately.     This  has  been  named  the  spastic  gait. 

In  diseases  involving  the  lumbar  enlargement  of  the  cord  and  affect- 
ing the  gray  matter  alone  or  the  entire  cord,  and  producing  the  second 
type  of  paralysis,  the  gait  is  the  paralytic  gait.  As  the  muscles  grow 
weaker  the  support  of  the  joints  becomes  imperfect,  so  that  the  aid  of 
canes  and  crutches  is  needed  to  support  the  hip,  and  there  is  a  tendency 
for  the  knee  to  be  overextended  and  for  the  ankle  to  turn.  The  par- 
alysis of  the  muscles  of  the  thigh  makes  the  ordinary  lifting  of  the 
feet  from  the  floor  impossible,  and  hence  the  leg  is  dragged  along  the 
floor,  the  inner  side  of  the  foot  touching  the  floor,  and  the  limb  drag- 
ging rather  loosely  as  it  is  pulled  forward  between  the  crutches.  There 
is  none  of  the  stiffness  of  the  spastic  gait  and  none  of  the  uncertainty 
of  the  ataxic  gait.  If  the  anterior  tibial  group  of  muscles  is  paralyzed 
completely,  there  will  be  a  drop-foot,  and  if  this  precedes  paralysis  of 
the  thigh  or  is  alone  present,  then  the  patient  in  stepping  forward  lifts 
the  leg  higher  than  normally  in  order  to  avoid  stubbing  the  toe. 
This  has  been  termed  the  stepping  gait,  as  the  patient  has  the  appear- 
ance of  stepping  over  an  obstacle  in  his  way  or  of  raising  his  legs  as 
if  about  to  go  up  stairs.  Such  patients  are  soon  unable  to  rise  from  a 
chair  or  to  ascend  steps. 

In  posterior  sclerosis  (locomotor  ataxia)  the  gait  is  uncertain,  the 
steps  being  of  irregular  length,  the  patient  not  appearing  to  place  the 
foot  upon  the  ground  in  the  position  that  he  desires,  and  hence  he 
loses  his  balance  frequently  and  makes  irregular  efforts  to  preserve  it. 
He  rises  with  difficulty  from  a  chair,  spreads  his  feet  far  apart,  and 
sways  for  a  moment  after  rising,  in  order  to  get  his  balance.  As  the 
disease  advances  the  gait  becomes  more  irregular,  with  a  tendency  to 
long  steps,  to  too  great  lifting  of  the  foot  from  the  floor,  too  sudden 
and  violent  placing  of  the  foot  downward  upon  the  floor,  so  that  the 
gait  has  been  called  a  stamping  gait.  The  feet  are  uniformly  placed 
too  far  apart,  as  the  patient  seeks  a  wide  base  of  support.  The  irregu- 
larity of  the  gait  is  increased  by  an  attempt  to  walk  backward  or  to 
walk  forward  with  the  eyes  closed.  This  has  been  named  the  ataxic 
gait. 

The  Position  Assumed  in  Bed.  —  When  the  patient  is  confined  to  his 
bed  after  an  injury  of  the  spinal  cord,  such  as  occurs  from  fractures 
and  dislocations,  the  position  assumed  in  bed  differs  according  to  the 
level  of  the  injury. 

If  the  sacral  segments  are  crushed,  the  patient  is  unable  to  move  the 
feet  and  ankles,  and  hence  the  feet  lie  in  an  extended  position,  assum- 
ing the  position  of  drop-foot.  The  thighs  and  knees  can  be  moved  in 
this  condition. 


TRE  DIAGNOSIS   OF   SPINAL-COED   DISEASES.  67 

If  the  lesion  involves  the  lower  half  of  the  lumbar  enlargement,  the 
patient  lies  ^^th  the  thighs  drawn  up,  the  legs  flexed,  and  he  is  unable 
to  straighten  the  legs  voluntarily  or  to  lift  the  feet. 

If  the  lesion  involves  the  entire  lumbar  enlargement,  the  patient  lies 
with  the  thighs,  legs,  and  feet  extended,  and  cannot  move  them. 

If  the  lesion  is  in  the  dorsal  region,  the  lower  extremities  are  par- 
alyzed more  or  less  completely,  but  there  is  no  tendency  to  drop-foot, 
and  the  limbs  offer  some  resistance  to  passive  motion,  the  tendon  re- 
flexes being  exaggerated  and  the  muscles  somewhat  rigid,  unless  there 
has  been  a  total  destruction  of  the  cord,  under  which  circumstances 
the  muscles  are  relaxed  and  the  tendon  reflexes  lost. 

If  the  lesion  involves  the  first  dorsal  and  last  cervical  segments  the 
hands  will  be  in  a  position  of  main  en  griffe,  but  the  elbows  and 
shoulders  can  be  freely  moved. 

If  the  seventh  cervical  segment  is  destroyed,  the  forearms  are  par- 
tially flexed  and  he  upon  the  body  with  the  hands  pronated.  Volun- 
tary movements  of  the  wrist  are  impossible,  but  the  elbow  and  shoulder 
can  be  moved. 

If  the  lesion  lies  at  the  sixth  cervical  segment,  the  arms  are  abducted 
from  the  side,  the  forearms  are  supinated,  wrists  and  fingers  being  par- 
alyzed. 

If  the  lesion  is  at  the  fifth  segment,  the  arms  lie  extended  and  re- 
laxed at  the  side  of  the  body,  all  motion  being  impossible. 

These  forced  positions  are  assumed  because  of  the  fact  that  at  any 
level  of  the  cord  the  muscles  controlled  at  and  below  the  lesion  will  be 
paralyzed,  and  those  controlled  by  the  cells  just  above  the  lesion  will 
be  actively  contracted  by  the  state  of  irritation  into  which  their  motor 
centres  are  thrown,  or  if  two  centres  of  opposing  action  are  irritated 
together,  the  stronger  will  overcome  the  weaker,  and  the  resulting  po- 
sition, once  assumed,  cannot  be  corrected  voluntarily ;  hence  the  fixed 
position  once  assumed  is  maintained. 

Disturbances  of  Sensation  are  important  symptoms  of  spinal-cord 
disease.  Any  irritability  of  the  sensory  areas  of  the  cord  may  lead  to 
a  hypersensitive  condition  of  the  skin,  to  the  perception  of  ordinary 
impulses  as  extraordinary  ones,  and  hence  to  the  too  keen  appreciation 
of  any  sensation  which  may  be  started  up.  The  irritation  in  the  cord 
may  be  so  great  as  to  lead  to  hallucinations  of  sensation ;  that  is,  to 
the  perception  of  sensations  in  the  skin  which  are  set  up  in  the  cord 
and  do  not  really  come  from  the  skin  (similar  in  origin  to  the  tingling 
felt  in  the  little  finger  on  compressing  the  ulnar  nerve  at  the  elbow). 
Hence  in  any  disease  of  the  cord  during  the  early  stage  of  congestion, 
or  in  the  irritation  produced  by  beginning  sclerosis,  patients  commonly 
complain  of  tingling  and  numbness,  of  sensations  of  burning  or  of 
cold,  of  sensations  of  pain,  of  fulness,  of  pressure,  and  of  weight. 
These  sensations  are  referred  to  the  skin  or  to  the  limbs,  and  to  par- 
ticular parts  of  the  skin  and  limbs  that  correspond  to  the  segment  of 
the  cord  which  is  irritated.  These  sensations  are  classed  together 
under  the  term  "  parsesthesise." 


68  THi:  SPINAL  COBD. 

Any  destruction  of  the  sensory  areas  of  the  cord  will  lead  to  a  sus- 
pension of  sensations  which  are  classed  together  as  ansesthesise.  Sen- 
sations are  of  several  kinds  —  namely,  sensations  of  touch,  the  loss  of 
which  is  tactile  anaesthesia ;  sensations  of  pain,  the  loss  of  which  is 
analgesia;  sensations  of  temperature,  the  loss  of  which  is  thermo- 
anesthesia ;  and  sensations  of  muscular  sense,  the  loss  of  which  leads 
to  imperfect  coordination  or  ataxia.  As  these  sensations  reach  the 
spinal  cord  through  the  sensory  nerve  roots,  any  disease  of  these  nerve 
roots  affects  uniformly  all  forms  of  sensibility.  But  the  sensory  nerve 
roots  transmitting  these  sensations  separate  one  from  another  after 
their  entrance  into  the  cord,  and  pursue  different  courses  in  their  way 
upward  to  the  brain.  Hence  it  is  possible  for  one  form  of  sensation 
to  be  lost  while  the  others  are  preserved  in  diseases  affecting  different 
tracts  in  the  spinal  cord.  Thus  in  diseases  of  the  root  zone  of  the 
column  of  Burdach,  through  which  all  sensations  pass,  we  have  anaes- 
thesia, analgesia,  and  ataxia.  In  diseases  of  the  central  gray  matter 
of  the  cord  we  have  analgesia  and  thermo-ansesthesia  without  anaes- 
thesia or  ataxia.  In  diseases  of  the  posterior  columns  of  the  cord  we 
may  have  ataxia  alone  or  associated  with  anaesthesia.  The  course  of 
these  various  impulses  into  the  cord  and  upward  through  the  cord  is 
not  as  yet  absolutely  determined,  but  so  far  as  it  is  known  it  is  shown 
in  the  diagrams  (Figs.  22,  23). 

The  sensory  fibres  are  the  axones  of  neurones  situated  in  the  pos- 
terior spinal  ganglia,  and  if  they  are  cut  off  from  connections  with 
these  ganglia  by  any  lesion  of  the  nerve  root  or  any  lesion  within  the 
cord,  they  will  degenerate  from  the  point  of  lesion  onward.  A  study 
of  the  degenerations  following  lesions  of  the  nerve  roots  and  following 
transverse  lesions  of  the  spinal  cord  has  given  us  some  knowledge  of 
the  course  of  the  sensory  tracts. 

The  fibres  of  the  posterior  nerve  root  on  entering  the  cord  divide 
in  a  Y-shaped  manner,  one  branch  turning  downward  and  the  other 


EXPLANATION  OF  FIGS.  22  AND  23. 

Fig.  22.  — Diagram  showing  long  sensory  fibres  in  the  posterior  columns  of  the  cord  ;  S,  sensory 
nerves  whose  fibres  turn  upward  after  entering  the  root  zone.  Each  successive  nerve  root  from  below 
upward  presses  the  fibres  already  ascending  inward  and  backward,  so  that  in  the  cervical  region  the 
fibres  which  have  come  from  the  sacral  region  lie  in  the  column  of  Goll  near  to  the  posterior  septum  ; 
the  fibres  from  the  lumbar  region  lie  in  the  column  of  Goll  external  and  anterior  to  those  from  the 
sacral  region  ;  the  fibres  from  the  dorsal  region  lie  at  the  lateral  part  of  the  column  of  Goll ;  and  the 
fibres  from  the  cervical  region  lie  in  the  column  of  Burdach.  This  diagram  also  shows  association 
neurones  of  the  cord  whose  axones  are  passing  upward  and  downward  in  the  marginal  portion  of  the 
posterior  column  and  in  the  lateral  (L)  and  antero-lateral  {AL)  columns  of  the  cord,  with  thin 
collaterals. 

Fig.  23.  —  Diagram  showing  the  course  of  long  sensory  columns  in  the  spinal  cord:  S,  sensory 
nerve  roots  whose  fibres  enter  the  root  zone  and  the  gray  matter.  On  the  right  side  of  the  diagram 
these  fibres  terminate  about  the  cells  of  the  column  of  Clarke  (C),  whence  fibres  pass  into  the  right 
direct  cerebellar  column  (DC),  and  thus  upward  to  the  cerebellum.  Sensory  fibres  also  terminate 
about  the  intrinsic  cells  of  the  cord  (e),  whence  fibres  cross  to  the  opposite  side  and  ascend  in  the 
column  of  Gowers  ((?).  On  the  left  side  of  the  diagram  sensory  fibres  are  seen  to  terminate  around 
intrinsic  cells  of  the  gray  matter  (A'),  whence  fibres  cross  over  to  the  opposite  side  of  the  cord  and 
ascend  in  the  antero-lateral  column  (AL). 


THE  DIAGNOSIS  OF   SPlNAL-COttD  DISEASES.  60 

Fig.  22.  Fig.  23. 


70  TEE  SPINAL  COBD. 

upward.  The  branch  which  turns  downward  is  short.  It  descends 
in  the  portion  of  the  column  of  Burdach  lying  anterior  and  external  to 
the  root  zone,  named  the  comma-shaped  column  of  Schultze,  and  ter- 
minates by  sending  its  fibres  (collaterals)  into  the  gray  matter  of  the 
posterior  horn.  The  special  function  of  these  fibres  is  unknown.  The 
branches  that  turn  up  are  of  various  lengths,  and  some  fibres  ascend 
all  the  way  to  the  medulla ;  these  are  the  long  sensory  tracts.  The 
great  majority  of  the  branches  which  turn  upward  terminate  soon  after 
their  entrance  into  the  cord  in  the  segment  into  which  they  enter  or  in 
the  segments  just  above  it;  these  are  the  short  sensory  nerve  fibres. 
They  terminate  in  branches  around  the  cells  of  the  gray  matter  in  the 
posterior  and  anterior  horns  and  in  the  central  gray.  A  few  of  the 
fibres  also  turn  upward  in  a  small  zone  lying  near  to  their  entrance 
and  between  the  tip  of  the  posterior  horn  and  the  periphery  of  the 
cord  known  as  Lissauer's  column. 

The  course  of  the  long  sensory  tracts  is  pretty  well  known,  and  is 
well  illustrated  in  the  figures  here  given  (Figs.  22  and  23).  When  a 
single  nerve  root  is  injured  or  destroyed  the  area  of  degeneration  at  its 
point  of  entrance  into  the  cord  is  quite  extensive,  but  at  higher  levels 
in  the  cord  the  area  of  degeneration  grows  smaller,  and  at  the  junction 
of  the  cord  with  the  medulla  it  is  quite  limited  in  extent.  This  is 
well  illustrated  in  Figs.  24  to  2G,  which  show  the  area  of  degeneration 
in  a  case  of  unilateral  tumor  involving  the  second  and  third  lumbar 
nerve  roots. ^  It  will  be  seen  that  at  the  second  lumbar  segment  the 
entire  nerve-root  zone  in  the  column  of  Burdach  is  degenerated.  In 
the  mid-dorsal  region  this  degeneration  is  limited  to  a  small  strand  in 
the  column  of  Goll,  and  in  the  cervical  region  to  a  small  strand  also 
in  the  column  of  Goll  and  near  to  the  median  line.  This  case  con- 
firms the  results  of  physiological  experiments  in  which  the  posterior 
roots  have  been  divided  in  monkeys.^  Such  a  case  demonstrates  that 
of  the  large  number  of  nerve  fibres  entering  in  any  one  posterior  nerve 
root,  but  a  few  extend  all  the  way  up  to  the  medulla.  If  a  series  of 
cases  of  transverse  lesion  of  the  cord  is  studied,  the  transverse  lesion 
in  different  cases  being  situated  at  different  levels,  it  is  found  that  the 
ascending  degeneration  in  these  long  fibres  within  the  posterior  col- 
umns of  the  cord  varies  in  extent  in  different  cases.  The  higher  the 
transverse  lesion  the  larger  the  area  of  degeneration  produced.  This 
is  demonstrated  in  Figs.  27  and  28,  showing  the  area  of  ascending 
degeneration  following  a  lesion  of  the  lower  lumbar  region  (Fig.  27), 
as  contrasted  with  the  area  of  degeneration  following  one  in  the  upper 
dorsal  region  (Fig.  28).  A  study  of  such  cases  has  made  it  possible 
to  determine  the  relative  position  in  the  columns  of  Goll  and  Burdach 
occujjied  by  the  long  sensory  fibres  coming  up  from  the  various  seg- 
ments below.  This  is  shown  in  Fig.  22.  While  the  lesion  in  the 
posterior  root  zone  causes  an  ascending  degeneration  in  the  posterior 
columns  only,  it  has  been  found  that  a  transverse  lesion  of  the  cord 

^  Case  reported  by  Osier.     Joum.  Nerv.  and  Ment.  Disease,  1889,  p.  499. 
^  Beitnige  zur  Anatomie  des  Centralnerven-systems  insbesondere  des  Eiickenmarkes, 
von  Prof.  i)r.  J.  Singer  uud  Dr.  E.  Munzer  in  Prag,  Wien,  1890. 


TRE   DIAGNOSIS   OF  SPINAL-COEV   DISEASES. 


71 


causes  an  ascending  degeneration  in  the  direct  cerebellar  column,  the 
antero-lateral^scending  tract,  or  column  of  Gowers,  and  many  fibres 
of  shorter  or  longer  extent  in  the  column  of  Burdach  and  in  the  ajitero- 
lateral  columns.     As  degeneration  only  occurs  in  a  fibre  which  is  cut 


Fjg.  24. 


Fig.  25. 


Fig.  26. 


Sections  of  tlic  spiiiiil  conl  at  the  cervical  (Fig.  24),  dorsal  (Fig.  25),  liniil)ar  (Fig.  21'.)  levels,  show- 
ing ascending  degeneration,  unilateral,  in  the  posterior  column  after  a  guaiiua  involving  the  second 
and  third  lumbar  nerve  roots.  The  relative  extent  of  the  degenerated  fibres  at  the  different  levels  is 
shown  in  the  sections. 


off  from  its  neurone,  it  is  evident  that  the  neurones  of  which  these 
degenerated  fibres  are  branches  lie  in  the  cord  itself  and  not  in  the 
posterior  spinal  ganglia.     It  has  been  stated  already  that  the  majority 


72 


TEE  SPINAL  COBD. 


of  the  fibres  entering  a  posterior  root  zone  are  short  fibres.     These 
terminate  in  fine  brushes  about  the  cells  lying  in  the  posterior  gray 


Fig.  27. 


Ascending  degeneration  in  the  mid-dorsal  region  after  transverse  myelitis  at  the  first  lumhar  seg- 
ment. The  degeneration  affects  the  posterior  median  columns,  together  with  the  posterior  commis- 
sural tracts,  also  the  direct  cerehellar  column,  the  column  of  Gowers  and  many  fibres  in  the  antero- 
lateral columns. 


matter  and  median  gray  matter  of  the  spinal  cord.  Numerous  neu- 
rones lie  in  these  regions  of  the  gray  matter,  and  these  neurones  send 
out  their  axones  into  the  lateral  columns  of  the  cord,  where  they  turn 
upward  toward  the  medulla,  forming  the  long  and  short  sensory  col- 
umns whose  existence  is  proven  by  the  study  of  degenerations.  Figs. 
22  and  23  show  the  situation  of  these  cells  in  the  posterior  and  median 
gray  matter  of  the  cord  with  their  axones  passing  into  the  various 
columns.  In  Fig.  22  it  will  be  noticed  that  some  cells  send  their 
fibres  into  the  columns  of  the  cord  on  the  side  on  which  they  lie. 
These  are  termed  tautomere  neurones.  In  Fig.  23  it  will  be  seen  that 
some  cells  send  their  fibres  across  the  median  line  into  the  opposite  col- 
umns of  the  cord.  These  are  termed  heteromere  neurones.  In  both 
figures  the  numerous  fibres  from  the  sensory  nerves  entering  the  gray 
matter  and  terminating  around  these  neurones  are  seen.  It  is  thus 
evident  that  the  course  of  the  sensory  impulses  coming  in  through  the 


TEE  DIAGNOSIS  OF  SPINAL-COED  DISEASES. 


73 


short  sensory  nerve  fibres  is  very  complex.  And  it  is  evident  that 
while  some  ^nsory  impulses  pass  upward  on  the  same  side  on  which 
they  enter,  many  sensory  impulses  are  sent  across  the  cord  and  pass 
up  on  the  opposite  side. 

Fig.  28. 


Ascending  degeneration  at  the  first  dorsal  segment  after  transverse  lesion  of  the  fifth  dorsal  seg- 
ment. The  ascending  degeneration  affects  all  of  the  posterior  columns  excepting  the  root  zone  of  the 
column  of  Burdach.  Also  the  direct  cerehellar  columns  and  columns  of  Gowers,  and  many  fibres  in 
the  antero-lateral  columns.  A  few  degenerated  fibres  are  seen  around  the  margin  of  the  anterior 
median  column. 

It  has  been  already  stated  that  sensations  entering  the  cord  are  of 
various  kinds.  It  is  not  yet  possible  to  assign  a  special  path  to  each 
of  these  various  sensations,  but  it  seems  probable  that  the  sensations  of 
muscular  sense  pass  upward  in  the  long  fibres  of  the  posterior  columns 
and  in  the  direct  cerebellar  tract  of  the  same  side  upon  which  they 
enter  ;  that  the  sensations  of  temperature  and  of  pain  pass  in  the  short 
fibres  to  the  central  gray  matter  of  the  side  on  which  they  enter,  are 
then  taken  up  by  new  neurones  which  transmit  them  across  the  cord 
and  upward  in  the  antero-lateral  tract ;  that  the  tactile  impulses  enter 
the  posterior  columns  and  also  the  gray  matter  of  the  cord,  and  are 
taken  up  by  neurones,  some  of  which  send  their  fibres  into  the  columns 
of  the  same  side ;  but  the  majority  send  their  fibres  across  the  median 
line  into  the  antero-lateral  columns  of  the  opposite  side.  (Fig.  23,  AL.'J 
It  can  be  positively  stated  that  a  condition  of  ataxia  implies  a  lesion 
of  the  posterior  column  of  the  cord;  that  a  condition  of  analgesia  and 
thermo-analgesia  implies  a  lesion  of  the  central  gray  matter  and  of  the 
ascending  antero-lateral  fibres,  and  that  a  condition  of  tactile  anses- 


74. 


TEE  SPINAL  COBD. 


thesia  implies  widespread  degeneration  in  the  antero-lateral  and  pos- 
terior columns  of  the  cord. 

In  a  unilateral  lesion  of  the  cord  it  is  usually  found  that  there  are 
tactile  anaesthesia,  analgesia,  thermo-analgesia  in  the  side  of  the  body 
opposite  to  the  lesion,  together  with  some  hypersesthesia  of  all  these 
sensations  in  the  same  side  of  the  body  as  the  lesion  ;  and  it  is  from  this 

Fig.  29. 


Ascending  degeneration  at  the  fifth  cervical  segment  after  transverse  myelitis  at  the  eighth  dorsal 
segment.  The  columns  of  Goll,  the  post-eommissural  tracts,  the  external  part  of  the  column  of  Bur- 
dach,  the  direct  cerebellar  column,  the  column  of  Gowers,  and  some  parts  of  the  antero-lateral  column 
are  degenerated. 

clinical  fact  that  the  conclusion  is  reached  that  sensory  impulses  pass 
across  the  cord  and  ascend  in  the  columns  of  the  opposite  side. 

In  syringomyelia,  in  which  the  central  gray  matter  of  the  cord  alone 
is  aifected  there  is  a  loss  of  temperature  and  pain  sense  only.  This 
occurs  on  the  side  of  the  lesion.  It  has  been  supposed  until  recently 
that  these  sensations  were  transmitted  upward  in  the  column  of  Gowers, 
but  recent  researches  prove  that  this  column  passes  to  the  cerebellum.^ 

In  locomotor  ataxia,  in  which  the  lesion  is  limited  to  the  posterior 
root  zone,  all  the  sensations  are  more  or  less  impaired,  and  there  is  sec- 
ondary degeneration  ascending  in  the  posterior  columns  only.  In  gen- 
eral myelitis  all  the  sensory  tracts  are  implicated,  and  here  too  all  forms 
of  sensation  are  impaired. 

In  transverse  lesions  of  the  cord  at  any  segment  it  is  evident  that 
the  impulses  reaching  that  segment  from  its  own  pair  of  nerves,  and 
the  impulses  passing  through  that  segment  to  and  from  the  segments 
below  it,  will  be  cut  off;  hence  after  transverse  lesions  there  is  a  con- 
dition of  total  anaesthesia  in  the  body  below  the  segment  which  is 
destroyed.     If,  therefore,  a  series  of  cases  is  brought  together  of  lesions 

^MacNolty  and  Horsley,  Brain,  1909. 


TBE  DIAGNOSIS  OF  SPINAL-COIW   DISEASES.  75 

of  the  cord  in  every  segment  from  the  last  sacral  up  to  the  upper  cer- 
vical, and  if  die  area  of  anaesthesia  in  the  body  in  each  of  these  cases  is 
accurately  determined,  it  is  evident  that  it  will  be  possible  to  ascertain 
the  exact  region  of  the  skin  related  to  the  individual  segments  of  the 
spinal  cord.  In  Plate  YII.,  page  46,  these  areas  are .  carefully  laid 
down.  It  is  evident,  therefore,  that  in  any  case  of  spinal-cord  disease  it 
is  necessary  to  test  the  sensations  and  to  compare  the  area  of  anaesthesia 
with  the  diagram  here  given,  and  thus  to  determine  the  level  of  the 
spinal  cord  afiected.  It  is  to  be  remembered,  however,  that  the  skin 
of  the  body  is  plentifully  supplied  with  sensory  nerves  which  anasto- 
mose freely  at  their  terminations,  and  the  researches  of  Sherrington 
have  demonstrated  that  each  part  of  the  skin  is  supplied  with  sensory 
nerves  from  two  adjacent  segments  of  the  cord ;  hence  a  condition  of 
anaesthesia  in  the  skin  indicates  a  suspension  of  function  of  two  seg- 
ments of  the  cord  at  least,  for  if  one  segment  alone  were  affected  the 
segments  above  and  below  it  would  be  capable  of  supplying  the  skin 
with  sensation.  This  conclusion,  drawn  by  Sherrington  from  physio- 
logical experience,  I  have  confirmed  in  a  case  of  spinal  disease  in  which 
it  became  necessary  to  divide  completely  the  posterior  nerve  roots  at 
the  sixth  dorsal  level.  This  division  did  not  produce  any  anaesthesia 
around  the  body,  because  the  fifth  and  seventh  nerves  supplied  the 
skin  of  the  trunk  in  the  domain  of  the  sixth  nerve  sufficiently  to  pre- 
vent anaesthesia.  If,  however,  two  adjacent  nerves  are  divided,  a  zone 
of  anaesthesia  is  produced.  The  overlapping,  therefore,  of  adjacent 
sensory  areas  is  not  to  be  forgotten  in  determining  the  level  of  the 
segment  affected.  Thus  if  in  a  case  the  areas  of  anaesthesia  on  the 
body  correspond  to  the  section  shown  in  the  diagram  as  belonging  to 
the  second,  third,  fourth,  and  fifth  sacral  segments  of  the  cord,  the 
lesion  of  the  cord  undoubtedly  involves  the  first  sacral  segment  also, 
but  is  certainly  not  any  higher. 

The  determination  of  the  area  of  anaesthesia  is  of  particular  impor- 
tance in  cases  of  compression  of  the  cord  by  tumor  or  by  dislocated  or 
fractured  vertebrae,  as  it  is  the  most  positive  indication  of  the  exact 
level  of  the  cord  which  is  invaded  by  disease.  Thus  in  the  cases  in 
which  tumors  have  been  successfully  removed  from  the  cord  the  level 
of  the  anaesthesia  has  been  the  guiding  symptom  for  the  surgeon.  In 
such  cases  the  normal  anatomical  relation  between  the  segments  of  the 
spinal  cord  and  the  vertebrae  is  not  to  be  forgotten. 

Small  areas  of  anaesthesia  in  the  body  corresponding  in  distribution 
to  the  diagram,  and  due  to  small  localized  foci  of  disease  in  the  spinal 
cord,  are  found  chiefly  in  syringomyelia,  in  hemorrhages  in  the  cord, 
in  small  areas  of  softening  in  the  cord,  or  in  tumor  of  the  cord.  These 
lesions  destroy  the  terminal  filaments  of  two  or  three  sensory  nerves 
and  do  not  invade  the  long  tracts  coming  from  the  parts  below  the 
level  of  the  lesion ;  hence  localized  anaesthesia  is  indicative  of  a  small 
limited  lesion,  not  of  general  transverse  diseases.  Such  small  limited 
lesions  are  not  very  common.     In  all  cases  the  anaesthetic  area  fades 


76  TEE  SPINAL  COED. 

gradually  iuto  the  area  of  normal  sensibility.  In  hysteria  the  line  of 
demarcation  between  sensitive  and  insensitive  skin  is  much  sharper. 

The  determination  of  the  exact  area  of  anaesthesia  in  any  case  is  of 
great  service  also  in  diiferentiating  lesions  of  the  .spinal  cord  —  first, 
from  lesions  of  the  nerve  trunks ;  secondly,  from  lesions  of  the  cauda 
equina ;  thirdly,  from  hysteria ;  fourthly,  from  multiple  neuritis.  In 
lesions  of  the  nerve  trunks  the  distribution  of  the  ansesthesia  is  diiferent 
from  its  distribution  in  the  spinal-cord  affections.  This  will  be  evi- 
dent if  the  areas  in  Plate  IV.  showing  the  distribution  of  the  nerves  of 
the  skin  as  compared  with  the  areas  in  Plate  VII.  showing  the  distri- 
bution of  the  nerves  from  the  segments. 

Fractures  of  the  lower  lumbar  vertebrae  or  of  the  sacrum  often  pro- 
duce pressure  npon  the  cauda  equina.  It  has  been  found  that  when 
such  pressure  is  slight,  it  is  the  nerves  lying  innermost  within  the 
cauda  which  suffer  most,  and  the  greater  the  compression  the  greater 
the  number  of  nerves  which  suffer.  The  distribution  of  the  anaesthesia 
in  caudal  lesions  resembles  closely  the  distribution  of  anaesthesia  in 
lesions  of  segments  of  the  cord,  and  from  a  study  of  the  anaesthesia 
alone  it  is  impossible  to  differentiate  absolutely  a  caudal  lesion  from  a 
cord  lesion.  A  diagnosis  may,  however,  be  made  —  first,  from  a  study 
of  the  surgical  indications,  chiefly  of  the  nature  of  deformity,  the  rela- 
tion of  the  vertebrae  to  the  segments  of  the  cord  being  remembered. 
The  spinal  cord  ends  at  the  first  lumbar  vertebra ;  hence  any  fracture 
below  that  level  necessarily  compresses  the  cauda  equina  and  does  not 
destroy  the  spinal  cord.  Secondly,  a  diagnosis  may  be  made  from  a 
study  of  the  paralysis  which  accompanies  the  anaesthesia.  This  paral- 
ysis is  very  slight  in  lesions  of  the  sacral  segments  of  the  cord.  Thus 
when  a  lesion  is  at  or  below  the  third  sacral  segment  the  paralysis  is 
confined  to  the  peronei  muscles.  When  the  first  sacral  segment  is  also 
involved,  the  paralysis  affects  the  anterior  and  posterior  tibial  muscles, 
and  it  is  only  when  the  entire  lumbar  region  of  the  cord  is  destroyed 
that  movements  of  the  hip-joint  will  be  affected.  In  caud?-equina 
lesions,  on  the  other  hand,  pressure  upon  the  nerve  roots  is  often  suf- 
ficient to  produce  widespread  paralysis  when  sensation  is  but  slightly 
affected.  Thus  in  a  lesion  of  the  spinal  cord  the  distribution  of  the 
paralysis  will  correspond  to  the  segment  of  the  cord  invaded  by  dis- 
ease, and  will  correspond  to  the  distribution  of  the  anaesthesia  pro- 
duced by  a  lesion  of  that  segment,  while  in  lesions  of  the  cauda  equina 
the  distribution  of  the  paralysis  may  be  much  more  extensive  than  that 
indicated  by  the  distribution  of  the  anaesthesia. 

The  distribution  of  the  anaesthesia  is  also  of  much  service  in  differ- 
entiating hysterical  paralysis  from  organic  disease  of  the  spinal  cord. 
Charcot  pointed  out  the  fact  that  in  hysterical  paraplegia  the  anaes- 
thesia never  involves  the  genital  organs  or  the  perineum  and  the  anus. 
Paralysis  of  the  bladder  and  rectum  is  a  very  rare  thing,  and  if  it 
occur  is  of  the  nature  of  retention  of  urine  rather  than  of  incontinence. 
Fig.  30  shows  the  area  of  anaesthesia  commonly  observed  in  hysterical 


THE  DIAGNOSIS  OF  SPINAL-COED   DISEASES. 


77 


Fig.  30. 


cases,  and  it  will  be  noticed  that  its  distribution  is  very  different  in 
outline  froirf  that  in  Plate  VII.  It  is  also  to  be  remembered  that  in 
hysterical  paraplegia  there  is  no  reaction  of  degen- 
eration of  the  muscles,  and  there  is  no  loss  of  ten- 
don reflexes. 

In  many  cases  of  traumatic  neurosis  or  of  irri- 
tation of  the  spine  following  injuries,  and  in  the 
so-called  spinal  concussion  associated  with  anaes- 
thesia, it  will  be  found  that  the  distribution  of  the 
anaesthesia  corresponds  to  the  hysterical  rather  than 
to  the  organic  type. 

In  multiple  neuritis  the  region  of  anaesthesia 
assumes  a  stocking-shaped  or  glove-shaped  area  on 
both  limbs  symmetrically,  and  does  not  extend  to 
the  trunk. 

It  is  important  to  be  able  to  locate  the  lesion 
accurately  in  spinal-cord  disease,  no  matter  whether 
the  lesion  thus  determined  corresponds  to  well- 
known  forms  of  spinal  disease  or  not.  It  has 
recently  been  shown  ^  that  vascular  diseases  of  the 
spinal  cord,  hemorrhages  into  the  cord  of  small 
extent,  or  long  perforating  hemorrhages  in  the  cord 
of  small  lateral  extent,  or  small  areas  of  softening 
of  the  cord  due  to  thrombosis  of  diseased  spinal 
arteries  or  their  branches,  are  more  common  than 
was  supposed.  The  diagnosis  of  these  conditions  is  only  possible  by 
an  accurate  study  of  symptoms,  and  such  a  study  may  indicate  a  local 
lesion  suspending  the  function  of  a  small  area  at  any  level. 

Ataxia.  —  Ataxia  is  a  symptom  of  spinal-cord  disease  due  to  an  in- 
terference with  the  muscular  sense  impressions  which  pass  into  the 
cord  through  the  posterior  nerve  roots  and  root  zone.  Both  the  auto- 
matic and  voluntary  movements  of  the  body  are  guided  by  the  im- 
pressions received  through  muscular  sense,  and  if  those  impressions 
are  lacking  the  movements  become  awkward.  Such  movements  can 
be  guided  by  the  eye,  and  hence  an  ataxic  will  always  perform  a  mo- 
tion more  deftly  if  he  watches  the  limb  which  is  moved,  but  deprive 
him  of  vision  by  closing  his  eyes  and  the  symptom  of  ataxia  is  readily 
developed.  The  lesions  of  the  spinal  cord  which  produce  ataxia  are 
those  which  destroy  the  fibres  in  the  posterior  external  column  or 
column  of  Burdach,  through  which  the  fibres  pass  which  convey  the 
impressions  of  muscular  sense.  As  has  been  already  stated,  these 
fibres  pass  upward  and  downward  in  the  column  of  Burdach,  sending 
their  collaterals  into  the  posterior  gray  matter  of  the  cord  at  different 
levels,  so  that  the  impressions  coming  in  through  a  single  nerve  are 
conveyed  to  many  segments  of  the  cord.  In  addition  there  are  the 
long  tracts  passing  to  the  medulla,  already  described  in  the  column  of 
Goll.      When  one  thinks  of  the  very  numerous  and  complex  move- 

^R.  A.  Williamson.     Manchester  Medical  Chronicle,  1895. 


The  area  of  anaesthesia 
in  hysterical  paraplegia ; 
the  genitals  are  not  anses- 
thetic. 


78  TEE  SPINAL  COBD. 

ments  which  are  involved  in  such  a  simple  act  as  lifting  a  glass  of 
water  to  the  lips,  involving  almost  the  entire  body,  made  in  order  to 
preserve  its  balance,  one  realizes  that  to  properly  guide  any  movement, 
however  simple,  a  very  extensive  action  of  a  large  number  of  muscles 
is  required.  This  action  is  guided  automatically  by  the  muscular 
sense.  It  is  only  when  the  muscular  sense  is  interfered  with  that  one 
realizes  its  extensive  use  and  its  constant  function.  The  disease  in 
which  ataxia  is  most  evident  is  posterior  sclerosis,  but  any  lesion  affect- 
ing the  same  area  of  the  cord  will  produce  ataxia.  Thus  multiple 
sclerosis,  or  tumors  of  the  meninges  on  the  posterior  surface,  or  tumors 
within  the  cord,  or  syringomyelia  when  it  involves  the  posterior 
columns,  or  a  general  myelitis,  are  capable  of  producing  this  symp- 
tom.    The  ataxic  gait  has  already  been  described. 

Pain.  —  Pain  is  a  symptom  of  considerable  importance  in  spinal-cord 
disease.  It  may  be  felt  in  the  spine  itself;  that  is,  in  the  back  and 
deeper  structures,  under  which  circumstances,  as  a  rule,  there,  is  a 
more  or  less  extensive  affection  of  the  nerve  roots  or  of  the  meninges, 
but  not  of  the  spinal  cord.  Severe  pain  in  the  back  and  spinal  liga- 
ments is  not  at  all  uncommon  in  functional  affections,  such  as  trau- 
matic hysteria  and  traumatic  neurasthenia  ;  in  nervous  prostration  ;  in 
hysteria.  It  is  sometimes  present  in  the  affections  of  the  viscera,  under 
which  circumstance  the  pain  is  really  a  "  referred  pain,"  referred  to  the 
back  when  the  actual  irritation  comes  from  the  sympathetic  nervous 
system  ccmnected  with  the  viscus  diseased.  (See  page  751.)  In  organic 
affections  of  the  bones,  ligaments,  and  nerve  roots  spinal  pain  is  also  a 
frequent  symptom. 

When  pain  is  produced  by  disease  of  the  spinal  cord  itself,  it  is  due 
to  an  irritation  or  injury  of  the  posterior  nerve  roots  at  their  entrance 
or  to  an  irritation  of  the  sensory  tracts  passing  upward  through  the 
spinal  cord.  Under  these  circumstances  the  pain  is  not  referred  to 
the  back,  but  is  felt  in  the  part  of  the  body  from  which  the  irritated 
nerve  root  or  sensory  tract  has  come.  Thus  in  locomotor  ataxia  the 
sharp  shooting  pains  are  referred  to  the  limbs  rather  than  to  the  back, 
and  as  the  disease  almost  uniformly  begins  in  the  second  and  third 
lumbar  segments  of  the  cord,  these  pains  are  usually  referred  to  the 
anterior  surface  of  the  thighs,  and  as  the  disease  advances  downward 
to  the  fourth  and  fifth  lumbar  segments,  the  pain  is  felt  in  the  feet ;  as 
it  advances  upward  into  the  dorsal  region,  the  pain  is  felt  about  the 
body.  When  the  process  has  ascended  to  the  lower  cervical  region 
pains  begin  to  be  felt  in  the  axilla,  on  the  inner  side  of  the  arms,  and 
in  the  little  fingers,  and  finally,  as  the  upper  segments  of  the  cervical 
region  are  invaded  by  the  sclerotic  process,  the  entire  arms  and 
shoulders  become  the  seat  of  pain.  The  location  of  pain,  therefore,  in 
any  case  of  disease  of  the  cord  is  an  indication  of  the  level  of  the 
disease,  and  comparison  of  the  distribution  of  the  pain  in  any  one  case 
with  the  diagram  (Plate  VII.)  will  enable  one  to  locate  the  affection. 

In  syringomyelia,  in  which  the  terminal  filaments  of  the  pain-sense 
nerves  within  the  gray  matter  are  destroyed,  the  analgesia  which  finally 


TEE  DIAGNOSIS  OF  SPINAL-COED  DISEASES.  79 

develops  is  usually  preceded  by  a  stage  of  pain,  and  here  too,  as  in 
locomotor  aifexia,  the  pain  is  referred  to  the  periphery  corresponding 
to  the  segments  of  the  cord  involved. 

Pain  referred  to  the  extremities  is  also  a  symptom  in  injuries  of  the 
cord,  in  hemorrhages  within  the  cord,  or  in  crushing  of  the  cord  such 
as  follows  dislocation  or  fracture  of  the  spine.  In  these  cases  the  pain 
is  referred  to  the  periphery,  but  is  less  exactly  referred  than  in  loco- 
motor ataxia,  for  the  injury  aflPects  all  the  sensory  tracts  coming  from 
the  parts  below  the  site  of  the  lesion,  and  these  are  damaged  to  a 
greater  or  less  extent ;  consequently,  the  reference  of  the  pain  is  more 
widespread.  Thus  in  a  hemorrhage  in  the  lower  cervical  region  in- 
tense pain  may  be  felt  in  the  entire  body  below  the  level  of  the  arms. 
In  these  cases  any  movement  of  the  body  causing  an  increased  mechan- 
ical pressure  upon  the  cord  may  be  attended  by  sharp,  shooting  pains 
felt  in  any  part  of  the  body  below  the  lesion.  Pain  is  an  early  symp- 
tom in  tumors  of  the  cord,  and  in  these  cases  it  may  be  referred  to  the 
periphery  from  which  the  nerve  root  comes,  which  is  primarily  com- 
pressed at  the  site  of  the  tumor.  Thus  in  a  tumor  in  the  dorsal  region 
the  pain  may  be  felt  in  the  thorax,  epigastrium,  or  abdomen.  It  may 
be  also  referred  to  the  parts  of  the  body  below  the  tumor,  because  of 
the  irritation  of  the  sensory  tracts  passing  through  the  cord  at  the  site 
of  the  tumor. 

There  are  some  cases  in  which  pain  is  referred  to  an  anaesthetic  por- 
tion of  the  body.  Thus  in  caries  of  the  spine,  in  localized  meningitis, 
in  injuries  of  the  nerve  roots,  and  in  some  cases  of  sclerosis  of  the  cord 
the  sensory  nerves  may  be  destroyed,  so  that  no  sensations  can  pass  in 
over  them,  and  hence  the  surface  of  the  body  may  be  anaesthetic ;  but 
at  the  same  time  the  proximal  ends  of  these  destroyed  nerves  may  be 
irritated  by  the  disease,  and  hence  painful  sensations  may  constantly 
be  sent  inward  to  the  cord.  These,  being  received  and  transmitted 
upward,  are  referred  to  the  anaesthetic  surface,  giving  rise  to  the  symp- 
tom called  anaesthesia  dolorosa.  This  condition  is  very  rarely  seen  in 
diseases  of  the  cord  itself,  but  is  very  common  in  diseases  of  the  verte- 
brae, especially  in  tumors  (carcinoma,  sarcoma)  and  in  caries  of  the  spine. 

Vasomotor  and  Trophic  Symptoms.  —  In  the  gray  matter  of  the  cord 
there  lie  certain  cells  which  regulate  the  mechanism  by  which  nutri- 
tion in  the  body  is  maintained.  It  seems  probable  that  these  mechan- 
isms are  set  in  activity  by  sensory  impulses  reaching  the  cord  through 
the  posterior  nerves,  because  many  posterior  nerve  or  nerve-root  lesions 
are  attended  by  trophic  disturbances  —  e.  g.,  joint  disease  and  perfor- 
ating ulcer  of  the  foot  in  locomotor  ataxia.  In  locomotor  ataxia  the 
joint  diseases  are  quite  frequent,  the  ankle,  knee,  and  hip  being  affected 
in  the  order  mentioned.  In  syringomyelia  it  is  the  elbow,  wrist, 
fingers,  and  shoulder,  in  the  order  mentioned,  which  are  most  commonly 
affected.  It  is  quite  noticeable  that  such  joint  affections  occur  chiefly 
among  the  lower  classes  or  in  persons  who  are  exposed  to  injury  or  to 
falls  ;  hence  it  is  concluded  that  their  origin  is  traumatic,  even  a  slight 
injury  being  sufficient  to  produce  them.     There  is  usually  at  first  an 


80  THE  SPINAL  COED. 

effusion  of  fluid  into  the  joint,  subsequently  a  thickening  of  the  ends 
of  the  bones.  If  such  joints  are  treated  by  perfect  rest  at  an  early 
stage,  the  disease  disappears.  If,  however,  they  are  neglected,  as  they 
are  very  liable  to  be  because  of  the  absence  of  pain,  the  process  goes 
on  and  increases  until  the  joint  is  rendered  useless.  It  is  a  very  sug- 
gestive fact  that  these  joint  diseases  appear  only  in  those  spinal  affec- 
tions in  which  the  sense  of  pain  is  impaired,  and  this  is  another  proof 
of  the  conservative  influence  of  pain  in  securing  rest  or  immobility  in 
the  diseased  part. 

The  mechanisms  started  by  these  sensory  impulses  which  control 
nutrition  lie  in  the  central  gray  matter  of  the  cord,  and  to  some  extent 
in  the  anterior  horn,  for  trophic  disturbances,  especially  eruptions  in 
the  skin,  necrosis  of  the  fingers,  atrophy  of  the  muscles,  fragility  of 
the  bones,  occur  in  lesions  limited  to  the  gray  matter  of  the  central 
area  or  invading  the  anterior  horn,  as,  for  example,  in  syringomyelia 
and  in  anterior  poliomyelitis.  It  is  probable  that  these  mechanisms 
are  not  the  only  ones  which  preside  over  nutrition,  and  that  through- 
out the  body  and  in  the  skin  the  nerve  mechanism  of  the  vessels  is 
quite  competent  to  regulate  nutrition,  provided  it  is  not  put  to  too 
great  a  strain.  Thus  bed-sores  and  cystitis  are  in  my  opinion  rarely 
due  primarily  to  lesions  of  the  spinal  cord,  and  may  usually  be  avoided 
by  proper  care.  In  the  normal  body  continuous  pressure  upon  any 
one  part  for  a  long  time  without  shifting  of  position  does  not  occur 
even  in  sleep,  and  if  a  similar  repeated  change  of  posture  is  kept  up 
in  a  case  of  spinal  paralysis,  bed-sores  will  not  appear.  It  is  un- 
doubtedly the  continued  pressure  without  change  of  position  which 
produces  such  sores,  and  this  is  proven  by  the  locality  in  which  they 
uniformly  appear  —  namely,  over  the  sacrum,  upon  the  trochanters, 
upon  the  heels,  or  upon  spots  subjected  to  greatest  pressure  when  the 
patient  lies  long  in  one  position.  In  cystitis  it  is  the  introduction  of 
germs  into  the  bladder  by  means  of  the  catheter  which  is  responsible 
for  the  disease,  or  else  an  over-distention  and  consequent  inflammation 
of  the  bladder  when  the  proper  catheterization  is  neglected.  While  it 
is  admitted,  therefore,  that  the  spinal  cord  has  trophic  functions  in  the 
sense  that  it  regulates  the  degree  of  circulation  and  the  rapidity  of 
processes  of  nutrition,  it  is  not  believed  that  a  lesion  in  the  cord  alone 
under  proper  care  of  the  patient  will  result  in  lesions  of  the  skin  or 
mucous  membranes. 

The  atrophy  of  the  muscles  which  occurs  in  spinal  paralysis  appears 
to  be  too  rapid  to  be  accounted  for  by  disuse,  and  the  fragility  of  the 
bones  which  appears  in  syringomyelia  can  only  be  accounted  for  by  a 
distinct  trophic  influence  of  the  cord  upon  the  bones. 

In  all  conditions  of  destruction  of  the  spinal  cord  the  part  of  the 
body  related  to  the  part  of  the  cord  destroyed  is  found  to  present  a 
bluish  appearance,  indicating  an  imperfect  capillary  circulation  due  to 
a  dilatation  of  the  veins,  and  also  a  cold  condition  due  to  imperfect 
processes  of  nutrition  going  on  in  consequence  of  the  venous  stasis. 
This  is  particularly  noticeable  in  the  parts  of  the  body  below  a  trans- 


THE   DIAGNOSIS   OF   SPINAL-COED   DISEASES.  81 

verse  lesion  of  the  cord,  in  syringomyelia,  and  also  in  anterior  polio- 
myelitis ;  hence  it  is  evident  that  the  spinal  cord  is  concerned  in  the 
vasomotor  mechanism.  Hence  in  any  cases  where  vasomotor  or  trophic 
symptoms  appear  in  a  patient  the  suspicion  is  aroused  of  a  spinal-cord 
disease. 


CHAPTER  V. 

THE    DIAGNOSIS  AND   LOCALIZATION  OF  BRAIN  DISEASES. 

The  structure  of  the  brain  and  the  localization  of  its  functions  are 
subjects  that  have  received  much  attention  during  the  past  twenty 
years.  To  give  even  a  concise  review  of  them  would  require  too  much 
space  in  this  text-book.  Hence  the  reader  is  referred  to  the  recent 
elaborate  works  of  Gordinier/  Barker,^  Ediuger,^  Van  Gehuchten/ 
Obersteiner/  Dejerine,^  Ferrier/  the  author,^  Lewandowsky^  and 
Jacob/''  for  the  details  of  anatomy  and  physiology.  In  this  chapter  a 
study  of  the  symptoms  occurring  in  brain  diseases  will  be  presented 
and  an  attempt  will  be  made  to  trace  each  symptom  to  its  anatomical 
basis,  so  that  its  significance  as  an  index  of  the  pathological  state 
present  may  be  clear. 

The  symptoms  that  present  themselves  in  cases  of  brain  disease  are 
broadly  divided  into  two  classes,  viz.,  general  and  local  symptoms. 

The  General  Symptoms  are  headache,  dizziness,  vertigo,  discomfort, 
and  general  sensations  of  fulness,  pulsation,  and  pressure  in  the  head; 
delirium,  excitement,  depression,  stupor,  and  coma ;  disturbances  of 
sleep;  nausea  and  vomiting;  convulsions;  optic  neuritis;  variations 
in  the  rate  of  pulse,  respiration,  and  temperature;  disturbances  of 
secretion  of  sweat  and  urine ;  disturbances  in  the  vasomotor  condition, 
and  disturbances  of  nutrition. 

These  symptoms  may  occur  in  the  course  of  any  cerebral  affection 
and  indicate  an  irritation  of  the  brain  or  an  arrest  of  its  functions  as 
a  whole.  They  occur  no  matter  where  the  disease  is  located  in  the 
brain,  and  are  as  common  when  one  locality  is  affected  as  when  another 
is  invaded.  They  are  important  symptoms  of  cerebral  lesion,  but  they 
do  not  indicate  its  position.  They  are  of  great  value  in  determining 
the  variety  of  disease  which  is  present,  and  it  is  from  the  history  of 
their  development  in  any  case  that  the  diagnosis  is  to  be  made. 

The  Local  Symptoms  of  brain  disease,  on  the  other  hand,  are  pro- 

'  Gordinier,  Anatomy  of  the  Nervous  System.     P.  Blakiston's  Son  &  Co.,  1899. 
^Barker,  The  Nervous  System.     D.  Appleton  &  Co.,  1899. 
^  Edinger,  Anatomy  of  the  Central  Nervous  System.     F.  A.  Davis  Co.,  1899. 
*  Van  Gehuchten,  Le  Systeme  Nerveux  de  1' tlomme  :  Van  In,  Lierre,  1902. 
^  Obersteiner,    Anleitung   beim   Studium  des   Baues   der  Nervosen  Centralorgane. 
Vienna,  1911. 

^Dejerine,  Anatomie  des  Centres  Nerveux.     Paris,  1902. 

'  Ferrier  in  Allbutt's  System  of  Medicine,  vol.  vii.     Macmillan  Co.,  1909. 

8  Starr,  Atlas  of  Nerve  Cells.     Macmillan  Co.,  1896. 

^  Lewandowsky,  Handbk.  d.  Neurologic,  1910. 

^°  Jacob,  Chr.,  Das  Menschenhirn,  Lehmann,  1911. 

82 


LOCAL  SYMPTOMS. 


83 


duced  by  affections  of  different  parts  of  the  organ,  and  each  symptom 
shows  that  a  certain  particular  region  is  diseased.  These  symptoms 
are  spasm  or  paralysis  of  one  or  two  limbs  or  of  one-half  of  the  body  , 
loss  of  perception  of  touch,  temperature,  pain,  or  position  of  one  or  two 
limbs  or  of  one-half  of  the  body  ;  loss  of  visual  power  or  of  the  power 
of  smell,  taste,  or  hearing;  and  aphasia,  or  disturbances  in  speech. 
These  symptoms  may  be  entirely  wanting  in  some  cases  of  disease  when 
that  does  not  involve  the  portions  of  the  brain  concerned  in  sensation 


The  outer  surface  of  the  left  hemisphere.  S,  Sylvian  fissure ;  <Si,  outer  portion  of  the  Sylvian 
fissure  ;  including  Si  asc,  posterior  terminal  branch  ;  S^,  anterior  ascending  branch ;  c,  central  fissure, 
including  dr.,  inferior  transverse  sulcus ;  pci.,  inferior  precentral  sulcus ;  pes.,  superior  precentral 
sulcus ;  pcm.,  median  precentral  sulcus ;  rtc.  i.,  inferior  retrocentral  sulcus ;  rtc.  s,  superior  retro- 
icentral  sulcus;  rtc.  Ir.,  transverse  retrocentral  sulcus;  sfr.,  median  marginal  fissure;  f\,  superior 
frontal  sulcus  ;  f«,  inferior  frontal  sulcus ;  /s,  median  frontal  sulcus ;  d,  diagonal  opercular  sulcus  ; 
r,  radiating  frontal  sulcus ;  fmx,  fnno,  fm^,  parts  of  orbital  marginal  sulcus ;  ip,  interparietal  sulcus  ; 
po,  inner  perpendicular  fissure  ;  occ.  ant..,  outer  perpendicular,  or  ape  fissure ;  occ.  lat.,  lateral  occipital 
fissure;  t\,  first  temporal  sulcus,  or  parallel  fissure,  including  (i  asc,  ascending  ramus;  <2i  second 
temporal  sulcus,  including  <2  asc,  ascending  branch  ;  A,  anterior  central  convolution  ;  B,  posterior 
central  convolution  ;  Fi,  superior,  or  first  frontal  convolution ;  F2,  median  frontal  convolution,  in- 
cluding Fo  med.,  median  layer ;  Fn  lat.,  lateral  layer ;  F^,  inferior,  or  third  frontal  convolution,  in- 
cluding p.  has.,  basal  part  of  opercular  portion  ;  p.  asc,  ascending  portion  of  the  opercular  portion  ; 
p.  triang.,  triangular  portion ;  p.  orb.,  orbital  portion ;  1,  lateral  root  of  the  superior  frontal  con- 
volution ;  2,  lateral  root  of  the  median  frontal  convolution  ;  Pj,  superior  parietal  lobe ;  Po,  inferior 
parietal  lobe  ;  including  G.  spm.,  supramarginal  convolution  ;  G.  ang.,  angular  convolution ;  G.  par. 
post.,  posterior  parietal  convolution ;  O,  occipital  lobe ;  Ti,  first  temporal,  or  parallel  convolution  ; 
Ti,  second  temporal  convolution  ;  T3,  third  temporal  convolution.     (Eberstaller.) 

or  in  motion.  One  of  them  may  occur  alone  if  a  lesion  is  of  small 
extent,  or  they  may  occur  in  certain  combinations.  The  important 
fact,  however,  in  connection  with  each  symptom  is  that  its  presence 
shows  the  localization  of  the  disease  which  is  present.     From  the  man- 


84  DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

ner  of  tlie  occurrence  of  these  local  symptoms,  whether  their  onset  is 
sudden  or  gradual,  some  information  can  be  gained  which  is  an  aid  in 
the  diagnosis  of  the  nature  of  the  disease,  but  the  character  of  the  local 
symptom  is  the  same,  no  matter  how  different  the  pathological  process. 
Thus  a  hemiplegia  presents  the  same  features  whether  the  lesion  is  a 
hemorrhage,  an  abscess,  or  a  tumor ;  but  in  any  of  these  diseases  its 
appearance  indicates  that  the  motor  portion  of  the  brain  is  involved. 

Fig.  31  shows  the  appearance  of  the  left  hemisphere  of  a  highly 
organized  brain.  In  Fig.  32  a  diagram  is  given  constructed  from  a 
series  of  photographs  of  the  neurones  of  the  cortex.  This  demonstrates 
the  various  layers  of  the  cortex  and  the  different  kinds  of  cells  in  the 
different  layers.  Recent  investigations  especially  by  Campbell  and 
Jacob  have  shown  that  the  histological  appearance  of  the  cortex  differs 
widely  in  different  regions;  and  while  the  general  existence  of  five  layers 
of  cells  may  be  admitted,  the  relative  development  of  the  layers  in  dif- 
ferent regions  makes  the  appearence  of  the  cortex  different  in  different 
regions.  This  difference  of  structure  is  a  confirmation  of  the  theory  of 
difference  of  function.  (Compare  Figs.  33,  45,  and  59  on  pages  87, 
96  and  120.)  It  has  been  confirmed  by  studies  of  the  cortex  in  em- 
bryos, and  also  by  numerous  pathological  studies  in  man.  It  affords 
one  of  the  strongest  arguments  for  the  doctrine  of  localization. 

General  Symptoms.  — Headache  is  due  to  a  disturbance  of  nutrition 
of  the  brain.  It  occurs  in  a  great  variety  of  diseases,  being  usually 
more  severe  in  constitutional  affections  than  in  diseases  of  the  brain. 
In  both  functional  and  organic  diseases  of  the  brain  it  is  a  very  com- 
mon symptom,  varying  in  intensity  and  locality.  It  may  be  a  dull, 
continuous  pain,  with  intermissions  or  remissions  and  exacerbations,  as 
in  the  various  forms  of  meningitis  and  in  brain  tumor.  It  may  be 
sudden,  sharp,  and  excruciating,  but  not  long  in  duration,  as  in  great 
congestion  of  the  brain,  in  endarteritis  and  preceding  apoplexy  or  from 
the  pressure  of  a  tumor.  It  may  be  intense  for  several  hours  in  the 
afternoon  and  evening,  as  in  syphilis  of  the  membranes  or  of  the  brain. 
It  may  take  any  form  in  cerebral  neurasthenia  and  hysteria,  but  never 
resembles  the  pain  of  a  neuralgia  in  its  exact  limitation  to  a  nerve 
trunk,  or  the  pain  of  a  migraine  in  its  precise  unilateral  position  and 
in  its  regular  recurrence  in  attacks  with  long  periods  of  freedom. 
Headache  is  one  of  the  chief  and  most  severe  and  agonizing  symptoms 
in  pachymeningitis  and  in  meningitis,  being  either  local  or  diffuse,  and 
is  continuous  to  the  end.  Headache  occurs  in  mild  attacks  in  the  con- 
dition of  endarteritis  which  precedes  apoplexy,  and  is  likely  to  be  of 
frequent  recurrence  on  mental  or  physical  exertion.  It  usually  ceases 
on  the  occasion  of  an  apoplectic  attack,  and  does  not  return.  Head- 
ache rarely  develops  in  cases  of  cerebral  atrophy  and  maldevelopment ; 
hence  idiots,  imbeciles,  and  hemiplegic  children  do  not  often  suffer 
from  it.  It  is  rarely  felt  by  paretics ;  hence  is  not  a  symptom  of 
meningo-encephalitis.     Headache  is  a  marked  symptom  of  brain  abscess 


ammiL  symptoms. 


85 


and  of  brain  tumor,  in  the  former  being  of  sudden  onset  and  severe 
throughout  t^e  short  illness,  and  in  the  latter  being  of  variable  inten- 


FiG.  32. 


Diagram  of  the  layers  of  cells  of  the  cerebral  cortex.  /,  superficial  layer :  a,  fusiform  ;  6,  trian- 
gular ;  c,  polygonal  cells  of  Cajal.  //,  layer  of  small  pyramid :  d,  smallest,  e,  small,  /,  medium-sized 
pyramids,  with  their  neuraxones  descending  to  the  white  matter,  giving  off  collaterals  in  their  course. 
///,  layer  of  large  pyramids;  g,  largest  (giant)  pyramidal  cells;  k,  large  pyramidal  cell  with  very 
numerous  dendrites;  all  pyramidal  cells  are  seen  to  send  long  apical  processes  up  to  /;  m,  Martinotti 
cell  with  descending  dendrites  and  ascending  neuraxone  ;  n,  polygonal  cells.  IV,  deep  layer:  p,  fusi- 
form cell ;  q,  polygonal  cell.  V,  the  white  matter  containing  the  neuraxones  from  pyramidal  cells  d, 
e,f,  g,  and  from  cell  of  the  deep  layer  p;  r,  neuroglia  fibre.     (Starr,  Atlas  of  Nerve  Cells.) 

sity  but  of  almost  daily  recurrence  from  beginning  to  end.  Headache 
is  present  in  thrombosis  of  the  cerebral  sinuses.  It  is  a  very  marked 
and  constant  symptom  in  all  forms  of  brain  syphilis.     Headache  occurs 


86  DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

in  conditions  of  anaemia  of  the  brain,  being  usually  felt  at  the  top  or 
back  of  the  head.  In  the  persistence  of  this  symptom  and  its  associa- 
tion with  other  general  and  local  symptoms  of  brain  disease  it  is  of 
value  in  diagnosis.  In  certain  cases  headache  is  always  felt  at  one 
part  of  the  head  —  frontal,  vertical  or  occipital,  unilateral,  in  the  temple, 
or  behind  the  ear.  Sometimes  such  a  local  pain  has  been  found  to  be 
due  to  disease  at  the  site  of  pain,  viz.,  to  abscess,  tumor,  especially 
gumma,  or  to  osteitis,  or  pachymeningitis ;  but  this  is  not  uniformly 
the  case,  and  in  brain  tumors  the  location  of  the  pain  is  not  a  reliable 
indication  of  the  position  of  the  tumor.  The  disturbance  of  the  circu- 
lation and  the  venous  congestion  which  lead  either  to  an  effusion  of 
fluid  into  the  lateral  ventricles,  into  the  membranes,  or  into  a  cyst  in 
the  brain,  or  to  a  state  of  cerebral  oedema  give  rise  to  most  intense  and 
persistent  headache,  a  common  symptom  in  brain  tumor. 

Dizziness  and  Vertigo  are  common  symptoms  in  brain  disease.  The 
origin  and  varieties  of  vertigo  are  discussed  in  connection  with  disease 
of  the  auditory  nerve  and  its  relation  to  the  cerebellum.  In  diseases 
of  the  brain  vertigo  is  usually  an  intermittent  symptom,  occurring  in 
attacks.  It  may  consist  of  a  mere  sense  of  swaying,  or  of  the  turning 
of  objects  about  one,  or  it  fnay  be  so  intense  as  to  force  the  victim  to 
lie  down  and  to  hold  on  to  the  bed,  the  delusion  of  motion  being  over- 
whelming. It  leads  to  an  unsteady  gait  and  to  staggering.  It  may 
lead  in  extreme  cases  to  forced  movements  of  rotation  in  an  attempt 
to  correct  the  delusional  sense  of  movement.  It  is  a  symptom  which 
appears  in  many  cases  of  endarteritis  prior  to  apoplexy,  and  often  per- 
sists after  an  apoplectic  attack.  It  occurs  in  abscess  and  tumor  of  the 
brain,  and  in  these  cases  may  be  constant.  If  persistent  and  severe  in 
all  these  cases  it  may  indicate  disease  in  the  cerebellum ;  but,  like 
headache,  it  is  not  a  reliable  local  symptom  unless  it  is  found  in  con- 
junction with  other  local  indications.  It  occurs  in  neurasthenia  and 
hysteria  as  well  as  in  many  constitutional  conditions.  It  is  undoubtedly 
an  indication  of  a  general  disturbance  of  function  and  nutrition  in  those 
mechanisms  of  the  brain  which  preside  over  equilibrium.  These  are 
too  extensive  and  located  in  too  many  different  regions,  however,  to 
make  vertigo  a  local  sign  of  disease. 

General  Cerebral  Sensations  of  discomfort,  of  pressure  in  the  head,  of 
fulness,  or  of  irregular  pulsation  are  felt  by  many  patients  with  func- 
tional and  organic  diseases.  They  are  more  commonly  noticed  in 
neurasthenia  and  hysteria  and  in  states  of  endarteritis  that  precede 
apoplexy  than  in  states  of  brain  softening.  Anything  which  increases 
the  intracranial  pressure,  the  existence  of  a  clot,  or  abscess,  or  tumor 
is  likely  to  cause  these  sensations,  and  they  are  invariable  with  effusion 
into  the  ventricles.  They  are  to  be  considered  as  of  more  than  casual 
importance  only  when  associated  with  other  signs  of  organic  diseases. 

Mental  Symptoms  of  many  kinds  develop  in  the  course  of  brain  dis- 
ease and  are  always  due  to  a  disturbance  of  cerebral  function.  A  state 
of  mental  excitement  may  indicate  an  irritation  of  the  brain  cortex, 
and  this  may  become  delirium  when  the  power  of  voluntary  control  of 


LOCAL  SYMPTOMS. 


87 


thought  is  impaired.  This  occurs  in  abnormal  constitutional  states 
and  fever  a^d  in  meningitis  much  more  frequently  than  in  gross  lesions 
of  the  brain,  though  after  an  apoplexy,  or  in  the  course  of  an  abscess, 
or  thrombosis  of  the  lateral  sinus,  or  in  syphilis  of  the  brain,  delirium 
at  night  is  not  uncommon.  Depression  of  brain  activity,  a  sense  of 
inability  to  think  or  reason,  a  true  slowness  of  the  association  processes, 
and  an  impairment  of  judgment  and  of  self-control  are  symptoms  of 
cerebral  lesions  found  in  all  vari- 
eties of  disease,  both  diffuse  and  Fig.  33. 
localized.  The  lack  of  self-control 
manifests  itself  by  an  emotional 
state  in  which  the  patient  shows 
signs  of  amusement  or  of  distress 
on  slight  cause,  and  is  uncontrol- 
lable in  temper  and  action.  All 
these  forms  of  mental  disturbance 
may  develop  in  any  kind  of  disease 
of  the  meninges  or  of  the  brain. 
They  are  particularly  prominent  in 
cases  of  affection  of  the  frontal 
lobes,  which,  as  is  well  known,  are 
the  supposed  site  of  the  higher 
mental  faculties,  of  coSrdinated 
ideas,  of  powers  of  attention,  of 
reasoning,  of  judgment,  and  of  self- 
control.  Defects  of  development 
in  these  lobes  are  followed  by  an 
inability  to  acquire  the  higher  ideas 
which  distinguish  man  from  ani- 
mals, and  hence  lead  to  weak- 
mindedness  and  idiocy.  There- 
fore, such  mental  symptoms  may 
be  considered  as  local  symptoms  of 
frontal  lesion  in  many  cases.  They 
occur  with  diseases  within  the 
frontal  lobes  as  well  as  in  cortical 
affections,  and  also  in  lesions  of  the 
corpus  callosum,  whose  fibres  asso- 
ciate the  action  of  the  hemispheres. 
They  are  particularly  common  in 
lesions  of  the  so-called  association 

areas  of  the  cortex.  They  are  less  marked  in  local  disease  of  the 
brain  that  is  confined  to  the  motor  and  sensory  regions.  Yet  they  do 
occur  in  general  diffuse  affections,  such  as  paresis  and  multiple  cerebral 
softening,  and  hence  must  be  regarded  as  general  symptoms  rather 
than  as  purely  local.  The  various  types  of  insanity  rarely  develop  in 
cases  of  gross  cerebral  disease,  but  dementia  is  the  terminal  mental  state 
in  many  cases  of  extensive  destruction  of  brain  tissue. 


Section  through  anterior  frontal  cortex.  Con- 
trast this  with  Fig.  45,  on  page  96.  (Chr.  Jacob. 
Das  Menschenhirn.) 


88  DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

Insomnia  is  u  conimou  symptom  of  all  forms  of  intracranial  disease 
which  cause  an  irritation  of  the  brain.  It  accompanies  cerebral  conges- 
tion and  anaemia,  but  is  not  due  to  either  exclusively.  It  is  always 
present  in  meningitis.  It  often  attends  gross  lesions,  especially  those 
that  increase  the  intracranial  pressure.  After  an  apoplexy  patients 
usually  sleep  badly,  are  restless,  and  wake  easily.  Sometimes  the  sleep 
is  disturbed  by  dreams.  Insomnia  is  an  early  sign  of  paresis.  Grind- 
ing of  the  teeth  in  sleep  is  a  sign  of  irritation  of  the  base  of  the  brain. 
It  is  most  common  in  meningitis  and  in  basal  tumors.  The  opposite 
condition  of  drowsiness  is  observed  in  many  chronic  cases  of  brain 
tumor,  in  cases  of  dementia,  in  cases  of  extensive  softening,  and  in 
cases  of  paresis.  Narcolepsy  or  sleeping  sickness,  in  which  patients 
fall  asleep  at  unseemly  times  and  places,  and  cannot  be  aroused,  is  a 
functional  state  not  found  in  connection  with  gross  lesions. 

Stupor  and  Coma  develop  after  a  sudden  shock  to  the  brain,  such  as 
occurs  in  concussion  of  the  head,  or  hemorrhage  in  the  brain,  or  with 
a  rapid  alteration  of  circulation  in  the  cerebral  vessels,  as  in  the  extreme 
congestion  of  sunstroke,  or  the  anaemia  of  hemorrhage,  or  in  a  condi- 
tion of  sudden  oedema  of  the  brain  such  as  follows  thrombosis  and 
embolism.  In  these  conditions  coma  comes  on  suddenly.  It  occurs 
in  apoplexy  when  the  clot  is  large,  when  it  lies  near  the  lateral  ven- 
tricle, or  when  a  large  vessel  is  obstructed  and  a  large  area  of  the 
cortex  is  deprived  of  blood.  In  any  disease  that  leads  to  an  increase 
of  the  intracranial  pressure  stupor  and  coma  may  develop  slowly. 
Thus  in  the  various  forms  of  meningitis  and  sinus  disease,  in  enceph- 
alitis, in  brain  abscess  and  tumor,  in  syphilitic  exudations,  in  ventricu- 
lar effusions  coma  comes  on  after  the  disease  has  been  in  progress  some 
time.  Coma  usually  succeeds  a  convulsion.  Many  poisons,  either  of 
external  or  internal  origin,  are  capable  of  causing  coma,  and  hence  it 
is  not  to  be  considered  as  a  symptom  of  brain  disease  unless  associated 
with  other  signs.  It  has  no  local  significance,  but  when  it  occurs  in 
the  course  of  a  cerebral  affection  it  is  a  sign  of  great  shock  or  extensive 
disease  of  the  brain,  and  hence  is  of  bad  prognostic  importance. 

Nausea  and  Vomiting  are  only  to  be  considered  as  cerebral  in  origin 
when  all  forms  of  disturbance  of  the  stomach  and  other  organs  can  be 
excluded.  Nausea  is  a  very  rare  symptom  of  brain  disease,  but  occa- 
sionally precedes  vomiting  of  cerebral  origin.  Vomiting  is  a  sign  of 
irritation  of  the  cerebral  tissue  dependent  upon  irregularities  of  the 
circulation  or  upon  an  increase  of  intracranial  pressure.  Thus  it  occurs 
in  endarteritis,  at  the  onset  of  apoplexy,  in  the  condition  of  brain  abscess 
and  brain  tumor,  and  is  a  very  common  symptom  in  every  form  of 
meningitis.  Cerebral  vomiting  often  comes  unexpectedly,  not  being 
preceded  by  nausea  or  attended  by  retching.  It  is  due  to  a  sudden 
convulsive  contraction  of  the  stomach,  which  expels  its  contents  vio- 
lently ;  hence  it  is  called  projectile  vomiting.  It  is  more  common  in 
affections  of  the  base  of  the  brain  or  of  the  cerebellum  than  of  other 
parts,  and  is  then  probably  due  to  local  irritation  of  the  pneumogastric 
nerve.  But  it  is  not  to  be  regarded  as  a  local  symptom,  since  it  occurs 
in  diseases  in  any  locality. 


LOCAL  SYMPTOMS.  89 

Convulsions  of  a  general  kind  affecting  the  entire  body  and  attended 
by  loss  of  c^sciousness  are  either  due  to  a  toxic  agent  in  the  blood 
which  irritates  the  brain  or  to  some  source  of  irritation  affecting  the 
organ  directly.  Alcohol,  lead,  uraemia,  high  fever  in  any  disease,  and 
many  drugs  may  cause  convulsions.  Meningitis  of  any  type,  ence- 
phalitis, paresis,  abscess,  tumor  of  the  brain,  and  syphilis  in  any  form 
may  produce  them.  Epilepsy  is  characterized  by  convulsions,  the  cause 
of  which  is  unknown,  and  in  hysteria  convulsions  may  develop.  Inter- 
mittent peripheral  irritation,  frequently  repeated  and  transmitted  inward 
to  the  brain  may,  in  a  hypersensitive  organism,  cause  sufficient  cumu- 
lated central  irritation  to  produce  a  general  convulsion.  A  convulsion 
may  consist  of  a  series  of  alternate  contractions  and  relaxations  of  the 
muscles  of  the  body,  termed  clonic  convulsions,  or  may  be  a  tense  rigid 
contraction  of  many  muscles  together,  termed  a  tonic  convulsion.  In 
epilepsy  the  tonic  stage  precedes  the  clonic.  The  two  are  usually  asso- 
ciated in  a  general  convulsion,  alternately.  The  convulsion  may  begin 
with  a  cry,  which  is  merely  the  result  of  a  sudden  spasm  of  the  muscles 
of  expiration,  may  lead  to  biting  of  the  tongue  and  frothing  at  the 
mouth,  the  saliva  being  churned  up  by  combined  motions  of  jaw  and 
respiration,  may  be  accompanied  by  an  emptying  of  the  stomach,  bowels, 
and  bladder,  if  these  are  full,  and  is  usually  followed  by  a  complete 
relaxation  of  the  tired  muscles  and  a  state  of  stupor  or  coma  lasting  a 
variable  time,  in  proportion  to  the  severity  and  length  of  the  con- 
vulsion. The  convulsion  may  last  from  two  minutes  to  ten  or  even 
twenty.  It  may  be  repeated  every  few  minutes  for  hours,  or  there  may 
be  but  one  attack. 

Physiological  experiment  points  to  the  existence  of  a  centre  in  the 
pons  Varolii  the  irritation  of  which  produces  a  general  convulsion,  but 
many  clinical  facts  seem  equally  to  prove  that  irritation  of  the  cortex 
of  the  brain,  at  first  local,  may  become  general,  and  thus  cause  a  con- 
vulsion. A  convulsion  is  not  a  local  symptom,  as  disease  in  any  part 
of  the  brain  may  bring  it  on.  Gowers^  conceives  a  condition  of  the 
motor  elements  of  the  brain  as  one  quite  comparable  to  the  condition 
of  a  particle  of  dynamite  or  gun-cotton,  ready  at  any  time  to  give  a 
manifestation  of  its  stored-up  energy  by  a  sudden  discharge.  In  reflex 
acts  a  slight  irritation  is  capable  of  exciting  a  single  response.  In 
voluntary  acts  a  conscious  effort  causes  a  regulated  and  maintained 
discharge  of  energy.  In  a  convulsion  there  is  an  involuntary,  tumultu- 
ous, and  irregular  discharge  which  continues  to  the  point  of  exhaustion. 
Others  have  thought  that  a  convulsion  was  rather  an  evidence  of 
paralysis  of  a  higher  set  of  controlling  or  inhibitory  centres  whose 
constant  action  is  exerted  in  repressing  the  instinctive  tendency  of 
motor  elements  to  act.  These  hypotheses  have  much  interest  but 
little  practical  importance. 

Optic  Neuritis  is  a  general  symptom  indicating  an  increase  of  intra- 
cranial pressure.     It  is  fully  discussed  in  Chapter  XXXV.     It  may 

^The  Dynamics  of  Life.     London,  1897. 


90  DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

occur  in  any  case  of  brain  disease  that  is  attended  by  effusion  of  blood 
or  serum  in  the  membranes  or  brain,  in  ventricular  effusions,  in  states 
of  extreme  congestion,  in  brain  abscess,  and  in  brain  tumor.  It  has 
no  localizing  value  as  a  symptom,  but  is  more  commonly  found  in 
affections  of  the  base  of  the  brain,  of  the  basal  ganglia,  and  of  the 
posterior  cranial  fossa.  It  rarely  develops  suddenly.  It  may  be  well 
marked  without  causing  blindness,  but  it  usually  results  in  a  dimness 
of  vision  and,  if  extreme,  in  a  loss  of  sight. 

Variations  in  the  Pulse  may  attend  cerebral  disease.  A  slowly  in- 
creasing intracranial  pressure  at  first  causes  a  slow  pulse,  but  when  it 
is  sudden  or  extreme  the  pulse  may  be  rapid.  Thus  a  large  hemor- 
rhage causes  a  rapid  pulse,  a  tumor  causes  a  slow  pulse  during  its 
course  and  a  rapid  pulse  at  the  end.  Variations  in  the  cerebral  circu- 
lation, such  as  attend  endarteritis  and  thrombosis  or  embolism  cause, 
sometimes  a  rapid,  sometimes  a  slow,  action  of  the  heart.  Irritation 
of  the  base  of  the  brain,  such  as  occurs  in  meningitis,  always  produces 
an  irregular  and  frequently  an  intermittent  pulse. 

Variations  in  the  Rate  of  Respiration  may  be  produced  by  intracranial 
disease,  but  are  less  common  than  changes  in  pulse  rate.  A  sudden 
increase  of  intracranial  pressure,  such  as  occurs  in  hemorrhage  or 
serous  effusions  of  considerable  size,  will  produce  an  irregular  respira- 
tion consisting  of  rapid  shallow  breathing,  gradually  growing  slower, 
followed  by  an  entire  arrest  and  then  by  a  long  inspiration  again, 
followed  by  rapid  breathing.  This  is  termed  Cheyne-Stokes  respira- 
tion, and  is  a  sign  of  unfavorable  prognostic  import.  A  permanent 
slowing  or  hastening  of  respiration  has  not  been  observed  in  organic 
disease,  but  may  occur  in  hysteria. 

Variations  in  Temperature  might  be  expected  as  a  frequent  symptom, 
since  the  regulation  of  body  temperature  is  controlled  by  cerebral 
centres  which  are  located  by  Ott  in  the  basal  ganglia.  As  a  matter 
of  fact,  they  rarely  appear  in  cerebral  disease  excepting  when  they  are 
due  to  general  constitutional  disturbances.  A  sudden  fall  of  tem- 
perature is  observed  after  a  large  cerebral  hemorrhage,  and  is  succeeded 
by  a  gradual  rise  which,  in  occasional  fatal  cases,  attains  a  very  high 
degree.  Sometimes,  after  an  apoplexy  from  any  cause,  the  temperature 
on  the  paralyzed  side  becomes  one  or  two  degrees  higher  than  on  the 
other  side.  It  is  very  rare,  however,  for  a  brain  tumor  to  cause  fever, 
no  matter  what  its  location  or  the  region  of  the  brain  that  it  irritates 
or  destroys. 

The  Secretion  of  Urine  is  under  the  control  of  a  nervous  mechanism 
in  the  medulla,  and  local  lesions  in  the  vicinity  of  the  pneumogastric 
nuclei  have  been  known  to  cause  diabetes  insipidus  and  mellitus. 
Some  irritation  of  these  nuclei  may  occur  from  disease  located  else- 
where in  the  cranium.  Thus  an  apoplexy,  no  matter  what  its  cause 
or  location,  is  usually  followed  at  once  by  an  increased  excretion  of 
urine,  and  this  may  contain  albumin  and  sugar  for  a  few  days.    Brain 


LOCAL  SYMPTOMS.  91 

tumors  may  also  cause  this  symptom  from  time  to  time  in  their 
course.  IJence  polyuria  or  glycosuria  must  be  regarded  as  general 
rather  than  as  local  symptoms.  They  are  not  infrequent  symptoms 
in  hysteria,  in  which  disease  a  suspension  of  renal  activity  may  also 
occur  as  a  nervous  symptom. 

The  Vasomotor  Condition  of  the  Body  is  also  regulated  by  centres  in 
the  medulla  that  any  disease  at  any  point  in  the  brain  may  irritate  or 
may  affect.  Hence  a  unilateral  venous  congestion,  with  relaxation  of 
the  vascular  walls,  undue  sweating,  and  local  oedema  may  attend 
cerebral  hemorrhage  or  softening.  It  is  also  a  symptom  of  meningitis 
and  occasionally  of  tumor. 

Emaciation.  —  In  some  diseases  of  the  brain  patients  become  rapidly 
emaciated.  This  happens  in  many  forms  of  meningitis,  especially  in 
the  posterior  cranial  fossa,  and  in  disease  of  the  cerebellum.  I  have 
also  seen  it  in  tumors  of  the  corpus  callosum  and  of  the  frontal  lobes. 
It  appears  to  be  irrespective  of  the  amount  of  food  or  of  the  digestive 
capacity  and  without  relation  to  any  known  factor.  It  must,  there- 
fore, be  due  to  some  affection  of  those  nervous  centres  which  preside 
over  the  mechanism  of  metabolism.  Their  position  in  the  brain  is 
unknown. 

Local  Symptoms.  —  Convulsive  Movements  limited  to  a  portion  of 
the  body  and,  when  extending  to  other  parts,  following  a  definite 
order  of  progress,  are  symptoms  of  irritation  in  the  cortical  motor  area 
of  the  brain.  Such  irritation  must  originate  in  the  cortical  cells,  as 
spasms  rarely  occur  from  subcortical  lesions.  The  motor  area,  as 
recently  laid  down  by  Sherrington  and  Griinbaum  ^  on  the  brain  of  the 
chimpanzee,  is  shown  in  Fig.  34  with  its  subdivisions.  Its  anterior 
limit  does  not  coincide  with  any  fissure.  Its  posterior  limit  is  at  the 
bottom  of  the  fissure  of  Rolando.  'Its  upper  border  reaches  over  an 
inch  upon  the  mesial  surface  of  the  hemisphere.  This  localization  of 
motor  functions  corresponds  closely  with  that  determined  in  man  by 
pathological  observation.  But  in  man  the  more  highly  complex  move- 
ments of  the  hands  have  been  attended  by  the  evolution  of  motor 
centres  for  the  hands  and  fingers,  closely  connected  with  the  tactile 
centres,  and  extending  into  the  posterior  central  convolution  behind 
the  fissure  of  Rolando,  in  its  middle  third.  Fig.  35  shows  the  motor 
area  of  the  left  hemisphere  in  man,  which  is  more  extensive  than  that 
of  the  right  hemisphere  in  right-handed  persons. 

Limited  convulsive  movements  are  termed  cortical  spasms  or  Jach- 
sonian  epilepsy,  after  Hughlings  Jackson,  who  described  them  in  1861. 
Spasmodic  turning  of  the  head  and  eyes,  or  of  the  eyes  alone  to  the 
right,  indicates  irritation  of  the  posterior  part  of  the  second  frontal 
convolution  in  the  left  hemisphere.  Such  movements  to  the  left  indi- 
cate irritation  in  the  same  area  of  the  right  hemisphere.     The  head 

^  Transactions  of  the  Path.  Soc.  of  london,  Vol.  53,  page  127,  1902  ;  also  Proceed, 
ings  of  tlie  Koyal  Society,  June  11,  1903.  I  am  indebted  to  Prof.  Sherrington  for  Fig- 
34. 


92 


DIAGNOSIS  AND  LOCALISATION  OP  BnAIN  DISEASES. 


Fig.  34. 

.  Anus  and  vagina 


Abdomen 


Chest 


Shoulder 
Elbow 
Wrist 


Fingers 
and  ThuTnb~ 


Ear''    y  ,  , 

Eyelid    y  / 

/■       Closure 
Nose      of  jaw 


Opening 
of  jaw 


Vocal 
cords 


Sulcus  centralis 


Mastication 


The  left  hemisphere  of  brain  of  chimpanzee,  showing  the  motor  areas 
of  the  cortex.    (Sherrington. ) 


Fig.  35. 


The  functional  areas  of  the  cerebral  cortex.    Left  hemisphere. 


LOCAL  SYMPTOMS. 


93 


turns  in  a  series  of  jerks,  and  the  eyes  have  a  conjugate  lateral  jerky- 
movement  J-  nystagmus  —  and  these  go  on  until  the  patient  looks  over 


Fig.  r,6. 


Fig.  37. 


Situation  of  tumor  causing  spasm  of  head  and 
eyes  to  the  right ;  successfully  removed. 


Situation  of  tumor  causing  spasm  of  right  side 
of  face  and  tongue  ;  also  motor  aphasia. 


tumor  is  shown  which 


Localized  spasm  of  the  face  in  a 
patient  suffering  from  Jacksonian 
epilepsy. 


the  shoulder.     In  Fig.  36  the  situation  of  a 
caused  this  form  of  cortical  spasm  as  the  uni- 
form commencement  of  Jacksonian  epilepsy. 

Spasmodic  twitching  of  the  facial  muscles 
on  one  side  indicates  an  irritation  in  the  lower 
third  of  the  motor  area  at  the  foot  of  the  cen- 
tral convolutions.  Such  twitching  may  begin 
in  the  forehead,  or  in  closure  of  the  eye,  or 
in  the  muscles  of  the  nose,  or  about  the 
mouth,  or  in  the  chin,  or  the  cheek  may  be 
drawn  down  by  the  platysma,  or  the  tongue 
may  twitch,  or  spasmodic  noises  may  be  made 
by  a  laryngeal  spasm.  In  Fig.  35  the  vari- 
ous subdivisions  of  the  facial  area  are  shown, 
each  portion  controlling  the  muscles  named. 
Wherever,  in  the  area,  the  irritation  begins 
it  usually  spreads  to  the  entire  region,   so 

that  the  entire  side  of  the  face  is  thrown  into  a  spasm.  The  spasm  is 
clonic,  the  rate  of  movement  being  six  or  eight  contractions  to  the 
second,  and  the  spasms  may  continue  for  several  minutes.  Fig.  37 
shows  the  situation  of  a  tumor  which  caused  such  localized  convulsions 
as  the  uniform  beginning  of  a  Jacksonian  epilepsy,  and  Fig.  38  shows 
a  patient  in  the  act  of  having  such  a  spasm  of  the  face. 

Spasms  of  the  face  often  extend  to  the  eyes  and  head,  and  vice  versa, 
as  the  irritation  spreads  outward  from  either  area  to  the  adjacent  one. 
This  occurred  in  the  case  shown  in  Fig.  39,  where  the  upper  facial 
muscles  were  involved  after  the  head  and  eyes  had  turned  to  the  right. 

Convulsive  movements  limited  to  the  upper  extremity  show  an  irri- 
tation in  the  middle  third  of  the  motor  area.  The  spasm  may  begin 
with  a  drawing  upward  of  the  shoulder  and  turning  downward  of  the 
head,  as  in  the  case  shown  in  Fig.  40,  or  by  the  movements  of  the 
arm  at  the  shoulder-joint,  or  by  movements  of  the  elbow  of  flexion  £ind 


94 


DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 


extension,  or  by  movements  of  the  wrist  and  fingers  and  thumb.  Or 
the  spasm  may  begin  in  the  hand  and  ascend  the  arm,  involving  all 
the  muscles  in  the  reverse  order  named.  Usually  the  hand  is  closed, 
the  wrist  flexed  and  pronated,  the  elbow  flexed,  and  the  arm  adducted 
when  the  spasm  is  at  its  height,  and  the  limb  trembles  violently  in  the 
convulsion.     The  flexors  thus  overcome  the  extensors  in  the  conflict 


Fig.  39. 


Fig.  40. 


Situation  of  tumor  causing  cortical  spasm 
and  motor  aphasia.  Head  and  eyes  turned  to 
the  right,  then  forehead  and  cheek  were  drawn 
to  the  right. 


Situation  of  cyst  causing  spasm  of  right 
shoulder,  extending  down  the  arm  and  to  the 
fingers.    Cyst  removed. 


between  both  in  the  spasm.  Occasionally  large  movements  at  the 
joints  are  made,  and  the  hand  makes  wide  excursions,  as  in  one  case 
of  mine,  following  a  cortical  hemorrhage,  where  the  arm  was  flung 
about  like  a  flail.  It  is  important  to  notice  the  order  of  extension  of 
the  spasm  in  the  arm,  as  this  gives  a  clue  to  the  point  on  the  cortex 
where  the  irritation  begins.  The  subdivisions  of  the  motor  area  for 
the  arm  are  shown  on  Fig.  35.     In  Fig.  41  the  location  is  shown  of 


Fig.  41. 


Fig.  42. 


Situation  of  tumor  causing  spasm  of  left 
fingers  and  thumb,  then  of  wrist,  elbow,  and 
shoulder.    Tumor  removed. 


Situation  of  tumor  causing  spasm  of  the 
right  thumb,  fingers,  and  arm,  then  of  foot  and 
leg,  finally  of  face.    Tumor  removed. 


a  tumor  removed  successfully  from  the  cortex.  In  this  case  the 
patient  suffered  from  many  Jacksonian  spasms  of  the  upper  extremity 
which  always  began  with  a  twitching  of  the  thumb  and  first  finger, 
then  a  closure  of  the  hand,  and  then  extended  up  the  arm.  Spasms 
beginning  in  the  upper  extremity  may  extend  to  the  face,  or  to  the  head 
and  eyes,  or  to  the  trunk  and  leg.  Eeference  to  Fig.  35  demonstrates 
that  this  extension  merely  indicates  a  radiation  outward  of  the  initial 


LOCAL  SYMPTOMS. 


95 


irritation  to  adjacent  areas.  And  it  is  evident  that  the  order  of  exten- 
sion of  the  spasm  gives  a  clue  to  the  position  of  the  point  of  initial  irri- 
tation. Thus  in  the  case  shown  in  Fig.  40  the  spasm  extended  to 
the  face  from  the  arm.  In  case  shown  in  Fig.  42  where  the  lesion 
was  a  cortical  tumor,  the  spasm  extended  from  the  arm  to  the  leg  before 
it  reached  the  face.  When  a  spasm  first  aifects  the  shoulder,  the  trunk, 
and  head,  and  eyes  are  usually  thrown  into  convulsion  before  the  leg 
or  face.  It  is  evident  from  a  study  of  a  large  number  of  such  cases 
of  Jacksonian  epilepsy  that  the  order  of  extension  of  the  spasm  is  not 
a  matter  of  accident,  but  is  governed  by  the  spread  of  irritation  from 
one  cortical  region  to  the  adjacent  region.  One  has  to  think  of  such 
a  spread  of  irritation  as  resembling  the  little  waves  on  a  lake  which 
extend  from  the  place  where  a  stone  strikes  the  surface  in  concentric 
but  ever-widening  circles. 

Convulsive  motions  limited  to  the  trunk  are  usually  of  the  nature 
of  bending  of  the  body  toward  one  side  with  a  drawing  up  of  the  thigh. 
They  are  not  often  observed  alone,  but  commonly  occur  as  the  spasm 


Fig.  43. 


Fig.  44. 


Situp.tion  of  tumor  causing  spasm  beginning 
in  the  left  thigh,  extending  to  the  body,  arm,  and 
face.    Removal  impossible. 


Situation  of  meningeal  thickening  and  adhe- 
sion after  trauma,  causing  spasm  of  leg,  begin- 
ning in  knee,  and  extending  to  foot  and  hip. 


extends  from  the  shoulder  to  the  leg  or  from  the  leg  to  the  shoulder. 

Convulsive  motions  limited  to  the  lower  extremity  may  begin  in  the 
thigh,  as  in  the  case  shown  in  Fig.  43,  and  extend  down  the  leg, 
finally  involving  the  foot  and  toes,  or  they  may  begin  with  a  twitch- 
ing of  the  toes  which  extends  to  the  ankle  and  knee,  and  finally  to  the 
hip,  as  in  the  case  shown  in  Fig.  44.  The  lower  extremity  is  usually 
extended  in  a  Jacksonian  attack,  is  held  rigid,  and  trembles,  the 
extensors  overcoming  the  flexors  in  their  action.  When  a  spasm 
begins  in  the  leg  it  extends  to  the  arm  or  trunk  before  it  reaches  the 
face,  and  usually  involves  the  head  and  eyes  before  it  affects  the  lower 
facial  muscles  or  the  tongue. 

Local  spasms  are  always  attended  by  a  sensation  of  tingling  in  the 
part  that  is  convulsed.  Sometimes  the  tingling  sensation  precedes  the 
actual  movement  by  some  seconds  or  even  minutes.  This  tingling 
has  been  named  by  Seguin  the  signal  of  an  attack.  It  is  an  interest- 
ing fact  that  if  a  strong  irritation  of  the  skin  is  made  in  or  near  to 
where  the  tingling  is  felt  as  soon  as  the  tingling  begins,  the  spasm  may 


96 


DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 


be  averted.  Thus  I  have  a  patient  who  wears  a  strap  about  his  wrist 
and  who  pulls  it  tight  as  soon  as  he  feels  a  tingling  in  his  fingers,  in 
this  manner  preventing  the  occurrence  of  a  Jacksonian  spasm  of  the 
hand  and  arm,  that  is  sure  to  follow  when  he  omits  the  constriction  of 
the  wrist.  Such  a  tingling  sensation  is  more  commonly  felt  in  the 
fingers  and  hand,  in  the  toes  and  foot,  or  in  the  face  than  it  is  upon  the 
trunk  or  high  up  on  the  limbs,  probably  because  the  sensibility  of  these 

Fig.  45. 


Section  through  the  anterior  central  convolution,  showing  large  motor  neurones  in  the  third 
layer.    Contrast  this  figure  with  Figs.  33  and  59,  pages  87  and  120.     (Chr.  Jacob,  Das  Menschenhirn.) 

parts  is  more  highly  evolved  and  keener  than  in  the  others.  Or,  to 
state  it  differently,  the  sensations  in  the  periphery  of  the  limbs  are  more 
accurately  localized  and  represented  in  the  cortex.  The  occurrence  of 
these  sensations  with  the  local  spasm  is  an  argument  for  the  coincidence 
of  sensory  and  motor  functions  in  the  cortex. 

Local  spasms  are  always  followed  by  a  condition  of  voluntary  weak- 


LOCAL  SYMPTOMS.  97 

ness  which  may  amount  to  an  actual  flaccid  paralysis  in  the  muscles 
which  have  been  convulsed.  These  muscles  cannot  be  moved  for  some 
minutes  or  ^ven  hours,  but,  finally,  unless  the  original  lesion  is  a  de- 
structive one,  power  returns.  The  paralysis  lasts  longer  in  the  muscles 
in  which  the  spasm  begins  than  in  the  others.  Hence  the  subsidence 
of  the  paralysis  presents  an  order  of  succession  in  the  limbs  which  is 
just  the  reverse  of  the  order  of  invasion  in  the  spasm.  Thus  in  the 
case  shown  in  Fig.  42  the  patient  was  quite  henliplegic  for  an  hour 
after  each  attack,  then  the  leg  began  to  recover,  then  the  face,  then 
the  upper  arm,  and  finally  the  power  returned  in  the  hand  and  fingers. 
When,  in  the  growth  of  the  tumor,  however,  she  began  to  be  perman- 
ently paralyzed,  the  paralysis  extended  in  the  reverse  order,  following 
the  order  of  extension  of  the  spasm.  Thus  the  order  of  extension  of 
a  spasm  and  of  a  permanent  paralysis  is  just  reversed  in  the  order  of 
recovery  in  a  temporary  paralysis.  The  tumor  was  removed  four 
years  ago,  and  she  is  now  perfectly  well. 

All  these  facts  are  of  great  importance  as  an  indication  of  the  extent 
and  location  of  small  cortical  lesions  in  the  brain,  and  are  the  main 
guides  to  the  surgeon  in  trephining. 

Local  spasms  do  not  invariably  indicate  a  primary  lesion  in  and  a 
direct  irritation  of  the  motor  region.  Such  irritation  may  be  indirect ; 
that  is  to  say,  an  irritation  may  start  from  a  point  on  the  cortex  out- 
side the  motor  area  and,  as  it  extends,  finally  reach  that  area,  and  then 
set  up  a  spasm.  The  spasm  will  begin  in  the  limb  the  motor  area  of 
which  is  the  part  reached  by  the  irritation,  and  then  if  the  spasm 
extends  it  may  follow  the  regular  order.  Sometimes  an  irritation 
starting  in  the  occipital  or  parietal  region  and  extending  forward 
reaches  the  motor  area  of  the  fingers  and  toes  simultaneously.  Then 
the  spasm  will  begin  in  the  hand  and  foot  together,  extending  up  both 
limbs  at  once.  This  may,  therefore,  be  a  clinical  fact  of  importance  in 
showing  that  the  lesion  lies  outside  the  motor  zone.  Thus  in  Fig.  41 
the  location  of  a  tumor  is  shown  which  caused  Jacksonian  attacks 
which  sometimes  began  in  the  toes,  sometimes  in  the  fingers,  and  always 
involved  both  extremities  in  part  before  either  was  entirely  convulsed. 
In  such  cases  other  sensory,  aphasic  or  mental  symptoms  may  precede 
the  attack.  Much  care  must,  therefore,  be  taken  in  watching  the 
exact  characteristics  of  Jacksonian  epilepsy. 

Another  fact  of  interest  is  that  the  movements  and  spasms  due  to  a 
cortical  irritation  always  resemble  voluntary  movements.  Jackson 
says :  "  The  convulsion  is  a  brutal  development  of  a  man's  own  move- 
ments. A  severe  fit  is  nothing  more  than  a  sudden  excessive  and 
temporary  contention  of  many  of  the  patient's  familiar  motions,  such 
as  winking,  articulating,  singing,  manipulating,"  etc.  They  are  never 
contractions  of  single  muscles  or  of  groups  of  muscles  which  may  have 
a  nerve  supply  from  one  nerve  alone,  or  which  may  have  a  relation  in 
their  spinal  representation.  Thus  cortical  spasms  can  always  be  dif- 
ferentiated from  spasms  due  to  irritation  of  a  nerve  trunk  or  of  a 
spinal  nerve  root,  or  of  the  motor  mechanism  of  the  spinal  cord.    They 

7 


98  DIAGNOSIS  AND  LOCALIZATION  OF  BSAIN  DISEASES. 

are  not  like  reflex  acts.  The  cortical  movements  can  always  be  vol- 
untarily imitated.  They  are  always  apparently  for  a  purpose  and  show 
a  certain  amount  of  coordination  and  adaptation  to  an  end.  Cortical 
acts  have  always  been  acquired  by  a  process  of  education  which  can  be 
seen  in  progress  by  watching  an  infant.  The  act,  when  finally  learned 
after  many  attempts  and  repetitious,  leaves  a  memory  which  has,  as 
a  physical  basis,  an  organized  and  inter-related  group  of  cells  in  the 
cortex.  Irritate  this  group,  and  the  act  is  performed.  Thus  cortical 
movements  may  be  started  by  electrical  applications  through  needles 
to  the  cortex,  and  in  surgical  operations  for  Jacksonian  epilepsy  it  is 
well  to  explore  the  cortex  with  a  faradic  current,  to  elicit  movements 
until  those  are  produced  which  exactly  resemble  the  spasm  of  the  dis- 
ease, and  then  to  extirpate  the  area  whose  exact  function  is  thus  estab- 
lished. The  localized  spasms  of  cortical  disease  are  rarely,  if  ever, 
successfully  imitated  either  by  malingerers  or  by  hysterics.  A  hemi- 
spasm sometimes  occurs  in  hysteria,  but  it  usually  begins  in  both  limbs 
at  once,  and  if  it  extends  from  one  limb  to  the  other,  or  if  it  extends 
along  a  single  limb,  it  rarely  preserves  the  exact  order  of  extension  so 
uniformly  observed  in  local  lesions.  Hence  it  is  not  difficult  to  distin- 
guish cortical  epilepsy  from  hysterical  attacks  of  a  convulsive  nature. 
Localized  convulsions  do  not  induce  a  loss  of  consciousness. 
Localized  convulsions  may  extend  from  one  part  to  another  until 

Fig.  46. 


Situation  of  focus  of  hemorrhagic  encephalitis,  causing  paralysis  of  the  tongue  and  lips.     (Dana.) 

the  entire  side  is  in  a  state  of  spasm.  They  may  then  extend  to  the 
other  side  of  the  body,  setting  up  general  convulsions,  which  may  be 
accompanied  by  loss  of  consciousness.  Whether,  in  such  cases,  the 
irritation  is  conveyed  to  a  convulsive  centre  in  the  pons  Varolii  or  to 
the  motor  cortex  of  the  opposite  hemisphere  by  way  of  the  commissural 
fibres  is  not  yet  determined. 

Irritation  in  the  cortex  does  not  necessarily  lead  to  convulsions. 
The  Jacksonian  attack -may  be  exclusively  sensory,  leading  to  hallu- 
cinations, or  when  in  the  speech  areas,  leading  to  temporary  attacks  of 
aphasia,  or  to  mental  states  of  disturbance  of  consciousness,  or  of  the 
will,  or  of  the  memory.  But  any  of  these  forms  of  attack  may  go  on 
to  a  localized  spasm,  and  a  localized  spasm  may  in  its  turn  produce 
subsequently  one  of  these  peculiar  states.  They  will  be  more  fully 
considered  when  sensory  and  aphasic  symptoms  are  discussed. 


LOCAL  SYMPTOMS.  99 

Paralysis  from  Cortical  Disease. —  Paralysis  is  a  symptom  of  local 
lesion  in  ^e  motor  area  of  the  brain  or  in  the  tract  from  it  to  the 
spinal  cord.  This  paralysis  is  one  of  voluntary  motion,  the  reflex  and 
automatic  acts  presided  over  by  the  spinal  and  subcortical  basal  cen- 
tres being  in  no  way  interfered  with.  The  character  of  the  paralysis 
depends  largely  upon  the  location  of  the  disease  and  upon  its  extent. 

Paralysis  of  motion  of  the  face,  arm,  or  leg,  of  the  head  and  eyes, 
or  trunk,  may  be  due  to  cortical  disease  when  the  motor  area  con- 
trolling their  movements  is  affected.  From  the  extent  of  the  paralysis 
it  is  possible  to  arrive  at  a  conclusion  regarding  the  extent  of  the 
lesion  in  the  cortex.  Lesions  of  the  cortex,  as  a  rule,  are  not  very 
extensive.  Thus  cortical  hemorrhages  are  rarely  very  large,  softened 
areas  from  embolism  or  thrombosis  in  the  cortex  are  limited  as  the 
collateral  circulation  is  good ;  an  abscess  or  a  tumor  in  the  cortex  is  at 
first  small.  Therefore,  as  a  rule,  from  cortical  disease  we  get  a 
paralysis  of  limited  extent,  the  face  alone,  or  arm  alone,  or  these 
together  being  paralyzed ;  or  the  leg  alone,  or  arm  and  leg  together 
being  affected.  This  is  termed  monoplegia  or  associated  monoplegise. 
Since  each  hemisphere  of  the  brain  controls  the  opposite  side  of  the 
body  chiefly,  the  paralysis  from  a  lesion  in  one  hemisphere  is  limited 
to  the  opposite  side  of  the  body.  Paralysis  of  one  side  is  termed 
hemiplegia.  In  cases  where  both  hemispheres  are  involved  we  may 
have  double  hemiplegia  or  diplegia ;  but  this  is  a  rare  condition.  As 
a  matter  of  fact,  each  hemisphere  is  connected  by  the  motor  tract  with 
both  halves  of  the  spinal  cord,  as  we  shall  presently  see,  and  there- 
fore in  every  case  of  hemiplegia  the  unparalyzed  side  is  really  a  little 
weaker  than  before  the  attack.  There  are  rare  cases  where  the  hemi- 
plegia has  been  on  the  side  of  the  lesion.  These  are,  however,  ex- 
plained by  an  absence  of  a  decussation  of  the  motor  tracts  in  their 
course.  But  the  separation  of  various  motor  areas  from  one  another 
in  the  cortex,  and  the  usual  limitations  of  lesions  to  small  regions  in 
the  cortex,  makes  hemiplegia  from  cortical  disease  rare.  The  usual 
result  of  a  cortical  lesion  is  a  monoplegia. 

The  Characteristics  of  Voluntary  Action.  —  The  paralysis,  as  already 
stated,  is  one  of  voluntary  motion  that  has  been  acquired  by  practice. 
If  we  study  voluntary  acts  as  they  are  performed  we  find  that  each  act 
involves  a  succession  of  movements  by  different  joints,  each  movement 
being  produced  by  the  contraction  of  a  number  of  different  muscles. 
The  act  of  lifting  a  feather  or  a  heavy  weight  involves  the  same  muscles, 
but  there  is  a  great  difference  in  the  order,  sequence,  and  relative  force 
,  expended  in  the  two  acts.  In  both  the  entire  muscular  system  of  the 
arm  is  called  into  play  but  the  force  of  each  is  different  and  the 
order  of  action  of  the  muscles  differs  The  moter  centres  of  the  cortex 
govern  acts  rather  than  muscles.  It  is  the  act  of  grasping,  of  lifting, 
of  opening  the  hand,  of  pointing,  etc.,  that  is  produced  by  cortical 
activity  rather  than  mere  contraction  of  flexors  and  extensors  of  the 
fingers.  Now  any  such  act  involves  the  coordinated  movement  of 
many  muscles   for   its   production.     We  must   think,  therefore,  of 


100        DIAGNOSIS  AND  LOCALIZATION  OF  BHAIN  DISEASES. 

the  motor  area  of  the  hand,  for  example,  as  containing  an  enormous 
number  of  centers  of  action  arranged  in  groups,  so  that  it  cannot  be 
divided  by  sliarp  lines  into  flexor  and  extensor  centres,  since  flexor 
and  extensor  action  occur  simultaneously  in  many  acts.  If  the  motor 
area  be  divided  at  all  it  must  be  into  regions  for  various  acts,  each 
having  its  predominant  feature.  Hence  in  subdividing  the  arm  area 
into  regions  for  shoulder,  elbow,  wrist,  and  fingers  it  is  intended 
to  separate  actions  whose  special  feature  is  the  movement  of  the  joint 
named.  In  each  division  all  the  joints  may  be  represented  in  part,  but 
in  diiferent  degrees.  Thus  the  lifting  of  the  arm  above  the  head  is 
primarily  a  shoulder  movement,  but  involves  some  action  in  all  the 
other  joints,  since  the  forearm  and  fingers  do  not  hang  limp.  And  a 
firm  grasp,  though  primarily  a  flexor  action  of  the  fingers,  involves 
some  act  of  support  by  all  the  muscles  of  the  arm.  In  the  case  shown 
in  Fig,  40  the  shoulder  motions  were  more  defective  than  those  of 
the  hand.  In  the  case  shown  in  Fig.  42  the  hand  motions  were 
impossible  and  the  shoulder  was  only  partially  paralyzed. 

As  the  pianist,  by  striking  the  keys  in  different  combination,  pro- 
duces an  unnumbered  variety  of  harmonies  from  a  small  number  of 
strings,  so  the  cortex,  by  exciting  the  spinal  neurones,  and  through 
them  the  muscles,  attains  a  countless  variety  of  movements.  The  vibra- 
tion of  a  single  string  gives  sound  but  no  music,  and  the  contraction 
of  a  single  muscle  gives  motion  but  not  an  act.  The  loss  of  a  single 
string  causes  discord,  but  does  not  stop  the  sounds,  for  other  strings 
still  vibrate.  The  paralysis  of  a  muscle  impairs  motion,  but  does  not 
stop  the  act.  The  strings  lie  silent  unless  the  keys  are  struck.  The 
muscles  are  not  called  into  action  unless  a  cortical  impulse  reaches  them. 
Thus  we  must  regard  the  cortical  motor  centres  as  different  in  their 
action  from  the  spinal  motor  neurones.  They  are  the  storehouses  of 
memories  of  movements,  and  are  capable  of  reproducing  these  move- 
ments which  they  have  acquired.  Skill  in  any  complex  act,  like  that 
of  the  musician,  is  only  gained  by  numberless  repetitions.  Watch  the 
process  of  learning  to  talk  or  to  write  in  a  child,  and  it  will  be  evident 
that  countless  futile  attempts  precede  success.  Such  attempts  are  really 
simultaneous  acts  in  many  centres,  causing  incodrdinated  movements, 
but  little  by  little  one  is  subordinated  to  another  in  vigor,  and  one  is 
made  to  precede  another  in  time  until  an  order  is  reached  and  an  accu- 
racy is  attained,  and  this  by  repetition  becomes  a  fixed  combination,  as 
easy  for  the  cortex  as  is  the  motion  producing  the  harmony  for  the 
pianist.  That  the  simplest  acts  are  first  acquired  is  to  be  expected,  but 
there  is  no  limit  to  the  skill  possible  and  no  limit  to  the  variety  of 
complex  movements  which  may  be  learned.  Every  artisan,  every 
musician,  every  dancer,  has  a  peculiar  individual  store  of  motor  mem- 
ories. Some  individuals  possess  a  greater  variety  of  them  than  others. 
Hence  the  motor  zone  on  the  cortex  is  of  different  extent  in  different 
persons,  each  newly  acquired  set  of  movements  increasing  its  area. 

The  Motor  Centres  of  Speech.  —  There  are  some  highly  complex  motor 
acts  which  seem  to  have  a  special  region  assigned  to  them.     The  act 


LOCAL  SYMPTOMS. 


101 


of  speech  and  the  act  of  writing  are  such,  and  these  are  located  in  the 
posterior  pai^  of  the  third  and  second  frontal  convolutions,  respectively. 
The  power  to  speak  and  to  write  are  lost  when  these  regions  are 
destroyed  by  disease.  Yet  such  a  destruction  does  not  necessarily 
involve  a  paralysis  of  the  muscles  which  carry  out  these  acts ;  it  does 
not  even  suspend  the  voluntary  movements  in  these  muscles,  which 
can  be  well  performed,  but  not  in  the  combination  needed  to  produce 
speech  or  writing. 

The  loss  of  the  power  of  speech  is  termed  aphasia.  There  are  two 
types  of  aphasia,  which  are  recognized  as  motor  and  sensory.  In  motor 
aphasia  the  act  of  talking  or  writing  is  lost.  In  sensory  aphasia  the 
understanding  of  words  heard  or  seen  is  lost.  The  centres  which  pre- 
side over  the  emission  of  language  are  highly  complex  motor  centres 
-which  appear  to  utilize  the  secondary  motor  centres  in  the  facial  area, 
setting  them  to  work  in  varying  combinations.  When  the  facial  centres 
are  paralyzed  attempts  at  speech  may  fail  from  imperfect  pronuncia- 
tion or  articulation.  This  is  termed  anarthria,  and  is  attended  by 
paralysis  of  the  tongue  and  lips,  as  in  the  case  of  Dana,  shown  in  Fig. 


Fig.  47. 


Tumor.     (Starr.) 


Tumor.     (Dejerine. ) 


Clot. 


(Starr.)  Clot.     (Hotchkiss.) 

Situation  of  lesions  causing  motor  aphasia. 


46.  When  the  motor  speech  centres  are  paralyzed  no  attempt  can 
be  initiated,  and  hence  words  cannot  be  produced  either  by  voice  or 
hand.  In  Fig.  47  a  number  of  cases  are  shown  wliere  the  lesion 
caused  motor  aphasia.  The  state  of  motor  aphasia  is  not  attended  by 
any  loss  of  comprehension  of  language.  The  understanding  is  good, 
and  tlu;  patient  knows  what  lie  wants  to  say  but  cannot  find  the  words. 


102         DIAGNOSIS  yiNV  LOCALIZATION  OF  BHAIN  DISEASES. 

Nor  can  lie  repeat  words  after  another.  The  motor  combination  appears 
to  be  lost.  The  condition  may  be  general  or  partial.  Some  words 
may  be  said  while  others  cannot,  and  sometimes  words  are  possible, 


Fig.  48. 


FPijc 


Scheme  of  the  projection  fibres  of  the  cerebral  cortex.  Cc,  corpus  callosum  ;  Cr,  internal  capsule, 
into  which  fibres  pass  from  the  cortex  above  the  Sylvian  fissure  ;  NC,  caudate  nucleus  ;  NL,  lenticular 
nucleus ;  Th,  optic  thalamus,  in  which  many  fibres  from  all  parts  of  the  cortex  end ;  Cip,  posterior 
division  of  the  internal  capsule,  corresponding  to  the  sensory  tract  in  Fig.  52  ;  Cisl,  sublenticular  part 
of  the  internal  capsule,  containing  fibres  of  auditory  tract  and  fibres  to  the  thalamus  from  the  cortex 
below  the  Sylvian  fissure;  CL,  Luys'  body;  NR,  red  nucleus  of  tegmentum;  Ln,  locus  niger;  VP, 
tract  from  capsule  to  pons  ;  Po,  pons ;  NP,  gray  matter  of  pons  ;  Oi,  Olive ;  Pi/,  pyramidal  tract  in 
medulla,  passing  to  FPi/d,  anterior  median  column  of  cord,  and  to  FPi/c,  lateral  column  of  cord  of 
opposite  side,  and  to  fPyh,  lateral  column  of  cord  of  same  side  ;  B,  medulla.  (Dejerine,  Anatomie  des 
Centres  Nerveux,  vol.  ii.,  p.  2.) 


PLATE  X 


J&ciaZis. 
Sypoghssm. 

J     Arm 
Coptic 

-Beuv 


<s:b 


CA. 


ffh 


ca^c 


Horizontal  Section  through  the  Right  Hemisphere.     (After  von  Monakow.) 

The  important  parts  of  the  internal  capsule  are  colored  red.  B.  Kn,  knee  of  corpus  callosum; 
Vh,  anterior  horn  of  lateral  ventricle;  F-^,  inferior  part  of  third  frontal  convolution;  I.  stric,  lenticulo- 
striate  division  of  internal  capsule;  Knie.  ic,  knee  of  internal  capsule;  I.  oph'c,  lenticulo-optic  division 
of  internal  capsule;  Th,  optic  thalamus;  ./,  island  of  l^eil;  d,  claustrum;  Operc,  operculum;  Ti,  first 
temporal  convolution;  r.  lie,  retrolenticular  region  of  internal  capsule;  C.  A.,  ammon's  horn;  calc,  cal- 
carine  fissure;  IJh,  posterior  horn  of  lateral  ventricle;  SS,  optic  radiation  of  Gratiolet;  T.^,  second 
temporal  convolution;  Facialis,  position  in  capsule  of  motor  tract  to  the  face;  Ilypoglossus,  position  of 
tract  to  the  tongue;  Arm,  position  of  tract  to  the  arm;  Bein,  position  of  tract  to  the  leg;  S.  B.,  sensory 
fibres;  .S.  visual  tract;  A  ,  auditory  tract. 


LOCAL  SYMPTOMS.  103 

but  their  combination  in  sentences  is  impossible.     Writing  is  usually 
lost  with  speech. 

Here,  then,  is  another  proof  that  the  cortical  motor  acts  are  not 
simple  muscular  motions,  but  are  highly  complex  combinations.  It  is 
as  if  an  electric  key  by  one  pressure  started  a  whole  mass  of  machinery 
whose  different  parts  were  independent  and  wholly  unlike  in  their 
activities. 

The  paralysis  from  cortical  disease  is  therefore  a  suspension  of  the 
power  of  highly  complex  actions,  and  when  it  is  due  to  a  destruction 
of  the  cortical  centres  it  is  attended  by  a  loss  of  the  memories  of  effort 
essential  to  those  acts. 

Paralysis  from  Subcortical  Disease.  —  Paralysis  may  also  be  due  to  an 
interruption  in  the  tract  which  conveys  these  cortical  impulses  to  the 
motor  centres  in  the  base  of  the  brain  and  spinal  cord.  This  tract  is 
called  the  motor  tract.     Its  course  is  shown  in  Fig.  48. 

It  arises  from  the  under  surface  of  the  cortex  of  the  posterior  part 
of  the  third  frontal  convolution,  the  anterior  central  convolution,  and 
the  paracentral  lobule,  and  passes  out  of  the  base  through  the  middle 
third  of  the  crus  cerebri.  Its  fibres  gather  together  at  the  middle  por- 
tion of  the  upper  surface  of  the  internal  capsule  ;  those  from  the  lower 
parts  of  the  cortex  passing  straight  inward,  those  from  the  upper  parts 
curving  outward  and  downward  to  pass  around  the  side  of  the  lateral 
ventricle.  Thus,  within  the  centrum  ovale  these  fibres,  if  looked  at 
from  in  front,  appear  like  the  sticks  of  a  fan,  and,  like  those  sticks, 
their  relative  position  is  altered  in  the  point  of  junction,  where  those 
passing  inward  from  the  lowest  part  of  the  cortex  lie  in  front  of  those 
that  pass  downward  from  its  upper  part.  Thus  in  the  capsule  the 
order  from  before  backward  is,  first,  the  fibres  conveying  speech  im- 
pulses to  the  pons  and  medulla ;  second,  the  fibres  conveying  facial- 
motor  impulses  to  the  pons;  third,  the  fibres  destined  to  the  arm 
centres  of  the  cord ;  fourth,  the  fibres  transmitting  impulses  to  the  leg 
centres  in  the  cord.  The  fibres  conveying  impulses  to  the  muscles  of 
the  trunk  lie  between  those  to  the  arm  and  leg.  Plate  X.  shows 
the  relative  positions  of  these  fibres  in  the  internal  capsule. 

From  the  anterior  half  of  the  posterior  division  of  the  capsule  this 
tract  passes  through  the  middle  third  of  each  crus  (where  the  fibres  con- 
nected with  the  oculo-motor  nuclei  are  given  off),  through  the  pons 
(Fig.  49)  (where  the  division  to  the  facial  nucleus  crosses  to  the 
opposite  side  and  ends),  and  thence  by  way  of  the  pyramids  of  the 
medulla  to  the  crossed  pyramidal  and  direct  anterior  median  columns 
of  the  spinal  cord.  It  is  evident,  however,  that  the  concentration  of 
this  tract  is  much  greater  in  the  capsule  than  in  the  centrum  ovale, 
where  the  individual  fibres  are  scattered  among  the  other  systems  and 
occupy  but  a  small  area  from  before  backward. 

The  character  of  paralysis  occurring  from  lesions  in  this  tract  is  also 
shown  in  Fig.  49.  Four  lesions  are  there  shown :  one  in  the  cen- 
trum ovale  near  the  cortex,  one  in  the  internal  capsule,  one  in  the 
crus,  and  one  in  the  pons.     It  is  evident  that  the  lesion  in  the  centrum 


104        DIAGXOSIS  AXD  LOCALIZATION  OF  BTiAIN  DISEASES. 

ovale  affects  one  set  of  fibres  only,  those  from  the  arm  centre,  and 
produces,  therefore,  a  monoplegia  only.  A  lesion  of  the  centrum  ovale 
would  have  to  be  very  extensive  to  involve  the  fibres  from  the  face, 
arm,  and  leg  centres  together.     But  a  small  lesion  in  the  internal  cap- 

FiG.  49. 


Scheme  of  the  motor  tract  to  show  the  effect  of  the  lesions  at  different  positions.  1,  cortical  or  sub- 
cortical lesion,  causing  monoplegia  of  left  arras;  2,  capsular  lesion,  causing  left  hemiplegia;  3,  crus 
lesion,  causing  left  hemiplegia  and.  right  third  nerve  palsy  ;  4,  pons  lesion,  causing  alternating 
paralysis  of  right  face  and  felt  arm  and  leg ;  S,  sylvian  fissure ;  O.T,  optic  thalamus ;  N.L,  lenticular 
nucleus  ;  C,  crus  ;  N.C,  caudate  nucleus  ;  VII.,  facial  nerve  ;  51,  medulla. 


sule  cuts  all  these  fibres  where  they  pass  in  a  condensed  tract,  and 
hence  produces  a  hemiplegia,  face,  arm,  and  leg  being  all  paralyzed  on 
the  opposite  side.  It  is  very  rare  to  get  a  monoplegia  from  a  lesion 
in  the  capsule.  Any  clot  larger  than  the  size  of  a  pea  destroys  the 
entire  motor  tract  here.  The  vast  majority  of  cases  of  hemiplegia  are 
due  to  a  lesion  of  the  internal  capsule.  And  the  figure  shows  that  it 
is  only  in  their  passage  through  the  capsule,  the  crus,  and  the  upper 
part  of  the  pons  that  these  fibres  can  be  affected  together  by  a  single 
small  lesion. 

A  lesion  in  the  crus  cerebri  causes  hemiplegia ;  but  since  the  optic 
tract  crosses  the  crus,  and  the  third  nerve  to  the  ocular  muscles  comes 
out  from  its  inner  surface  it  is  hardly  possible  for  both  of  these  nerves 
to  escape  in  a  lesion' at  this  point.  Hemiplegia  associated  with  oculo- 
motor palsy  with  or  without  hemianopsia  is  characteristic  of  a  lesion 


LOCAL  SYMPTOMS.  105 

of  tlie  crus.  It  has  been  termed  Weber's  syndrome.  The  eye  affected 
is,  of  course^  the  one  on  the  side  of  the  lesion,  and  hence  on  the  side 
opposite  to  the  paralysis  of  the  face,  arm,  and  leg.  It  is  turned  out- 
ward, cannot  be  moved  upward,  downward,  or  inward,  and  its  pupil 
is  dilated.  Ptosis  is  present,  that  is,  the  upper  lid  falls,  and  the  ■  eye 
is  closed  by  paralysis  of  the  levator  palpebrse. 

Hemiplegia  may  be  due  to  a  lesion  in  the  upper  part  of  the  pons, 
but  when  the  lower  part  of  the  pons  is  affected  the  face  escapes  on  the 
side  on  which  the  limbs  are  paralyzed.  This  is  because  of  the  decus- 
sation of  the  tract  to  the  facial  nucleus  which  enters  the  raphe  in  the 
pons  and  crosses  to  the  other  side.  But  such  a  lesion  interrupts  the 
course  of  the  tract  to  the  facial  nucleus  on  the  side  of  the  lesion. 
Hence  from  lower  pons  lesions  we  get  alternating  paralysis;  that  is, 
a  paralysis  of  the  face  on  the  side  of  the  lesion  and  of  the  arm  and 
leg  on  the  opposite  side.  If  the  tract  to  the  facial  nucleus  is  affected 
the  eye  can  be  closed.  If  the  nucleus  itself  is  destroyed  the  eye  can- 
not be  closed.  This  form  of  paralysis  cannot  be  caused  by  a  lesion 
elsewhere. 

The  motor  tract  to  the  hypoglossal  nucleus  which  governs  the  tongue 
pursues  a  course  analogous  to  that  of  the  facial  nucleus,  but  crosses 
the  middle  line  at  the  upper  part  of  the  medulla.  A  condition  of 
paralysis  of  one-half  of  the  tongue  and  of  the  arm  and  leg  on  the 
opposite  side  is  produced  by  a  lesion  in  the  medulla ;  such  lesions  are 
rare. 

The  decussation  of  the  motor  tract  takes  place  in  the  pyramids  of 
the  medulla.  It  is  incomplete,  some  fibres  crossing  to  the  lateral  tract 
of  the  cord,  others  going  down  in  the  anterior  median  column  of  the 
same  side.  The  degree  of  the  decussation  varies  in  different  persons. 
In  some  it  is  almost  total,  in  others  it  is  partial.  In  some  it  is  irregu- 
lar, many  fibres  crossing  on  one  side  and  few  on  the  other.  Thus 
Fig.  16,  page  51,  shows  a  marked  asymmetry  in  the  lateral  tracts  and 
anterior  median  columns  due  to  such  irregular  decussation.  Flechsig 
has  remarked  this  difference  in  foetal  cords,  and  has  even  found  one  case 
in  sixty  in  which  no  decussation  occurs  at  all.  This  variability  in  the 
anatomy  of  the  motor  tract  explains  the  different  degree  of  paralysis  in 
different  cases  of  hemiplegia.  In  some  cases  the  paralysis  is  very 
complete  on  one  side  and  hardly  perceptible  on  the  other.  In  other 
cases  there  is  distinct  weakness  on  the  side  opposite  to  the  hemiplegia. 
A  few  rare  cases  have  been  reported  by  Brown-Sequard  in  which  the 
hemiplegia  was  on  the  side  of  the  lesion,  and  in  these  we  may  assume 
that  there  was  no  decussation  of  the  motor  tract.  A  lesion  of  the 
medulla  may  affect  both  tracts  at  their  decussation,  and  hence  cause 
paralysis  of  all  four  limbs.     This,  again,  is  very  rare. 

The  motor  tract  in  the  spinal  cord  in  the  lateral  column  may  be 
affected  in  the  disease  lateral  sclerosis  which  has  been  already  studied. 
A  unilateral  affection  of  the  tract  in  the  spinal  cord  is  not  common, 
hence  a  hcnn'plcgia  from  s})inii,l  lesion  is  extremely  rare. 

These  types  of  hemiplegia  0(!cur  from  every  kind  of  l)rain  disease. 


IOC         DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES, 

The  alternating  form  is  indicative  of  a  lesion  on  the  base  either  within 
or  outside  of  the  brain  axis. 

Disturbance  in  the  Sense  of  Touch  is  a  local  symptom  of  disease  in 
the  brain.  There  is  some  discussion  as  to  the  area  of  the  cortex  which 
receives  these  sensations  and  as  to  the  course  of  the  tract  which  brings 
them  in. 

The  probable  localization  of  the  sensory  centres  is  m  the  posterior 
central  convolution  and  in  the  adjacent  portions  of  the  cortex  of  the 
parietal  lobules.  Lesions  in  this  locality  usually  cause  a  loss  of  tactile 
sense  in  the  opposite  side  of  the  body.  The  sensory  area  can  be 
divided,  like  the  motor  area,  into  subdivisions  for  face,  arm,  and  leg, 
which  lie  respectively  in  the  lower,  middle,  and  upper  thirds  of  this 
region.  A  collection  of  cases  of  local  cortical  disease  made  by  me  in 
1884  led  to  this  conclusion.  Dana  subsequently  collected  other  cases 
which  supported  it,  and  Nothnagel,  Charcot,  Gallet,  and  von  Mona- 
kow  accept  this  localization. 

Some  authorities  have  held  that  the  sensory  and  motor  areas 
exactly  coincide.  Dejerine,  Flechsig,  Henschen,  Mott,  and  Schafer 
urge  this  view.  But  my  cases  prove  that  lesions  in  the  motor  area  in 
front  of  the  fissure  of  Rolando  rarely  cause  loss  of  tactile  sense,  while 
lesions  behind  it  usually  produce  anaesthesia. 

Another  theory  of  the  localization  of  sensory  centres,  held  by  Ferrier 
and  Mills,  places  them  in  the  hippocampal  region.  This  has  been 
founded  on  physiological  experiment,  but  is  not  confirmed  by  patho- 
logical records  in  man,  and  those  who  hold  it  have  not  attempted  any 
subdivision  of  this  area  for  the  diiferent  limbs.  I  think  it  may  be  dis- 
carded, for  in  many  cases  of  operation  upon  the  cortex  in  living  persons 
for  the  relief  of  epilepsy  and  for  the  removal  of  tumors  I  have  seen 
small  lesions  of  the  cortex  of  the  convexity  near  the  motor  area  cause 
a  loss  of  sensation,  sometimes  temporary,  sometimes  permanent. 

This  loss  of  sensation  is  usually  limited  to  one  side  of  the  face  or  to 
one  limb  in  cases  of  cortical  disease  for  the  same  reason  that  paralysis 
in  cortical  disease  is  more  commonly  of  the  monoplegic  than  of  the 
hemiplegic  variety.  A  loss  of  sensation  in  the  entire  side  of  the  body, 
termed  hemiansesthesia,  is  more  commonly  caused  by  a  lesion  of  the 
sensory  tract  within  the  brain  than  from  disease  of  the  cortex. 

Irritation  of  the  Cortical  Centres  for  Touch  causes  a  sensation  of 
tingling  in  the  part  of  the  body  which  corresponds  to  the  centre  affected. 
This  is  termed  parsesthesia.  It  has  been  already  alluded  to  as  a  fre- 
quent warning  and  as  a  constant  accompaniment  of  Jacksonian  epilepsy, 
under  which  circumstances  it  is  a  temporary  symptom.  It  is  frequently 
observed  as  the  result  of  slight  disturbances  of  circulation  in  the  cortex, 
and  hence  is  a  prodrome  of  apoplexy.  It  occurs  as  a  permanent  symp- 
tom in  cases  of  pressure  on  the  cortex,  and  hence  is  found  in  cases  of 
cortical  clot  and  of  cortical  tumor.  The  tingling,  when  temporary,  is 
often  followed  by  a  slight  temporary  anaesthesia,  and  when  permanent 
is  always  attended  by  a  sliglit  tactile  anaesthesia. 

Disturbance  of  the  sense  of  touch  includes  both  superficial  sensations 


LOCAL  SYMPTOMS.  107 

of  touch,  temperature,  and  paiu,  and  deep  sensations  of  articular  and 
muscular  sense.  There  is  no  question  that  these  different  elements  in 
the  sense  of  touch  are  independent  of  one  another,  and  it  is  certain  that 
they  are  transmitted  to  the  cortex  by  different  sensory  tracts  which  we 
shall  presently  trace.  There  is  every  reason  to  believe  that  they  are 
received  in  different  neurones  of  the  cortex,  for  clinical  experience 
teaches  that  one  sense  may  be  lost  when  others  are  preserved.  Thus 
in  one  of  my  cases  of  apoplexy  the  patient  lost  the  sense  of  temperature 
alone  on  one  side  ;  in  another  case  the  patient  could  perceive  cold  sen- 
sations only,  and  had  a  constant  feeling  of  cold,  but  could  not  perceive 
warm  sensations ;  in  still  another  case  sensations  of  temperature  and 
pain  were  lost,  while  touch  was  preserved  in  one  side.  In  two  cases  ^ 
of  operation  upon  the  cortex  I  have  seen  a  complete  loss  of  muscular 
sense  in  the  arm  as  the  result  of  a  small  lesion  in  the  superior  parietal 
lobule  just  behind  the  motor  area.  The  first  of  these  cases  was  in  a 
man,  aged  twenty-one  years,  who  had  a  fracture  of  the  left  parietal 
bone  at  the  age  of  five  years,  and  a  severe  fall  on  the  head  at  the  age 
of  sixteen  years.  For  five  years  he  had  suffered  from  intense  pain  in 
the  left  parietal  bone  about  half-way  from  the  boss  to  the  median  line. 

Fig.  50. 


Situation  of  a  tumor  causing  loss  of  muscular  sense  and  ataxia  in  the  right  hand, 
with  astereognosis. 

a  point  that  at  the  operation  was  found  to  be  just  over  the  junction  of 
the  superior  and  inferior  parietal  lobules.  (Fig.  50.)  The  pain, 
though  constant,  was  subject  to  great  exacerbations  about  once  a  week, 
in  which  he  would  become  maniacal,  violent,  and  abusive,  and  some- 
times unconscious.  He  had  little  memory  of  his  acts  when  the  attack 
was  over.  Prior  to  the  operation  he  had  no  paralysis,  ataxia,  or  loss 
of  sensation.  Trephining  at  the  seat  of  pain  revealed  an  angioma  three 
quarters  of  an  inch  in  size,  limited  to  the  pia  mater,  as  the  brain  cortex 
appeared  normal.  In  removing  this  the  brain  was  slightly  lacerated 
by  the  passage  of  the  needles  carrying  ligatures  to  tie  the  veins  and  by 
an  exploratory  puncture  to  ascertain  the  possible  existence  of  a  cyst. 
Recovery  from  the  operation  occurred,  but  immediately  after  it  and  for 
six  weeks  a  state  of  marked  ataxia  was  present  in  the  right  hand  with 

'One  of  these  is  reported  in  tlie  American  Journal  of  the  Medical  Sciences,  Nov., 
1894,  with  McCosh.     The  second  case  was  seen  with  him  at  the  Presbyterian  Hospital. 


108        DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

a  loss  of  muscular  sense.  All  purely  coordinated  movements  were 
impossible  ;  an  attempt  to  place  the  finger  on  the  nose  failed,  the  finger 
being  carried  to  the  ear  or  far  to  one  side  or  above  the  head.  He  was 
unable  to  tell  with  closed  eyes  what  position  had  been  given  to  the 
fingers,  and  was  unable  to  reproduce  such  positions  in  the  left  hand. 
There  was  no  loss  of  power.  Tactile  and  temperature  senses  were  keen. 
The  muscular  sense  returned  gradually,  and  he  has  been  perfectly  well 
for  the  past  seven  years.  The  second  case  was  almost  identical,  a  cyst 
being  found  and  removed  from  the  same  location  with  little  injury  to 
the  cortex,  but  with  the  production  of  temporary  ataxia  in  the  hand. 
I  have  often  seen  a  marked  ataxia  from  cortical  lesions  associated  with 
hemianaesthesia,  but  these  two  cases  were  without  other  motor  or  sensory 
symptoms.  While,  therefore,  there  is  every  reason  to  believe  that  the 
cortical  centres  for  the  perception  of  touch,  temperature,  pain,  and 
muscular  sense  are  independent  of  one  another,  so  far  as  our  present 
knowledge  goes,  it  is  necessary  to  locate  them  grossly  in  the  same 
locality,  viz.,  near  to,  but  behind,  the  motor  zone.  It  is  possible  that 
various  layers  of  the  cortical  neurones  possess  diiferent  functions,  but 
this  is  only  a  hypothesis. 

The  characteristics  of  cortical  hemiansesthesia  are  its  common  associ- 
ation with  hemiplegia,  the  limb  which  is  most  paralyzed  being  most 
ansesthetic.  But  there  is  no  necessary  relation  between  motor  and  sen- 
sory disturbance,  as  either  may  occur  alone,  or  one  may  be  intense 
while  the  other  is  slight. 

The  anaesthesia  from  cortical  lesion  is  never  total.  Each  side  of  the 
body  is  connected  with  both  hemispheres  of  the  brain,  though  the 
crossed  connection  is  far  more  complete  than  the  direct  one.  Hence 
a  slight  degree  of  sensibility  in  the  ansesthetic  side  is  always  to  be 
found,  especially  in  cases  of  any  length  of  duration.  Cortical  anaes- 
thesia usually  subsides  gradually  after  a  lesion,  and  the  sensibility 
returns  in  part  even  if  not  wholly.  Dejerine  noticed  that  this  was 
more  common  in  young  persons  than  in  middle-aged  or  old  ones.  The 
anaesthesia  from  organic  lesions  is  always  most  intense  in  the  distal 
part  of  a  limb;  the  fingers  and  hand  are  more  insensitive  than  the 
forearm,  and  this  than  the  arm  or  trunk;  the  toes  and  sole  are  more 
insensitive  than  the  leg,  and  this  than  the  thigh;  the  face  is  more  in- 
sensitive than  the  neck  or  trunk,  and  the  lips  are  the  most  insensitive 
part  of  the  face  when  it  is  affected.  There  is  no  sharp  limit  to  the 
anaesthesia,  it  fades  gradually  into  an  area  of  moderate  sensation. 
Dejerine  calls  attention  to  all  these  characteristics  as  enabling  one  to 
distinguish  it  from  the  anaesthesia  occurring  in  hysteria.  This  latter 
is  often  found  without  paralysis,  is  absolute,  does  not  disappear  gradu- 
ally, is  of  uniform  intensity  in  the  entire  side,  and  is  often  associated 
with  concentric  diminution  in  the  visual  field  of  the  eye  on  the  anaes- 
thetic side. 

Tests  of  Sensation.  —  Touch  is  to  be  tested  by  cotton-wool  or  by  a 
camel' s-hair  brush  or  by  the  fingers  of  the  examiner,  the  two  sides  of 
the  body  being  touched  simultaneously  in  symmetrical  places,  and  the 


LOCAL  SYMPTOMS.  109 

patient  being  requested  to  compare  the  two  sensations.  Temperature 
sensations  ^ay  be  similarly  tested,  test  tubes  with  hot  and  cold  water 
being  used.  Pain  sense  may  be  tested  by  needles  or  by  a  faradic 
brush.  Muscular  sense  may  be  tested  by  the  use  of  differently 
weighted  balls  or  coins  held  in  the  hands  and  compared,  or  by  weights 
being  placed  on  the  hands  which  are  supported,  each  side  being  tested 
separately  or  the  two  together.  The  sense  of  position  may  be  tested 
by  giving  a  position  to  one  limb  or  hand  and  asking  the  patient  to 
reproduce  this  in  the  other 'side.  If,  for  example,  the  right  hand  is 
put  in  a  clenched  position  and  the  blindfolded  patient  cannot  detect 
that  he  must  clench  the  left  one  in  order  to  imitate  this  position  he 
has  a  loss  of  muscular  sense  in  the  right  hand,  for  the  error  lies  not  in 
the  power  of  movement  but  in  the  perception  of  the  kind  of  motion 
necessary.  A  further  test  may  be  made  by  demanding  the  execution 
of  accurate  and  coordinated  movements,  such  as  picking  up  a  pin,  but- 
toning the  clothes,  carrying  a  glass  of  water  to  the  mouth. 

The  cortical  sensory  area,  in  the  act  of  handling  any  object,  receives 
simultaneously  numerous  tactile  and  temperature  sensations  associated 
with  the  sensations  of  pressure  and  of  weight  and  of  the  position  of 
the  fingers  involved  in  feeling  the  object.  These  sensations  leave 
behind  them  a  permanent  trace  in  some  physical  change  in  the  cortical 
cells.  They  are  combined  into  a  sensory  memory  of  the  object,  so 
that  following  the  acquisition  of  this  complex  sensory  mental  picture 
we  can  recognize  the  object  by  the  sense  of  touch,  even  if  we  do  not 
see  it.  Thus  we  have  tactile  memories  of  objects  such  as  an  orange, 
a  rose,  a  bell,  a  pen,  which  enable  us  to  call  them  to  mind  and  to  rec- 
ognize them  when  felt.  This  power  of  recognizing  objects  by  touch 
has  been  named  stereognosis.  It  is  evident  that  the  stereognostic 
sense  is  not  a  simple  thing,  but  involves  a  simultaneous  activity  in  a 
large  number  of  different  tactile  sensory  neurones  which  are  grouped 
together.  The  recognition  of  an  object  by  it  is  comparable  rather  to 
the  recognition  of  a  melody  in  music  as  distinguished  from  a  single 
note.  This  is  one  of  the  functions  of  the  portion  of  the  cortex  con-. 
cerued  in  tactile  sensations.  And  in  fact,  when  we  consider  our  sen- 
sations for  a  moment  we  perceive  that  it  is  always  some  definite  object 
which  we  recognize  in  perceiving  a  tactile  sensation  rather  than  the 
sensation  alone.  It  is  the  touch  of  a  finger,  of  a  pin,  of  cotton-wool, 
of  a  hard  weighty  object  rather  than  touch  or  firmness  or  weight  which 
we  feel.  These  abstract  qualities  of  objects  are  only  known  by  a 
process  of  analysis  and  synthesis,  a  process  of  generalizing  from  a 
large  array  of  particulars.  The  thing  which  we  primarily  feel  and 
recognize  by  touch  is  not  a  quality,  it  is  an  object.  It  is  necessary, 
therefore,  to  regard  the  sensory  area  of  the  cortex  as  the  storehouse  of 
tactile  memories  of  objects  just  as  we  regard  the  motor  area  as  the  store- 
house of  motor  memories  of  combinations  of  motion.  The  loss  of  the 
power  of  recognition  by  touch  has  been  named  astereognosis. 

The  majority  of  our  tactile  perceptions  are  acquired  through  the 
hand.     We  have  few  memories  of  facial  or  pedal  sensations,  and  prac- 


110         DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

tically  none  of  the  trunk.  Hence  the  tactile  memory  area  is  more 
extensive  in  the  middle  third  of  the  posterior  central  convolution  and 
in  the  adjacent  part  of  the  inferior  parietal  lobule.  (Fig.  51.)  Red- 
lich^  (1893)  collected  twenty  cases  of  lesions  limited  to  this  area  which 
had   been   attended   by  astereognosis,  and  Walton  ^   has  recently  sup- 

FiG.  51. 


The  area  of  the  cortex  in  which  a  lesion  causes  astereognosis. 

ported  this  view  by.  other  cases.  The  area  involved  is  somewhat 
higher  than  the  supramarginal  gyrus,  which  obviates  the  objection  of 
Dejerine  that  in  lesions  there  he  has  not  seen  the  symptom. 

A  loss  of  tactile  memories  may  therefore  occur  as  a  symptom  of 
disease  in  the  sensory  area  of  the  cortex,  and  it  is  a  valuable  localizing 
symptom.  It  forms  a  part  of  the  condition  of  apraxia,  to  which  we 
shall  allude  later.     It  is  the  essence  of  the  condition  of  astereognosis. 

Astereognosis  is  to  be  elicited  in  a  patient  by  placing  various  objects 
one  after  another  in  the  hand,  the  eyes  being  closed,  and  asking  their 
names  and  uses.  Each  hand  must  be  tested  separately  as  each  hemi- 
sphere contains  its  own  set  of  memories.  If  aphasia  is  present  names 
may  not  be  given,  but  by  signs  a  patient  can  usually  indicate  whether 
he  recognizes  an  object  and  its  uses  by  touch. 

Sensory  Symptoms  from  Subcortical  Lesions.  —  Hemiansesthesia,  hemi- 
analgesia,  hemithermo-ansesthesia,  and  hemiataxia  may  be  produced  not 
only  by  lesions  in  the  sensory  area  of  the  cortex,  but  also  by  an  inter- 
ruption in  the  sensory  tracts  conveying  these  sensations  from  the  body. 

In  studying  diseases  of  the  spinal  cord  we  have  already  seen  that 
these  sensations  ascend  by  separate  tracts.  Tactile  sensations  ascend 
partly  in  the  posterior  columns  and  partly  in  the  antero-lateral  tracts. 
Temperature  and  pain  sensations  ascend  in  the  antero-lateral  ascend- 
ing tract.  Muscular  sensations  ascend  partly  in  the  posterior  columns 
and  partly  in  the  direct  cerebellar  columns.  (See  Figs.  22  and  23, 
page  69.) 

It  is  necessary  to  trace  these  various  tracts  upward  from  the  cord  to 
the  cortex  of  the  sensory  area.  The  sensory  tracts  are  not  as  direct 
and  continuous  as  the  motor  tracts  through  the  nervous  system.  The 
study  of  ascending  degenerations  in  the  spinal  cord,  cerebral  axis,  and 
brain  has  shown  that  such  degenerations,  though  intense  and  extensive 

1  Wiener  klin.  Wochen.,  1893, 
-2  Brain,  1901. 


LOCAL  SYMPTOMS. 


Ill 


Fig.  62. 


Cor/^  Qi, 


ArUerwr.^ 


^Medulla- 

-Moior 
clecusjcbtLon 

/ 


Lateral pyrturudal 
tract. 


The  cerebral  axis.    The  course  of  the  motor  tract  {py). 


112         DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

just  above  a  transverse  lesion,  become  less  so  the  higher  we  get,  and 
hence  we  conclude  that  the  short  tracts  greatly  outnumber  the  long 
ones.  In  fact,  we  do  not  find  continuous  sensory  tracts  from  the  cord 
to  the  cortex.  We  have  a  series  of  short  connecting  tracts  interrupted 
by  gray  masses,  and  a  moment's  consideration  shows  tlie  reason  of  this 
anatomical  arrangement.  The  effect  of  a  sensation  in  any  part  of  the 
body,  especially  if  it  be  of  sufficient  importance  to  give  rise  to  pain,  is 
not  merely  a  conscious  perception.  The  primary  effect  is  a  multitude 
of  reflex  actions  entirely  below  the  sphere  of  consciousness.  Thus  an 
ordinary  painful  impression  results  in  the  withdrawal  of  the  limb,  is 

Fig.  53. 


The  projection  tracts  joining  the  cortex  with  lower  nerve  centres.  Sagittal  section,  showing  the 
arrangements  of  tracts  in  the  internal  capsule.  A,  tract  from  the  frontal  lobe  to  the  anterior  half  of  the 
capsule,  thence  in  part  to  the  optic  thalamus,  A^  and  in  part  to  the  pons,  and  thus  to  the  cerebellar 
hemisphere  of  the  opposite  side  ;  B,  motor  tract  from  the  central  convolutions  to  the  facial  nucleus  in 
the  pons  and  to  the  spinal  cord  ;  C,  sensory  tract  from  posterior  columns  of  the  cord,  through  the  pos- 
terior part  of  the  medulla,  pons,  crus,  and  capsule  to  the  parietal  lobe  ;  D,  visual  tract  from  the  optic 
thalamus  (OT)  to  the  occipital  lobe  ;  E,  auditory  tract  from  the  int.  geniculate  body  (to  which  a  tract 
passes  from  the  VIII.  N.  nucleus)  to  the  temporal  lobe  ;  F,  superior  cerebellar  peduncle ;  G,  middle 
cerebellar  peduncle  ;  H,  inferior  cerebellar  peduncle;  ON,  caudate  nucleus ;  CQ,  corpora  quadrigemina. 
The  numerals  refer  to  the  cranial  nerves. 


setting  up  of  vasomotor  and  trophic  reflex  acts,  or,  if  the  irritation  be 
kept  up,  of  a  general  sense  of  uneasiness  throughout  the  body,  even 
complex  reflex  acts  to  remove  the  source  of  pain,  a  quickening  ot 
respiration  and  pulse^  a  facial  expression  of  discomibrt,  and  even  auto-' 


LOCAL  SYMPTOMS. 


113 


matic  cries.  All  these  effects  can  be  produced  in  an  animal  whose 
cortex  is  removed  and  in  a  man  asleep  or  under  ether.  It  is  therefore 
evident  that  an  intense  sensory  impression  may  throw  into  activity  the 
entire  subcortical  nervous  system,  or,  in  other  words,  may  be  distrib- 
uted to  a  multitude  of  motor  mechanisms  in  cord  and  cerebral  axis  and 


Fig.  54. 


The  tract  of  muscular  sense — the  interolivary  tract. 

subcortical  ^an^Yia  in  addition  to  awakening  cortical  activity  and  caus- 
ing a  conscious  i)erception.  This  effect  could  not  be  attained  were  the 
sensory  tracts  from  one  region  of  tlie  body  to  the  cortex  continuous 
and  single.  It  could  only  be  attained  by  an  arrangement  which  pro- 
vides for  the  termination  of  a  single  sensory  tract  at  various  levels,  a 
8 


114        DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

mechanism  which  disseminates  a  single  sensation  to  a  number  of  differ- 
ent motor  centres.  The  diagram  (Plate  I.,  frontispiece)  shows  the  ex- 
istence of  a  sensory  tract  made  up  of  a  number  of  segments  each  joined 
to  the  next,  but  each  having  interpolated  between  it  and  the  next  a 
neurone  capable  of  turning  aside  some  of  the  impulses  received  into 
side  channels. 

Fig.  65. 


'  ^.> 


.^ 


fheLeinm-' 


't) 


Lemiuxuj 
]ied:iuu:L?iis 

Cerebri 


l.r/'/r'!SruJ 


mm  - 


The  LirnjuMUi^ 


Lemniscus 
Ports 


IMM 


*%  W/,^ 


<^i 


I  he  Xem/usm  > 


-r 


T'071S  ' 

-Li'/nriiscus. 


fliKwulj^ 


The  tract  of  muscular  sense  —  the  lemniscus. 


The  Sensory  Tract.  — With  these  facts  in  mind  it  is  possible  to  trace 
the  sensory  tract  upward  and  to  understand  the  symptoms  which  Avill 
present  themselves  when  it  is  injured.     It  will  then  be  clear  that  from 


LOCAL  SYMPTOMS.  115 

the  distribution  of  the  symptoms  of  antesthesia  it  is  often  possible  to 
locate  a  lesion  in  the  sensory  tract. 

1.  The  Tract  of  Muscular  Sense.  —  The  posterior  columns  of  the  cord 
end  in  the  nuclei  gracilis  and  cuneatus  of  the  medulla  (Fig.  54),  each 
axone  terminating  in  a  brush  about  the  neurone  bodies  which  form 
these  nuclei.  From  these  neurones  axones  pass  upward,  crossing  over 
in  the  sensory  decussation  to  form  the  opposite  interolivary  tract  which 
lies  between  the  olives.  The  hypoglossal  nerves  on  their  way  from  the 
nucleus  to  their  exit  lie  on  the  outer  side  of  this  tract.  Ascending  in 
this  tract  the  sensory  fibres  enter  the  lemniscus,  which  lies  in  the  pons 
Varolii  behind  the  pyramidal  tracts  and  deep  transverse  fibres.  Many 
axones  branch  from  the  lemniscus  to  end  about  neurones  lying  in  the 
gray  matter  of  the  pons,  both  in  the  floor  of  the  ventricle,  in  the 
formatio  reticularis,  and  in  the  deep  gray  matter.  From  these  neu- 
rones axones  again  enter  the  lemniscus  to  ascend.  The  lemniscus  ascends 
(Fig.  55)  through  the  crus  cerebri,  where  it  curves  about  the  lateral 
surface,  sending  many  of  its  axones  into  the  corpora  quadrigemina 
posterior  and  anterior,  then  sinks  into  the  base  of  the  brain,  and,  pass- 
ing through  the  lower  part  of  the  internal  capsule,  terminates  largely 
in  the  optic  thalamus  in  its  outer  nucleus.  (Fig.  53.)  It  is  possible 
that  all  the  lemniscus  fibres  end  there.  It  is  possible  that  some  pass 
up  through  the  capsule  and  turn  outward  to  end  in  the  cortex  of  the 
posterior  central  convolution  and  adjacent  parietal  region.  It  is  pos- 
sible that  the  only  sensory  fibres  entering  the  cortex  come  from  the 
optic  thalamus,  but  many  authors  have  traced  the  lemniscus  directly  to 
the  cortex,  at  least  in  part. 

In  1884  I  showed,^  by  an  analysis  of  twenty-six  cases  of  very  small 
local  lesions  limited  to  the  medulla  and  pons, "that  an  interruption  in 
these  fibres  either  (1)  in  the  posterior  columns  of  the  cord ;  (2)  in  the 
interolivary  tract;  (3)  in  the  lemniscus,  or  (4)  in  the  internal  capsule 
where  the  lemniscus  passes  produces  a  condition  of  ataxia  and  loss  of 
the  muscular  sense  in  the  limbs  of  the  opposite  side.  Since  that  date 
many  similar  cases  of  small  lesions  in  the  lemniscus  have  been  reported 
with  the  same  result.  Hence  it  may  be  stated  that  this  is  the  tract  of 
muscular  sense  and  that  a  lesion  of  this  tract  causes  ataxia. 

If,  therefore,  hemiataxia,  not  of  cortical  origin,  occurs,  the  conclu- 
sion can  be  drawn  that  the  lemniscus  is  affected.  The  position  of  the 
lesion  in  the  course  of  the  lemniscus  can  be  determined  only  by  a 
study  of  other  symptoms  coincident  with  the  ataxia.  As  the  medulla, 
pons,  and  crura  are  very  small  and  contain  the  nuclei  of  the  various 
cranial  nerves  at  different  levels  some  symptoms  referable  to  an  affec- 
tion of  one  of  these  cranial  nerves  will  usually  enable  one  to  locate  the 
lesion.  Thus,  if  there  is  oculomotor  paralysis  with  hemiataxia  the 
lesion  is  in  the  crus.  If  there  is  trigeminal  anaesthesia  or  facial  palsy 
with  hemiataxia  the  lesion  is  in  the  pons.  If  there  is  auditory  or 
hypoglossal  paralysis  the  lesion  is  in  the  medulla.  The  figures  indicate 
the  course  of  this  tract  and  its  relation  to  the  various  cranial  nerves. 

2.  The  Tract  of  Tactile,  Pain  and  Temperature  Senses.  —  The  ascend- 

'  Journal  of  Nervous  and  Mental  Disease,  July,  1884. 


116       DIAGNOSIS    AND   LOCALIZATION    OF   BEAIN    DISEASES. 

Fig.  56. 


£^<?-^ 


'"ieste/orm,  body 


nuc.^raciJis 

71UC  curiea/us 
^Zaieml  column/ 


yyhic/L  /ux-vc  crossed 
i-n,  tfie  ccrrd . 


The  sensory  tract  tbrough  the  cerebral  axis. 


LOCAL  SYMPTOMS. 


117 


Fig.  57. 


V  nerve 


ing  sensory  fibres  in  the  lateral  columns  of  the  spinal  eord  which  convey 
these  sensations  pass  directly  into  the  corresponding  portion  of  the 
medulla  oblongata  which  is  known  as  the  formatio  reticularis.  This 
is  shown  in  Fig.  5G.  It  is  made  up  of  the  interlacing  of  three  sets 
of  fibres  :  (a)  transverse,  the  commissural  fibres  of  the  medulla,  pons, 
and  cerebellum ;  (6)  vertical,  the  cranial  nerve  fibres  ;  (c)  longitudinal, 
the  sensory  tract ;  and  hence,  however  it  is  cut,  it  appears  as  a  net- 
work or  reticular  formation,  whence  the  name.  .  It  is  strewn  with 
isolated  neurone  bodies  and  has  collections  of  these  at  many  points ; 
thus  the  nucleus  ambiguus,  the  superior  olive,  the  nuclei  of  the  lem- 
niscus, and  the  red  nucleus  are  collections  of  neurones  lying  in  this 
formation.  The  formatio  reticularis  is 
continuous  from  the  medulla  through 
the  pons  and  into  the  cms,  whence 
its  fibres  enter  the  internal  capsule. 
Through  this  formatio  reticularis  many 
fibres  pass  up  and  some  fibres  end. 
From  it  many  ascend  through  the  crus 
to  enter  the  optic  thalamus.  The  for- 
matio reticularis,  therefore,  contains  a 
long  ascending  tract.  The  sensations 
sent  along  this  from  the  spinal  cord, 
like  those  in  the  lemniscus,  pass  either 
indirectly  by  way  of  the  thalamus  or 
directly  by  way  of  the  internal  capsule 
to  the  sensory  cortex.  The  ascending 
tract  of  Gowers  in  the  spinal  cord 
takes  the  same  course  into  the  formatio 
reticularis.  It  ends  in  the  vermis  of 
the  cerebellum.  In  the  outer  third  of 
the  formatio  reticularis  is  found  a  col- 
umn of  small  cells  of  peculiar  structure, 
resembling  the  substantia  gelatinosa  of 
the  posterior  horn  of  the  spinal  cord, 
and  in  this  column  terminate  the  fibres 
of  the  sensory  part  of  the  trigeminal 
nerve  (Fig.  57)  which  turn  downward  after  entering  the  pons  Varolii. 
Thus  the  outer  portion  of  the  formatio  reticularis  contains  the  sensory 
tract  from  the  face  of  the  same  side. 

Lesions  of  the  formatio  reticularis  interrupt  the  sensory  tract  and 
cause  anaesthesia.  It  is  evident  from  the  diagram  that  a  lesion  which 
involves  the  formatio  reticularis  on  one  side  of  the  pons  and  medulla 
will  produce  an  alternating  anaesthesia  —  i.  e.,  loss  of  sensation  in  the 
face  on  the  side  of  the  lesion  and  in  the  body  on  the  other  side. 
Alternating  anaesthesia  is  as  characteristic  a  symptom  of  lesions  of  the 
formatio  reticularis  of  the  pons  and  medulla  as  alternating  paralysis  is 
of  lesions  in  the  motor  tract  in  the  pons. 

In  the  upper  });ii-t  of  tlic  ])ons  tlie  sensory  tract  from  the  face 
crosses  tlie  median  line,  and  hence  a  lesion  in  the  formatio  reticularis 


The  sensory  tract  in  the  crus,  pons,  and 
medulla,  showing  nucleus  and  roots  of  V 
nerve.  A,  lesion  causes  right  hemianses- 
thesia  ;  B,  lesion  causes  alternating  hemi- 
ansesthesia ;  left  face  and  right  side  of  body. 


118         DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

in  the  upper  third  of  the  pons,  or  in  the  crus  cerebri,  or  in  the  internal 
capsule  will  produce  a  unilateral  hemianaesthesia.  A  lesion  involving 
both  halves  of  the  formatio  reticularis  will  produce  bilateral  sensory 
symptoms. 

The  diagrams  show  the  relation  of  this  tract  to  the  cranial  nerves 
which  make  their  exit  through  the  formatio  reticularis.  A  lesion 
which  is  situated  in  the  crus,  pons,  or  medulla,  causing  anaesthesia  of 
the  body,  will  also  cause  some  symptoms  referable  to  one  or  more  of 
the  cranial  nerves,  and  hence  such  a  lesion  can  readily  be  localized. 

Hemianaesthesia  is  usually  due  to  a  lesion  of  the  sensory  tract  in  the 
internal  capsule,  where  it  lies  either  just  behind  the  motor  tract  or 
mingled  with  it.  Such  a  hemianaesthesia  is  usually  attended  by  hemi- 
analgesia  and  hemithermo-anaesthesia  and  often  by  hemiataxia.  A 
dissociation  of  sensations,  that  is,  the  loss  of  either  touch,  pain,  or  tem- 
perature sense  alone,  the  others  being  intact,  has  not  been  observed 
after  lesions  of  the  internal  capsule.  Hemianaesthesia  from  lesions  of 
the  capsule  is  rarely  absolute,  for  the  sensory  decussation  is  not  a  com- 
plete one,  and  sensations  from  one-half  of  the  body  are  sent  to  both 
hemispheres  of  the  brain.  As  a  rule,  the  entire  half  of  the  body  is 
anaesthetic  after  a  lesion  of  the  capsule.  But  as  the  sensory  tract 
ascends  to  the  cortex  from  the  capsule  its  fibres  spread  out  like  the 
sticks  of  a  fan,  those  transmitting  sensations  from  the  leg  passing  up- 
ward to  the  summit  of  the  sensory  area  of  the  cortex,  while  those 
transmitting  sensations  from  the  face  pass  outward  to  the  vicinity  of 
the  Sylvian  fissure.  Hence  in  lesions  in  the  centrum  ovale,  as  in 
lesions  of  the  cortex,  one  limb  or  part  of  the  body  may  be  anaesthetic 
instead  of  the  entire  side.  And  the  same  rule  applies  to  symptoms  of 
loss  of  sensation  as  to  paralysis :  that  the  nearer  the  lesion  to  the  cor- 
tex the  more  likely  are  the  symptoms  to  be  limited  to  a  single  limb. 
Thus  in  the  cases  shown  in  Figs.  42  and  43  there  was  a  loss  of  sensa- 
tion as  well  as  of  motion  in  the  hand  and  arm  of  the  opposite  side. 

When  the  close  approximation  of  the  sensory  to  the  motor  tract 
throughout  its  course  is  considered  it  is  no  longer  a  matter  of  surprise 
that  in  the  majority  of  cases  of  brain  disease  a  loss  of  sensation  attends 
paralysis.  It  is  to  be  noticed,  however,  that  in  all  this  course  the  sen- 
sory tract  lies  behind  the  motor  tract.  If,  therefore,  a  lesion  is  ad- 
vancing from  behind  forward  in  the  brain  sensory  disturbance  precedes 
paralysis,  but  if  the  lesion  is  advancing  from  before  backward,  paralysis 
precedes  anaesthesia.  This  observation  has  an  important  bearing  upon 
diagnosis,  for  it  sometimes  enables  one  to  determine  whether  the  dis- 
ease under  view  is  a  progessive  or  a  stationary  one,  and,  if  progressive, 
to  settle  the  direction  in  which  it  is  extending.     (See  Fig.  53.) 

3.  The  Cerebellar  Tract  from  the  Spinal  Cord. —  The  direct  cerebellar 
tract  from  the  cord  turns  through  the  corpus  restiforme  of  the  medulla 
outward  and  goes  directly  to  the  cerebellum.  (Fig.  54.)  Some 
fibres  from  the  nuclei  gracilis  and  cuneatus  of  each  side  join  it  in  the 
corpus  restiforme  and  end  with  it  in  the  cerebellum.  It  is  also  joined 
by  fibres  from  the  opposite  olivary  body.  These  tracts,  therefore,  do 
not  reach  the  sensory  cortical  centres,  and  hence  impulses  passing  along 
them  have  nothing  to  do  with  sensations  of  touch,  temperature,  and 


LOCAL  SYMPTOMS. 


119 


pain,  or  with  those  muscular  sensations  which  are  destined  to  guide 
voluntary  movements.  They  are  concerned  in  the  transmission  of 
those  muscmar  sensations  which  are  essential  to  the  control  of  equi- 

FiG.  58. 


The  visual  tract.  The  result  of  a  lesion  anywhere  between  the  optic  chiasm  and  the  cuneus  is  to 
produce  homonymous  hemianopsia.  H,  lesion  at  chiasm  causing  bilateral  temporal  hemianopsia;  N, 
lesion  at  chiasm  causing  unilateral  nasal  hemianopsia  ;  T,  lesion  at  chiasm  causing  unilateral  temporal 
hemianopsia ;  8N,  substantia  nigra  of  crus ;  L,  lemniscus  in  crus ;  RN,  red  nucleus ;  III,  third 
nerves. 

librium,  as  we  shall  see  later  when  we  consider  the  connections  and 
functions  of  the  cerebellum.     It  is  not  to  be  forgotten,  however,  that 


120        DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

in  lesions  affecting  the  medulla  and  pons  this  tract  may  be  injured,  and 
thus  vertigo  and  staggering  may  be  produced. 

Disturbance  of  Vision  is  a  local  symptom  of  cortical  disease  of  the 
occipital  lobe  of  the  brain  and  of  the  visual  tract  from  the  eyes  to  it. 
The  optic  nerves  pass  from  the  eyes  to  the  optic  chiasm.  A  partial 
decussation  of  the  optic  fibres  occurs  in  the  optic  chiasm,  leading  to 
the  junction  in  the  optic  tract  of  fibres  from  the  corresponding  half  of 
both  eyes.      For  this  reason  cerebral  blindness  is  always  a  hemianopsia 


Fig.  59 


Section  through  occipital  cortex.     Contrast  this  with  Figs.  33  and  45  tn  pages  S4  and96. 
(Chr.  Jacob,  Das  Menschenhirn.) 

or  blindness  in  one-half  of  both  eyes,  each  hemisphere  of  the  brain 
receiving  impressions  from  the  opposite  visual  fields.  The  exact 
anatomy  of  the  optic  nerves  and  optic  tracts  will  be  considered  in 
Chapter  XXXV.  Suffice  it  here  to  mention  that  each  optic  tract 
ends  in  the  pulvinar  of  the  optic  thalamus,  in  the  external  geniculate 
body,  and  in  the  corpus  quadrigeminum  anterior  (Fig.  58),  and  that 
from  these  ganglia  the  visual  tract  issues  into  the  posterior  fifth  of  the 


LOCAL  SYMPTOMS. 


121 


internal  capsule,  and  turning  backward  in  it  passes  through  the  cen- 
trum ovale,  outside  of  the  posterior  horn  of  the  lateral  ventricle,  and 
terminates  in  the  cortex  of  the  occipital  lobe.  Tlie  exact  termination 
of  these  fibres  is  in  the  cortex  about  the  calcarine  fissure  and  in  the 
cuneus,  a  wedge-shaped  lobule  on  the  median  surface  of  the  hemi- 
sphere. (Fig.  60.)  But  the  convolutions  of  the  convexity  of  the 
occipital  lobe  are  also  concerned  in  the  reception  and  storing  up  of 

Fig.  60. 


Lesions  of  the  occipital  lobe  causing  hemianopsia.     (From  Heuschen.) 

visual  impressions,  and  hence  lesions  in  any  part  of  the  occipital  cortex 
are  productive  of  hemianopsia. 

There  seems  to  be  a  projection  of  the  visual  field  to  some  extent 
upon  the  cortex  of  the  cuneus,  for  small  lesions  there  may  cause  small 
sector-like  defects  in  the  visual  field.  Henschen,  in  his  elaborate  work 
on  the  putliology  of  the  brain,  has  confirmed  this  conclusion,  which 
Wilbrandt  and  Hun   liad  reached  from  a  smaller  number  of  cases. 


122         DIAGNOSIS  AND  LOCALIZATION  OF  BBAiN  DlSHASES. 

Fig.  59,  17,  shows  the  location  of  the  lesion  in  Hun's  case.  The 
defect  in  the  visual  fields  in  this  patient  was  limited  to  the  upper 
quadrant.  Lesions  in  the  upper  part  of  the  cuneus  cause  blindness  in 
the  lower  quadrant  of  both  visual  fields,  and  lesions  in  the  lower  part 
of  the  cuneus  cause  blindness  in  the  upper  quadrant  of  both  visual 
fields.  It  is  rare  for  a  lesion,  however,  to  be  so  exactly  limited  as  to 
cause  this  effect.  As  a  rule,  lesions  anywhere  in  the  occipital  lobe 
produce  hemianopsia.  Superficial  lesions  in  the  occipital  cortex  may 
cause  a  loss  of  color  vision  only,  hemichromatopsia,  which  may  be 
partial,  that  is,  limited  to  one  or  two  colors  only,  or  total.  Such 
cases  are  very  rare  and  are  usually  soon  followed  by  hemianopsia.  A 
few  cases  have  been  reported  of  bilateral  lesions  causing  double 
hemianopsia.  In  one  such  case  central  vision  was  preserved  (Forster), 
in  others  the  blindness  was  total.  Total  blindness  has  also  been 
caused  by  a  maldevelopment  of  the  occipital  lobes  of  the  brain,  as  in 
cases  reported  by  Haab  and  Spiller. 

Lesions  of  the  cortex  produce  defects  of  the  visual  field  that  are 
symmetrical  in  both  eyes,  as  shown  in  Fig.  61. 

Psychical  Blindness.  —  The  loss  of  the  power  of  vision  is  attended  by 
a  loss  of  visual  memories  when  the  lesion  is  cortical,  and  hence  the 


Fig.  61. 


VI  KI 

The  visual  fields  in  a  case  of  left  homonymous  hemianopsia  of  cortical  origin. 

recognition  of  objects  seen  is  imperfect,  and  the  mind  cannot  recall  the 
appearance  of  things  formerly  familiar.  Thus  I  have  known  patients 
who,  after  such  a  lesion,  did  not  recognize  the  members  of  their  family 
by  sight,  though  they  recognized  them  by  the  sound  of  their  voices. 
This  condition  is  known  as  psychical  blindness.  The  patient  fails  to 
recognize  objects,  and  cannot  recall  them  to  mind. 

It  would  seem,  from  a  study  of  more  or  less  extensive  lesions  in  the 
occipital  cortex  as  if  the  memories  of  things  seen  were  largely  located 
in  the  left  hemisphere  in  right-handed  and  in  the  right  hemisphere  in 


LOCAL  SYMPTOMS.  12S 

left-handed  persons,  thus  according  with  the  location  of  our  memories 
of  speech,  ^t  any  rate,  the  loss  of  these  memories  is  much  more  fre- 
quent when  the  left  hemisphere  is  destroyed. 

The  major  part  of  our  knowledge  of  objects  is  acquired  through  the 
sense  of  sight,  and  a  moment's  thought  will  convince  one  not  only  that 
the  extent  of  cortex  occupied  by  memory  pictures  must  be  great,  but 
also  that  it  must  be  increasing  daily  through  life.  The  only  definite 
conception  we  can  have  of  this  process  is  that,  while  all  objects  are 
originally  perceived  through  impressions  sent  to  the  cuneus,  the 
memories  of  these  objects  are  stored  in  secondary  centres  outside  of  the 
cuneus  in  the  occipital  cortex.  Hence  the  larger  the  number  of  things 
seen  and  remembered  the  greater  the  area  concerned  in  vision  in  any 
individual. 

Fig.  62. 


The  area  of  the  cortex  in  which  a  lesion  causes  psychical  blindness. 

The  condition  known  as  psychical  blindness  is  due  to  a  loss  of  these 
memories  and  is  diagnostic  of  a  lesion  of  the  cortex  of  the  occipital 
lobe.  It  occurs  from  a  lesion  of  the  convexity  as  well  as  from  a  lesion 
of  the  cuneus.  It  is  usually  attended  by  hemianopsia,  always  when 
the  cuneus  is  destroyed.     (Fig.  62.) 

Word-blindness.  —  There  is  a  special  class  of  visual  memories,  those 
that  make  up  our  knowledge  of  written  and  printed  language,  which 
have  a  special  location.  This  is  in  the  junction  of  the  occipital  and 
parietal  regions  in  an  area  known  as  the  angular  gyrus.  Lesions  in 
this  locality  cause  a  loss  of  the  memory  of  words  as  seen ;  hence  an 
inability  to  read.  This  symptom,  while  really  a  part  of  psychical 
blindness,  is  termed  word-blindness,  and  may  occur  without  psychical 
blindness.  It  is  often  accompanied  by  hemianopsia,  because  a  lesion 
in  this  locality,  if  not  altogether  superficial,  may  involve  the  visual 
tract  which  lies  beneath  the.  cortex  here.  But  word-blindness  may 
occur  alone  without  hemianopsia.  It  may  be  attended  by  hemian- 
sethesia  or  hemiataxia,  but  in  such  cases  the  lesion  involves  the  cortex 
in  front  of  the  angular  gyrus.  Fig.  63  shows  the  exact  extent  of  the 
lesion  causing  word-blindness  in  six  cases.  Patients  suffering  from 
word-blindness  not  only  lose  the  power  of  reading,  a  condition  termed 
alexia,  but  sometimes  lose  also  the  power  of  writing,  a  condition 
termed  agraphia,  being  unable  to  call  up  the  proper  motor  images 
needed  to  form  the  word.     Yet  there  are  cases  on  record  where  alexia 


124        DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

has  occurred  without  agraphia,  and  I  have  seen  two  patients  who 
could  not  read  what  they  had  just  written.  In  such  cases  one  must 
imagine  that  the  sound  of  the  word  as  mentally  pronounced  awakened 
the  motor  memory  of  its  production  in  speech  and  in  writing,  even 
when  its  appearance  could  not  be  recalled.  Word-blindness  is  an  im- 
portant division  of  sensory  aphasia. 

Visual  Aura. —  Irritation  of  the  occipital  cortex  produces  forced  re- 
vivals in  consciousness  of  visual  memories.     These  may  consist  simply 


Fig.  63. 


(Dejerine.) 


(Jfacewen.) 


(Henschen.) 


(Pick.) 


(Broadbent.)  (Bernheim. 

Situation  of  lesions  causing  word-blindness;  alexia. 

of  flashes  of  light  or  color  or  of  actual  images.  They  are  known  as 
visual  hallucinations  when  consciousness  accepts  them  as  real  and  does 
not  distinguish  them  from  actual  objects  seen.  Such  hallucinations 
often  precede  a  Jacksonian  epilepsy  and  are  known  as  visual  aurse. 
They  are,  as  a  rule,  unilateral,  the  hallucination  aj)pearing  on  the  side 
opposite  to  the  lesion.  They  are  analogous  to  the  tingling  sensations 
known  as  the  signal  symptom  in  localized  spasm.     They  are  sometimes 


LOCAL  SYMPTOMS. 


125 


followed  by  temporary  hemianopsia,  the  excitement  of  the  cortical 
neurones  causing  an  exhaustion. 

It  is  prooable  that  the  visions  of  delirium,  whether  due  to  poisons, 
such  as  alcohol,  opium,  or  cannabis  indica,  or  due  to  inflammation  of 
the  membranes  of  the  brain  or  of  the  brain  itself,  are  produced  by 
irritation  of  the  visual  area  of  the  cortex. 

Subcortical  Hemianopsia.  —  Hemianopsia  may  be  due  to  a  lesion  in 
the  visual  tract  beneath  the  cortex  or  to  a  lesion  in  the  basal  ganglia 
in  which  the  optic  tract  terminates.  In  this  case  the  limit  of  blindness 
in  the  visual  field  is  usually  less  extensive  and  less  symmetrical  in  the 
two  eyes  than  when  the  lesion  is  cortical.  In  cortical  hemianopsia  the 
line  of  limitation  of  vision  is  usually  vertical  and  passes  within  one  or 
two  degrees  of  the  point  of  central  vision  in  both  eyes.  In  subcortical 
hemianopsia  the  line  is  irregular  and  passes  from  five  to  ten  degrees 
outside  the  point  of  central  vision  in  both  eyes.     (Fig.  64.) 

Fig.  64. 


VI  VI 

The  visual  fields  in  left  homonymous  hemianopsia  due  to  a  subcortical  lesion. 

Hemianopsia  due  to  subcortical  lesions  is  usually  attended  by  other 
symptoms.  Thus  if  it  is  right  hemianopsia  and  the  lesion  is  just 
beneath  the  cortex  at  any  point  in  the  temporo-occipital  radiation  some 
form  of  word-blindness  or  aphasia  is  a  very  common  symptom.  If  the 
lesion  is  in  the  region  of  the  internal  capsule  or  optic  thalamus,  hemi- 
ataxia  or  hemiansesthesia  is  very  often  an  associated  symptom.  This 
is  due  to  the  proximity  in  this  locality  of  tlie  sensory  tract.  Hemi- 
plegia may  also  accompany  it  from  the  same  cause.  If  the  hemianopsia 
is  due  to  a  lesion  in  the  corpus  geniculatum  externum  the  fibres  of  the 
corpora  quadrigemina  are  involved  and  the  reflex  acts  of  the  pupils 
are  affected  as  they  are  in  lesions  of  the  optic  tract.  Then  the 
hemiopic  pupillary  reflex  of  Wernicke  can  be  elicited.  (See  Chapter 
XXXV.)  Lesions  limited  to  the  corpora  quadrigemina,  while  caus- 
ing disturbances  in  the  movement  of  the  two  eyes,  double  vision,  and 


126        DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

imperfect  pupillary  reflexes,  do  not,  when  alone,  cause  any  loss  of 
vision.  They  produce  vertigo  and  a  staggering  gait,  like  cerebellar 
disease,  from  implication  of  the  red  nuclei  lying  under  the  corpora 
quadrigemina. 

Hemianopsia  may  be  due  to  a  lesion  in  the  optic  tract  on  one  side. 
It  is  then  associated  with  Wernicke's  hemiopic  pupillaiy  reflex.  The 
optic  tract  lies  upon  the  cms,  and  lesions  affecting  it  often  simultane- 
ously affect  the  motor  tract,  and  cause  hemiplegia,  and  also  involve  the 
oculomotor  nerve,  causing  ptosis  and  double  vision.  The  forms  of 
hemianopsia  due  to  lesions  in  the  optic  chiasm  are  described  in 
Chapter  XXXV. 

Lesions  of  the  occipital  lobe,  or  of  the  internal  capsule,  or  of  the 
basal  ganglia  do  not  cause  blindness  of  one  eye,  as  was  formerly  held 
by  Charcot  and  Ferrier.  A  blindness  in  one  eye  or  a  concentric 
diminution  of  the  visual  field  of  one  eye  is  due  either  to  functional 
disturbance  (hysteria)  or  to  a  lesion  of  the  optic  nerve,  visible  by  the 
ophthalmoscope. 

Disturbance  of  Hearing  is  rarely  due  to  disease  in  the  brain  unless 
this  disease  affects  both  temporal  lobes  or  the  tracts  to  them.  Each 
ear  is  connected  with  both  temporal  lobes,  hence  a  unilateral  lesion 
fails  to  cause  total  deafness,  and  there  is  no  such  thing  as  a  partial 
cortical  deafness  to  high  or  low  sounds  or  tones  which  might  be 
analogous  to  hemianopsia  or  hemichromatopsia.  A  cortical  deafness, 
therefore,  has  not  been  observed  excepting  in  a  few  rare  cases  where 
both  temporal  lobes  have  been  destroyed.  In  these  cases  the  patient 
has  become  totally  deaf.  The  exact  localization  of  the  auditory  centres 
is  in  the  middle  part  of  the  first  temporal  convolution,  and  extends  to 
the  adjacent  cortex  within  the  Sylvian  fissure  and  over  the  convexity 
into  the  second  temporal  convolution.  In  cases  of  congenital  deaf 
mutes  these  parts  are  found  to  be  atrophied.  In  some  cases  of  irritat- 
ing lesions  of  this  area  Jacksonian  attacks  of  epilepsy  have  been  pre- 
ceded by  an  auditory  aura,  loud  sounds,  bells,  whistles,  etc.,  being 
heard  just  as  the  attack  was  coming  on. 

Psychical  Deafness.  —  Our  auditory  perceptions,  like  those  of  sight, 
leave  behind  them  a  trace  which  constitutes  our  auditory  memory. 
We  have  such  memories  of  sounds,  of  musical  melodies  and  harmonies, 
and  also  of  words  heard.  Hence  lesions  in  the  auditory  area  may 
deprive  a  patient  of  these  memories,  even  though  he  may  retain  his 
power  of  hearing.  It  is  an  extraordinary  fact  that  our  memories  of 
things  heard  appear  to  be  stored  in  one  hemisphere  only,  the  left  in 
right-handed  and  the  right  in  left-handed  persons.  Hence  psychical 
deafness  and  amusia  and  word-deafness  are  symptoms  of  a  unilateral 
lesion  of  the  temporal  cortex. 

A  patient  with  psychical  deafness  no  longer  recognizes  any  noise  or 
sound.  The  bark  of  a  dog,  the  cry  of  any  animal,  the  sound  of  waves, 
or  bells,  or  whistles,  the  voice  of  a  familiar  person  no  longer  awakens 
recognition,  and  such  sounds  cannot  be  called  to  mind. 

A  patient  with  amusia,  which  is  a  variety  of  psychical  deafness,  no 


LOCAL  SYMPTOMS.  127 

longer  recognizes  melodies  and  harmonies  with  which  he  was  familiar. 
He  no  long^  takes  pleasure  in  hearing  music  or  operas  in  which  he 
may  formerly  have  delighted,  and  all  music  seems  to  him  new  and 
strange.  He  cannot  call  to  mind  familiar  tunes,  and  hence  cannot  sing 
or  hum  them. 

Word-deafness.  —  A  patient  with  word-deafness,  which  may  occur 
independently  of  or  in  connection  with  amusia  and  mind-deafness,  is 
no  longer  able  to  recall  the  names  of  objects,  and  is  no  longer  able  to 
understand  spoken  language.  He  is  like  one  who  hears  an  unknown 
tongue.  He  hears  the  sounds  of  voices,  but  attaches  no  meaning  to 
what  is  said.  He  may  be  able  to  read  and  he  may  be  able  to  speak  in 
the  sense  of  saying  words  correctly,  but  he  usually  talks  jargon,  as  the 
sounds  no  longer  guide  his  talking.  This  is  the  second  and  more 
common  form  of  sensory  aphasia. 

Our  auditory  memories  of  words  are  the  first  to  be  acquired  in 
infancy,  and  long  before  an  infant  can  talk  he  has  learned  to  associate 
the  sound  of  names  with  their  corresponding  objects.  The  memory 
pictures  of  objects,  acquired  chiefly  by  sight,  but  also  by  all  other 
senses,  are  the  fundamental  basis  of  thought  in  all  animals,  and  these, 
grouped  together,  form  the  concept  of  each  object  which  they  know. 
To  this  concept  is  soon  joined  by  association  the  sound  of  its  name ; 
later,  in  man,  the  motions  needed  to  produce  this  sound  are  learned ; 
lastly,  the  appearance  of  the  letters  making  this  word,  and  finally,  the 
motions  necessary  to  write  the  word.  The  auditory  memories  are  not 
only  the  first  speech  elements  acquired,  they  are  the  most  important. 
■Reading  and  writing  are  subordinate  to  speech,  a  result  of  the  later 
evolution  of  language,  and  are  an  evidence  of  education,  not  of  natural 
acquisition.  The  sound  of  objects  in  nature  has  been  taken  to  indicate 
these  objects  in  the  early  evolution  of  speech,  and  is  still  so  in  the  edu- 
cation of  many  infants.  The  baby  learns  "  bow-wow  "  before  it  learns 
"  dog."  The  study  of  language  reveals  that  a  large  number  of  words 
—  e.  g.,  murmur,  whisper,  puflp,  bang  —  have  a  similar  origin.^  As 
our  knowledge  of  the  names  of  objects  is  constantly  growing,  as  we 
acquire  new  languages,  it  is  evident  that  our  auditory  memories  are 
constantly  increasing  in  nunJber.  We  must  believe,  therefore,  that  the 
cortical  area  in  which  they  are  stored  varies  in  size  in  different  persons, 
according  to  the  degree  of  education  in  language.  This  is  proven  by 
cases  of  gradually  advancing  diseases  in  this  area.  I  have  a  patient 
who,  as  such  disease  went  on,  lost  first  his  memory  of  English,  then  of 
German,  and  finally  of  French,  the  latter  being  his  native  tongue. 
The  names  of  objects  first  acquired  appear  to  persist  longest  in  memory. 
Thus  a  patient  who  is  word-deaf  will  often  comprehend  single  words 
when  he  cannot  understand  a  sentence,  and  one  finds  one's  self  uncon- 
sciously talking  to  an  aphasic  very  much  as  one  talks  to  an  infant. 
The  condition  of  word-deafness  may  be  and  usually  is  incomplete,  some 
words  being  recognized  and  remembered  and  others  forgotten.  Some 
patients  retain  nouns,  but  forget  adjectives  and  verbs  and  prepositions. 

'  Trench,  On  the  Study  of  Words.     Macmillan  <&  Co, 


128 


DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 


Others  lose  all  uouns  and  talk  in  roundabout  phrases.  Thus  one  of 
my  patients  could  not  name  a  knife  or  a  scissors,  but  said  "  it  is  the 
thing  you  cut  with."  Occasionally  all  the  word-memories  are  lost 
and  nothing  that  is  heard  is  understood. 


Fig.  65. 


(Girandeau.) 


(\Vernicke.) 


(Eichhorst. ) 


(Claus.) 


(Seppilli.)  (Hitzig.) 

Situation  of  lesions  causing  word-deafness  ;  sensory  apliasia. 

The  lesion  of  word-deafness  is  located  in  the  cortex  and  subcortical 
region  just  beneath  it  in  the  first  and  second  temporal  convolutions,  an 
area  a  little  wider  than  that  concerned  in  hearing  alone.  Fig.  65 
shows  the  location  of  the  lesion  in  six  cases  of  this  kind. 

The  Auditory  Tract.  —  Deafness  from  subcortical  lesions  is  a  rare 
symptom.     Fig.  66  shows  the  course  of  the  auditory  nerve  impulses 

Description  of  Fig.  66. 
The  auditory  tract.  TH,  optic  thalamus;  INT,  int.  capsule;  CI,  Corp.  geniculatum  int.;  TEM, 
temporal  lobe;  L,  lemniscus;  CQA,  corp.  quadrigeminuni  ant.;  CQP,  corp.  quad,  post.;  R,  red  nucleus 
of  tegmentum;  SN,  substantia  nigra;  CE,  corp.  geniculatum  ext.;  P,  pes  pedunculi  and  pyramidal 
tract-  NU  nucleus  of  lemniscus;  VEN,  ventral  nucleus  of  auditory  nerve;  V,  fifth  nerve;  OL,  olivary 
nucleus  ■  O,  olive ;  X,  decussation  of  auditory  fibres ;  AC,  auditory  nerve  from  cochlea ;  T,  dorsal 
nucleus  of  auditory  nerve. 


Fig.  66. 


CE 


The  auditory  tract  from  ear  to  brain. 


180         DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

from  their  entrance  into  the  medulla  to  their  termination  in  the  cortex. 

The  neurone  bodies,  of  which  these  fibres  are  the  axones,  lie  in  the 
spiral  canal  of  the  cochlea.  It  is  the  cochlear  division  only  of  the 
eighth  nerve  which  has  to  do  with  hearing. 

A  C,  the  acoustic  nerve  fibres,  enter  the  side  of  the  medulla  in  a  large 
trunk.  We  can  distinguish  fourteen  diiferent  sets  of  fibres.  Many  of 
these  fibres  (1-8)  penetrate  the  medulla  opposite  the  entrance  of  the 
nerve.  Others  (9-14)  turn  upward  on  entering,  and  penetrate  the 
pons  at  a  little  higher  level.  These  various  fibres  pass  to  diiferent 
destinations;  1  crosses  through  the  olive,  turns  upward  in  the  inter- 
olivary  tract,  and  enters  the  opposite  lemniscus,  and  passes  up  in  it; 
2  terminates  about  a  neurone  body  in  the  ventral  nucleus  (VEN). 
From  this  body  an  axone  arises  which  crosses  to  the  opposite  side  and 
turns  upward  in  the  lemniscus ;  3  terminates  about  a  neurone  body  in 
the  ventral  nucleus  (VEN).  From  this  body  an  axone  arises  which 
passes  upward  in  the  lemniscus  of  the  same  side ;  4  terminates  about 
a  neurone  body  in  the  ventral  nucleus  (VEN).  From  this  body  an 
axone  arises  which  passes  about  the  outer  side  of  the  medulla  through 
the  tuberculum  acusticum  (T),  and  thence  through  the  formatio  retic- 
ularis of  the  medulla  to  the  raphe,  where  it  crosses  to  the  opposite 
side  and  turns  upward  in  the  lemniscus ;  5  terminates  about  a  neurone 
body  in  the  dorsal  nucleus  or  tuberculum  acusticum  (T).  From  this 
body  an  axone  passes  through  the  formatio  reticularis  into  the  lemniscus 
of  the  same  side  and  turns  upward  in  it ;  6  terminates  about  a  neurone 
body  in  the  dorsal  nucleus  (T).  From  this  body  an  axone  passes 
through  the  formatio  reticularis,  crosses  in  the  raph6  and  enters  the 
lemniscus  of  the  opposite  side,  turning  upward  in  it;  7  terminates 
about  a  neurone  body  in  the  dorsal  nucleus  (T).  From  this  body  an 
axone  passes  across  the  floor  of  the  fourth  ventricle  in  the  striae  acusticse 
to  the  raph6,  turns  downward  in  it,  crosses  to  the  other  side,  enters 
the  lemniscus  and  turns  upward  in  it;  8  passes  through  the  dorsal 
nucleus  into  the  strise  acusticse  and  accompanies  7  in  its  course. 

The  ventral  nucleus  of  the  auditory  nerve  consists  of  a  long  column 
of  cells  which  extend  upward  into  the  pons.  Hence  in  a  section 
through  the  lower  half  of  the  pons  the  ventral  nucleus  is  still  visible 
(VEN)  and  the  fibres  ascending  from  the  auditory  nerve  trunk  to 
terminate  in  or  to  pass  through  this  nucleus  (fibres  9-14)  are  easily 
traced.     The  course  and  ending  of  these  fibres  is  as  follows  : 

No.  9  turns  inward  and  ends  about  a  neurone  body  lying  in  the 
upper  olivary  nucleus  (OL).  From  this  body  an  axone  arises  which 
crosses  the  median  line  in  the  trapezium,  enters  and  ascends  in  the 
opposite  lemniscus ;  10  terminates  about  the  neurone  body  in  the  ven- 
tral nucleus,  wlience  a  new  axone  arises  and  passes  to  the  olivary 
nucleus,  terminating  about  a  neurone  body  there.  From  this  neurone 
body  an  axone  arises  which  joins  9  and  pursues  the  same  course;  11 
passes  through  the  ventral  nucleus  and  ends  about  a  neurone  body  in 
the  mass  of  gray  matter  lying  adjacent  to  but  ventral  of  the  lemniscus, 
and  dorsal  of  the  olivary   nucleus.     This  is   the  trapezoid  nucleus. 


LOCAL  SYMPTOMS. 


131 


From  its  neurone  bodies  axones  arise,  some  of  which  enter  the  lemnis- 
cus of  the  same  side,  but  many  of  which  cross  the  median  line  to  enter 
the  lemniscus  of  the  opposite  side  and  ascend  in  it ;  12  terminates  in 
the  ventral  nucleus  about  a  neurone  body.  This  body  sends  its  axone 
to  the  collection  of  neurones  lying  within  the  deep  transverse  fibres  of 
the  pons,  the  trapezoid  body.  These  neurone  bodies  in  turn  send  their 
axones  into  the  lemniscus  of  the  same  and  of  the  opposite  side;  13 
terminates  about  a  neurone  body  in  the  ventral  nucleus.  The  body 
sends  an  axone  directly  into  the  lemniscus  of  the  same  side  ;  14  passes 
through  the  ventral  nucleus  and  crosses  in  the  trapezoid  from  the 
opposite  side,  where  it  turns  upward  in  the  lemniscus. 

It  is  thus  evident  that  all  the  fibres  of  the  acoustic  nerve,  so  far  as 
its  cochlear  division  is  concerned,  transmit  their  impulses  into  the 
lemniscus  of  the  same  or  of  the  opposite  side.  The  trapezoid  fibres 
may  be  termed  the  acoustic  decussation  or  chiasm,  and,  as  in  the  optic 


Fig.  67. 


Diagram  of  the  course  of  the  auditory  tract.  VTTT,  auditory  nerve  nuclei  and  fibres  ;  fra,  fascicu- 
lus reflectorius  acustieus ;  cts,  corpus  trapezoides  ;  ols,  olive  superior ;  cpb,  corpus  paratrigeminus ; 
cap,  corpus  quadrigeminus  posterior  ;  crja,  corpus  quadrigeminus  anterior  ;  cgm,  corpus  geniculatum 
anterior;  brp,  brach.  conjunctivum;  Ihal,  thalamus.     (Jacob.) 

chiasm,  the  majority  of  the  fibres  cross  to  the  opposite  side  (X).  The 
termination  of  fibres  ascending  in  the  lemniscus  is  very  complex,  (a) 
Some  fibres  terminate  about  the  cells  of  the  nucleus  lemuisci  in  the 
pons  (Nu)  which  nucleus  in  turn  sends  axones  to  the  corpora  quadri- 
gemina  of  the  same  and  of  the  opposite  side  (15).  (6)  Some  fibres 
terminate  about  the  large  quadripolar  cells  of  the  posterior  corpus 
quadrigeminum  (16)  (CQP).  (c)  Some  fibres  terminate  about  the  large 
cells  of  the  first  layer  of  cells  in  the  anterior  corpus  quadrigeminum 
(17)  (CQA).  (d)  Many  fibres  terminate  about  neurone  bodies  in  the 
corpus  geniculatum  internum  (CI),  whence  new  axones  arise  which 
pass  to-  the  cortex  of  the  temporal  lobe  (TEM).  (e)  Some  fibres  pass 
directly  through  the  internal  capsule  from  the  lemniscus  to  the  temporal 
lobe.     (See  Fig.  67.) 

Since  each  of  the  nuclei  in  which  lemniscus  fibres  terminate  is  cout 
nected  with  motor  mechanisms  as  well  as  with  the  cortex  of  the  temr 
poral  lobe,  it  is  evident  that  the  auditory  impulses  can  awaken  numerr 


132        DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

ous  reflex  and  automatic  acts  as  well  as  conscious  sensations  of  hearing. 
Hence  the  act  of  turning  eyes  and  head  or  assuming  postures  of 
strained  listening  and  other  automatic  acts  are  made  possible  by  these 
fibres. 

Fig.  QQ  shows  that  the  connection  of  each  ear  is  with  both  sides 
of  the  brain,  but  that  the  crossed  connection  is  more  extensive  than 
that  with  the  same  side.  The  diagram  does  not  show  the  existence  of 
a  corresponding  set  of  neurones  whose  axones  pass  in  the  direction  the 
reverse  of  those  shown.  Degenerative  changes  after  experimental 
injuries  prove  their  existence.  Hence  a  second  diagram  might  be 
drawn  showing  axones  of  exactly  complementary  course.  These  are 
omitted  from  this  diagram  for  the  sake  of  clearness. 

It  is  evident  that  a  lesion  limited  to  one  lemniscus  will  not  cause 
deafness  any  more  than  a  lesion  limited  to  one  temporal  lobe.     There 


Fig. 


The  olfactory  system  and  tracts.  Bol,  olfactory  bulb:  Pol,  olfactory  tract;  Eolp,  deep  oltactory 
fibres ;  Roll,  internal  olfactory  fibres ;  Eole,  external  olfactory  fibres.  The  deep  fibres  pass  to  the 
thalamus  (Th)  and  its  nuclei ;  Tc,  tuber  cinereum ;  Tm,  tuber  mammillare,  and  to  tsc,  taenia  semi- 
circularis,  and  tth,  taenia  thalami ;  also  into  the  anterior  commissure,  coa.  The  internal  olfactory 
fibres  pass  into  the  striae  of  Lancisi,  sL,  and  thus  to  the  fasciola  cinerea,  Fc.  The  external  olfactory 
fibres  pass  directly  to  the  uncinate  gyrus,  U.  Tg,  fornix  from  the  corpus  mammillare  (Tm)  to  Am- 
mon's  horn  (Cg)  ;  NA,  nucleus  amygdalae  ;  Cing,  cingulum  ;  Cc,  corpus  callosum  ;  Fc.  fasc.  cinerea  ; 
FG,  Gudden's  fasciculus ;  FM.  fasc.  retroflexus  of  Meynert ;  Na,  ant.  nuc.  of  thalamus ;  Gip,  inter- 
peduncular ganglion  ;  Gh,  ganglion  habenula ;  Qa,  Qp,  Corp.  quadrigemina ;  Po,  pons ;  VA,  fascic. 
Vicq.  d'Azyr.     (Dejerine.) 

are  cases  on  record,  however,  in  which  the  lemniscus  on  both  sides  has 
been  affected  in  pons  lesions  and  in  these  deafness  has  occurred.  In 
ten  cases  of  limited  lesions  in  the  pons,  which  I  collected,  there  was 
deafness.^  Hence  in  any  case  where  a  pons  lesion  is  suspected  from 
its  characteristic  symptoms,  viz.,  alternating  paralysis  (see  page  104), 
^Central  Deafness,  Jour.  JSTerv.  and  Ment.  Dis.,  1910,  July. 


LOCAL  SYMPTOMS. 


isa 


alternating  anaesthesia  (see  page  117),  or  cranial  nerve  palsies  (see 
Chapter  X]5^*SV.),  it  is  well  to  test  for  deafness,  and  if  it  is  found  to 
regard  it  as  a  local  symptom  of  disease  of  the  lateral  part  of  the  lem- 
niscus in  the  cerebral  axis. 

Disturbance  of  the  Sense  of  Smell  is  occasionally  elicited  in  diseases 
of  the  brain.  This  sense,  which  is  of  vital  importance  to  animals, 
being  their  chief  guide  to  food,  is  of  little  importance  to  man,  and 
hence  its  organs  are  gradually  undergoing  retrograde  evolution.  In 
the  mammals,  and  especially  in  carnivora,  the  olfactory  bulb  and  its 
cerebral  tracts  are  enormous  and  are  the  best  protected  of  all  parts  of 
the  brain  from  injury,  lying  deep  within  the  cranium.  Fig.  68,  from 
Dejerine,  shows  the  numerous  connections  of  the  olfactory  bulb  and 


Fig.  69. 


The  olfactory  bulb  and  tract.  A,  Schneiderian  membrane  in  nose  in  which  lies  peripheral  olfac- 
tory neurone ;  B,  glomerulus  of  olfactory  bulb ;  C,  mitral  cells  with  dendrites  in  B  and  axones  in  D, 
olfactory  lobe ;  E,  granule  cells ;  F,  cells  in  olfactory  lobe  ;  H,  G,  fibres  of  olfactory  tract.  (Ramon  y 
Cajal.) 

fibres  and  their  relations  to  the  subcortical  ganglia,  and  their  termina- 
tion in  the  uncinate  gyrus  and  nucleus  amygdalus  which  lie  at  the  apex 
of  the  temporal  lobe.  It  is  in  this  area  that  physiologists  have  located 
the  sense  of  smell  in  the  cortex.  It  is  not  improbable  that  here,  too, 
is  located  the  sense  of  taste,  its  tract  coming  from  the  optic  thalamus 
by  way  of  the  trigonum.  The  taste  impulses  can  reach  the  thalamus 
from  the  fifth  nerve  nucleus  by  way  of  the  formatio  reticularis.  (See 
Chapter  XXXY.) 

There  are  a  few  cases  on  record  in  which  irritation  of  the  apex  of 
the  temporal  lobe  has  caused  hallucinations  of  smell  or  of  taste.  Such 
hallucinations  may  precede  Jacksonian  epileptic  attacks,  as  in  a  case  of 
Hamilton  and  one  of  Jackson,  in  both  of  which  local  meningitis  had 
affected  both  uncinate  gyri.  Destructive  lesions  of  this  area  with  loss 
of  smell  have  not  as  yet  been  recorded.  The  usual  cause  of  a  loss  of 
smell,  when  not  due  to  nasal  disease,  is  some  local  lesion  on  the  base 
in  the  frontal  lo])e,  destroying  the  olfactory  bulb  or  tract. 

Disturbance  of  the  Sense  of  Taste  as  a  local  symptom  of  brain  dis- 
ease has  n(jt  been  observed.     The  nerves  concerned  in  this  sense  are 


134        DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

the  fifth  and  ninth,  and  a  loss  of  taste  must  still  be  referred  to  their 
affection.     (See  Chapter  XXXV.) 

We  have  memories  of  smell  and  taste  which  are  undoubtedly  stored 
in  the  cortex  of  the  uncinate  gyrus  and  which  go  to  make  up  the  con- 
cepts of  objects  which  possess  odor  and  flavor.  We  have  no  record  of 
cases  of  psychical  anosmia,  though  such  symptoms  are  perfectly  possible. 

Disturbance  of  Thought  and  in  the  Use  of  Language.  —  Thus  far  the 
symptoms  produced  by  a  lesion  in  one  functional  area  of  the  cortex  or 
in  the  tract  leading  to  it,  or  from  it  through  the  brain  have  been  con- 
sidered, and  also  the  possible  combinations  of  these  symptoms  when 
adjacent  areas  or  tracts  are  involved  together.  But  while  the  cortical 
areas  liave  undoubtedly  distinct  functions,  it  is  not  to  be  forgotten  that 
they  are  closely  joined  to  one  another  by  means  of  the  multitude  of 
association  fibres  and  commissural  fibres  which  go  to  make  up  the 
larger  part  of  the  white  matter  of  the  centrum  ovale. 

It  can  be  shown  by  careful  dissection  that  each  convolution  is  joined 
to  the  two  adjacent  convolutions  by  fibres  which  pass  around  the  separ- 


The  association  fibres.  A,  between  adjacent  convolutions ;  B,  between  frontal  and  occipital  areas  ; 
C,  between  frontal  and  temporal  areas,  cingulum ;  D,  between  frontal  and  temporal  areas,  fasciculus 
uucinatus;  E,  between  occipital  and  temporal  areas,  fasciculus  longitudinalis  inferior;  CN,  caudate 
nucleus  ;  OT,  optic  thalamus. 

ating  fissures.  (Fig.  70.)  Also,  that  bundles  of  fibres  exist  which 
pass  from  each  convolution  to  the  convolution  next  but  one,  and  so  on. 
Hence,  it  may  be  stated  that  each  convolution  has  a  possible  connec- 
tion with  every  other.  Besides  this  association  of  convolutions  by 
small  bundles  of  fibres,  it  is  possible  to  find  a  distinct  set  of  associa- 
tion tracts  which  pass  between  more  or  less  distant  regions.  One 
such  tract  passes  from  the  frontal  lobe,  collecting  its  bundles  from  all 
three  convolutions,  backward  to  the  occipital  lobe.  Another  tract 
joins  the  occipital  with  the  anterior  part  of  the  temporal  lobe.  An- 
other passes  from  the  upper  two  temporal  convolutions  forward  to  the 
third  frontal  convolution,  passing  beneath  tlie  island  of  Reil.  And  a 
tract  from  the  frontal  to  the  posterior  temporal  area  may  also  be  found. 


LOCAL  SYMPTOMS. 


135 


Their  relative  degree  of  development  in  animals  is  directly  in  the  ratio 
of  the  cortical  development,  and  their  actual  number  is  an  index  of 
mental  capacity. 

The  function  of  these  association  fibres  is  to  form  the  physical  basis 
for  the  association  of  concrete  memories  and  of  psychical  acts,  and 
their  integrity  is  necessary  to  thought.  By  studying  subjectively  the 
association  of  ideas  in  the  mind  the  importance  of  their  function  becomes 
evident. 

We  have  already  seen  that  each  sensory  impression  leaves  a  memory 
picture  which  is  the  basis  of  our  recognition  of  an  object.  But  each 
object  produces  on  the  mind  a  number  of  diiferent  impressions  simul- 
taneously. These  are,  therefore,  associated  together  in  the  mind  and 
form  a  concept  consisting  of  numerous  memory  pictures  joined  together 
by  means  of  the  association  fibres  in  the  brain.  These  fibres  form  as 
essential  a  part  of  concepts  as  the  various  memory  pictures.  Thus  a 
single  memory  picture  has  little  meaning  unless  it  is  associated  with 
others ;  a  new  object  seen  has  to  be  brought  into  relation  with  objects 

Fig.  71. 


Diagram  to  illustrate  the  concept  "bell"  and  to  show  the  varieties  of  apraxia  and  aphasia.  The 
memory  pictures  are  relics  of  past  perceptions  received  through  diiferent  senses.  Their  association 
makes  up  the  mental  image  bell.  The  word  image  is  made  up  of  the  memories  of  the  sound  and  appear- 
ance of  the  word,  and  of  the  uttering  and  writing-effort  memories ;  these  are  joined  together.  The 
mental  image  and  the  word  image  are  also  joined  with  one  another,  making  up  the  concept  "bell." 
(Charcot.) 

already  known  before  it  becomes  understood  or  fixed  in  the  mind.  It 
must  also  be  brought  into  relation  with  its  name.  And  thus  the  sim- 
plest object,  "a  rose"  or  "a  bell,"  is  known  only  when  the  numerous 
sensory  impressions  which  it  awakens  arc  united  into  a  complex  mental 
substratum.      Wc  have  no  single  ideational  centre  or  concept  centre  in 


136         DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

the  brain,  but  each  concept  is  made  up  of  many  pictures.  This  Is 
shown  in  the  dia'jram  of  Charcot  of  the  concept  bell.  (Fig-  71.) 
When  we  hear  a  peal  and  it  awakens  the  memory  of  the  word  "  bell " 
and  also  the  appearance  of  a  large  church  bell  or  small  dinner  bell, 
whose  shape  and  cold,  hard,  metallic  feel  and  weight  can  be  called  to 
mind,  and  when  we  recollect  the  corresponding  word  in  French  or 
German  or  any  other  language  that  we  know,  and  also  recall  the  effort 
needed  to  pronounce  the  corresponding  word  in  our  own  and  in  the 
foreign  tongue,  and  also  to  write  it  and  to  read  the  letters  that  make 
up  the  word,  we  have  merely  aroused  in  consciousness  one  after  an- 
other a  number  of  dormant  memory  pictures  by  sending  impulses  over 
association  fibres  that  unite  these  pictures  together.  Hence,  association 
fibres  are  necessary  to  thought,  and  a  break  in  them  or  a  destruction 
of  their  terminal  stations  will  impair  mental  action. 

A  defect  in  the  association  of  these  memory  pictures,  so  that  recog- 
nition of  an  object  or  its  use  when  one  memory  of  it  is  awakened  is 
impossible,  has  been  termed  apraxia.  Astereognosis  is  the  inability 
to  recognize  an  object  by  the  sense  of  touch. 

Aphasia  is  the  loss  of  the  power  of  using  language.  We  have 
already  seen  that  the  basis  of  language  is  a  series  of  memory  pic- 
tures:  (1)  of  the  sound  of  a  wordj  (2)  of  the  effort  necessary  to 
enunciate  it ;  (3)  of  the  appearance  that  its  printed  or  written  symbols 
present ;  and  (4)  of  the  effort  needed  to  produce  these  symbols  in  writ- 
ing. These  memory  pictures  are  intimately  connected  with  each  other 
by  means  of  association  fibres  which  pass  in  all  directions  between  the 
areas  in  which  they  are  located.  They  are  also  joined  to  the  memory 
pictures  which  make  up  the  concept.  They  are  so  intimately  joined 
that  if  one  is  lost  all  the  others  feel  the  effect,  and  a  break  in  the 
mechanism  leads  to  a  defect  in  the  act  of  speech. 

The  defects  of  speech  are  broadly  divided  into  sensory  and  motor 
aphasia,  according  to  the  predominant  feature  of  the  defect.  Sensory 
aphasia  consists  in  a  lack  of  power  to  recognize  or  to  recall  the  sound 
or  appearance  of  words.  If  the  sound  alone  is  lost  the  condition  is 
termed  word-deafness ;  if  the  appearance  is  lost  the  condition  is  termed 
word-blindness.  Motor  aphasia  consists  in  a  lack  of  power  to  initiate 
the  effort  and  set  in  action  the  motor  mechanism  needed  to  pronounce 
or  to  write  a  word.  The  former  is  termed  motor  or  ataxic  aphasia,  the 
latter  motor  agraphia.     They  are  usually  associated. 

1.  Word-deafness.  —  If  the  memory  of  the  sound  of  the  word  is 
lost  the  word  cannot  be  called  to  mind  and  cannot  be  recognized  when 
heard.  If  the  patient  be  shown  a  watch  he  cannot  name  it,  and  can- 
not recognize  the  word  watch  when  he  hears  it.  He  may  recall  its  use 
and  even  show  that  he  can  tell  the  time,  but  its  name  has  been  lost. 
Such  a  patient,  therefore,  Is  In  the  condition  of  a  man  who  hears  a 
foreign  language ;  he  hears  sounds,  but  attaches  no  meaning  to  them. 
He  makes  no  associations  between  the  word  and  its  concept.  He  does 
not  understand.  This  condition  usually  very  much  Impairs  his  use 
of  words,  for  he  is  unable  to  call  to  mind  the  sound  of  the  word,  and 


PLATE  XI 


The  Connections  of  the  Areas  of  the  Cortex  Included  in  the  Zone  of  Language. 

(Dejerine.) 

The  upper  part  of  the  figure  represents  a  vertical  section  through  the  brain.  The  lower  part 
represents  a  horizontal  section  below  the  transverse  clotted  lines.  F,  frontal;  Fa,  anterior  central: 
F3,  Broca's;  T,  temporal;  Pc,  angular  convolutions;  Arc,  arcuate  fibres  joining  the  angular  gyrus  Pc 
and  the  temporal  convolutions  T^  to  Broca's  convolution  F-j  and  to  the  motor  centre  for  the  arm  Fa; 
pet,  association  fibres  between  the  angular  gyrus  and  temporal  convolutions;  pec,  association  fibres 
between  the  two  hemispheres;  pef,  fibres  joining  the  left  angular  gyrus  to  the  right  motor  centre; 
pc/.j,  fih)res  joining  the  left  angular  gyrus  to  the  right  third  frontal  convolution;  peo,  pe6,  fibres  joining 
the  left  angular  gyrus  to  the  occipital  convolutions  of  the  left  and  right  lobes;  tct,  commissural  fibres 
between  the  two  temporal  lobes;  Th,  thalamus;  NC,  caudate  nucleus;  Nl,  lenticular  nucleus;  Cia, 
internal  cap.sule,  anterior  limb;  Cip,  posterior  limb;  Cirl,  CSgt,  visual  tract;  VI,  lateral  ventricle-  cjf, 
commissural  fibres  in  corpus  callosum  Co;  C,  cuneus. 


LOCAL  SYMPTOMS.  137 

therefore  is  unable  to  say  it.  But  the  motor  act  of  pronunciation  may 
be  initiated  ^f  the  impulse  can  be  sent  to  its  centre  without  passing 
through  the  auditory  centre.  This  is  the  case  in  exclamations,  when 
one  does  not  choose  his  words ;  hence  many  patients  can  swear,  through 
they  cannot  talk.  If,  in  a  patient  with  word-deafness,  there  is  no 
accompanying  word-blindness,  he  may  be  able  to  read  aloud  as  well 
as  to  himself — that  is,  his  motor  speech  memory  may  be  aroused  by 
way  of  his  visual  memories  without  the  intervention  of  the  auditory 
memories.  And  if  he  has  no  apraxia  it  is  also  possible  for  any  of  the 
concept  memories  to  awaken  the  motor  speech  memory ;  hence  the 
thought  of  an  object  or  seeing  it  may  lead  to  the  enunciation  of  its 
name  without  thought  of  how  the  name  sounds.  For  this  reason 
patients  who  are  word-deaf  and  cannot  understand  what  is  said  to 
them  may  be  able  to  say  certain  words  but  rarely  an  entire  sen- 
tence. 

The  lesion  in  total  word-deafness  is  located  in  the  middle  part 
of  the  cortex  of  the  first  and  second  temporal  convolutions  in  the 
left  hemisphere  in  right-handed,  and  in  the  right  in  left-handed 
persons. 

2.  WoED-BLiNDNESS. —  If  the  memory  of  the  appearance  of  the 
word  is  lost,  the  visual  image  of  it  cannot  be  called  to  mind  or  recog- 
nized, and  then  the  patient  will  be  unable  to  read,  because  the  shapes 
of  the  letters  and  words  seen  arouse  no  recollection.  This  is  also 
termed  alexia.  Tie  will  also  be  unable  to  write  spontaneously,  for  he 
cannot  remember  how  the  letter  that  he  wishes  to  write  looks.  The 
appearance  of  letters  is  sometimes  retained  when  words  are  lost. 
Figures  are  sometimes  recalled  when  words  are  forgotten,  and  many 
a  patient  can  do  mathematical  calculations  on  paper  who  cannot 
read  or  write  ordinary  words.  The  reverse  may  also  be  true,  the 
patient  being  able  to  read  and  write,  but  being  unable  to  understand 
or  to  write  figures  or  to  calculate.  Such  patients  may  play  cards  or 
other  games,  if  they  are  not  psychically  blind.  It  is  not  infrequently 
the  case  that  persons  who  are  thus  word-blind  can  write  at  dictation, 
or  copy,  and  yet  show  no  evidence  of  understanding  what  has  just 
been  written.  Here  the  writing  centre  has  been  called  into  activity 
through  some  association  tract  without  the  intervention  of  the  word 
memory  picture.  A  distinction  must  be  made  between  those  who  have 
lost  the  memory  picture  and  those  in  whom  it  cannot  be  recalled  by 
ordinary  means.  The  first  have  cortical  word-blindness;  the  second 
have  subcortical  word-blindness.  The  word  memory  picture  may  be 
cut  off"  from  its  ordinary  channels  of  connection  with  other  memory 
pictures  and  yet  remain  intact.  In  such  a  case  some  roundabout  road 
to  it  will  lead  to  its  revival  in  consciousness,  and  it  will  be  found  to 
be  preserved,  though  inaccessible  by  ordinary  means. 

The  condition  of  visual  amnesia  with  word-blindness  is  due  to 
a  lesion  involving  the  inferior  parietal  convolutions  and  angular 
gyrus,  and  is  often  associated  with  psychical  blindness,  but  may 
occur  independently  of  it.     The  lesion  is  in  the  left  hemisphere   in 


138     '  DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

right-handed  persons  and  in  the  right  hemisphere  in  left-handed  per- 
sons ;  a  few  exceptions  to  this  rule  have  been  observed.^ 

Word-deafness  and  word-blindness  frequently  occur  together,  and 
then  the  lesion  is  found  involving  both  the  temporal  convolutions  and 
the  angular  gyrus. 

3.  Optical  Aphasia  or  Intercoetical  Sensory  Aphasia.  — 
When  the  association-fibres  between  the  memories  of  sight  and  the 
memories  of  sound  are  severed  a  condition  of  aphasia  results  which  is 
characterized  by  an  inability  to  recall  the  name  of  a  thing  seen  and  to 
picture  to  the  mind  the  appearance  of  a  thing  named.  Yet  the  name 
is  recognized  when  heard  and  the  object  is  recognized  when  seen. 
This  condition  has  been  described  under  different  names  by  different 
observers.  Freund  ^  named  it  optical  aphasia  or  transcortical  aphasia, 
and  these  terms  are  used  by  the  Germans.^  I  prefer  the  term  inter- 
cortical  sensory  aphasia  as  less  obscure  and  misleading:  A  patient 
suffering  from  this  type  of  aphasia  has  not  lost  his  memory  pictures, 
for  he  is  able  to  recognize  anything  once  heard  or  seen.  He  can, 
therefore,  hear,  understand,  and  read ;  but  if  he  is  asked  to  call  to  his 
mind  some  place  or  person  whose  name  is  given  —  e.  g..  Lake  George, 
Lake  Como,  President  McKinley  —  he  cannot  do  so.  The  impulse 
started  from  the  word-hearing  centre  cannot  reach  and  arouse  the 
visual  memories ;  nor  can  the  association  be  made  in  the  opposite 
direction,  for  if  he  is  shown  an  object  or  a  person  —  a  watch,  a  chain, 
or  some  familiar  face  —  he  cannot  recall  the  name,  though  he  recog- 
nizes it  when  heard. 

There  are  numerous  cases  on  record  with  autopsies  which  prove  that 
the  lesion  in  this  condition  lies  in  the  long  association  tract  within  the 
temporal  and  occipital  lobes.  This  tract  is  shown  in  Plate  IX. 
Such  a  lesion  may  be  an  abscess  of  the  brain  secondary  to  ear  disease, 
or  a  tumor,  or  a  focus  of  softening.  In  a  case  described  in  the  chap- 
ter upon  abscess  of  the  brain  this  symptom  was  made  the  basis  of  a 
surgical  operation  which  proved  successful.  Should  an  extensive  cor- 
tical lesion  occur  in  the  convolutions  between  the  temporal  and  occip- 
ital gyri  and  invade  the  white  matter  beneath  them  the  same  symptoms 
would  be  produced. 

4.  Motor  Aphasia.  —  If  the  memory  of  the  effort  needed  to  pro- 
nounce a  word  is  lost,  a  true  paralysis  of  active  speech  occurs,  though 
the  muscles  may  not  be  weakened.  This  is  the  ordinary  form  of  motor 
aphasia,  due  to  a  lesion  of  Broca's  centre  in  the  posterior  part  of  the 
third  frontal  convolution  on  the  left  side  in  right-handed  persons.  It 
is  to  be  noted  that  such  a  loss  of  speech  involves  a  loss  of  the  power 
of  repeating  words  after  another,  as  well  as  of  voluntary  speech,  and 
is  not  accompanied  by  any  inability  to  understand  spoken  or  written 
language.  In  the  uneducated,  as  in  children,  the  acts  of  talking  and 
writing  are  closely  joined,  as  may  be  seen  by  watching  the  lips,  which 

^  Mills  and  Weissenburg  :  Medicine,  Nov.,  1905. 
'^ Freund,  Arch.  f.  Psych.,  xx.,  276. 
^  Vorster,  Arch,  f.  Psych.,  xxx.,  341. 


LOCAL  SYMPTOMS.  139 

move  in  the  act  of  writing.  But  among  those  accustomed  to  write 
much  these/acts  are  independent,  and  it  is  probable  that  many  edu- 
cated aphasics  may  be  able  to  answer  questions  in  writing  when  their 
efforts  at  speech  fail ;  but  as  a  matter  of  experience,  it  is  found  that 
speech  and  writing  are  usually  lost  together.  Reading  aloud  will  also 
be  lost  in  motor  aphasia,  for  here,  too,  the  inability  to  articulate 
hampers  the  patient. 

Such  patients  can  usually  say  "  no  "  and  "  yes,"  and  often  retain  the 
power  of  using  monosyllabic  words.  They  talk,  if  at  all,  as  a  baby 
talks  who  is  just  learning  to  speak.  Sometimes  a  short  phrase,  uttered 
just  before  the  attack  of  aphasia  occurred,  can  be  and  is  repeated  over 
and  over.  Thus  I  knew  a  woman  whose  only  phrase  was  "  Ah,  dear 
me,  I  don't  know ! "  and  this  was  said  on  all  occasions,  with  varying 
inflection,  being  the  only  thing  she  could  say. 

5.  Agraphia. — The  independence  of  the  effort-memories,  neces- 
sary for  writing,  from  the  effort-memories  of  speech,  though  questioned 
by  Dejerine,  must  be  admitted.  When  these  are  lost  alone  the  con- 
dition is  known  as  agraphia.  In  such  a  state  the  pen  cannot  be  used. 
Copying,  writing  at  dictation,  and  voluntary  writing  are  all  lost.  It 
has  been  noted  already  that  when  a  word  cannot  be  called  to  mind,  or 
read,  or  mentally  enunciated,  in  the  majority  of  persons,  it  cannot  be 
written.  But  words  can  then  often  be  written  at  dictation,  if  the: 
person  is  one  who  has  written  much.  Hence  sensory  agraphia  and 
motor  agraphia  must  be  distinguished,  the  former  being  a  part  of 
word-blindness,  the  latter  not  at  all  associated  with  inability  to  read., 
The  lesion  of  motor  agraphia  is  not  certainly  known,  though  a  few 
facts  point  to  the  posterior  part  of  the  second  frontal  convolution  as 
the  probable  seat  of  this  function.^  It  is  not  unlikely,  however,  that 
the  more  exact  localization  of  fine  movements  of  the  thumb ,  and 
fingers  in  the  anterior  central  convolution  may  be  followed  by  the  dis- 
covery of  the  writing  centre  in  this  vicinity.  An  interesting  case  is; 
recorded  by  Trousseau  of  a  deaf  mute  who  had  learned  to  talk  by  the 
finger  language  and  who  lost  this  power  by  a  lesion  near  the  motor 
centre  for  the  fingers,  though  the  fingers  were  not  paralyzed. 

6.  Paraphasia  or  Intercortical  Motor  Aphasia.  —  The 
forms  of  aphasia  thus  far  studied  are  due  to  a  loss  of  distinct  memory 
pictures.  The  several  memory  pictures  which  are  united  in  the  word- 
image  may  thus  be  reasonably  regarded  as  separate  from  one  another 
in  their  location  in  the  brain.  But,  since  they  are  joined  together  to 
form  the  word-image,  it  follows  that  the  association-fibres  joining  the 
various  areas  are  as  necessary  to  the  use  of  even  a  single  word  as  the 
various  areas  with  their  memories.  It  is  really  by  association  only 
that  an  object  or  a  word  becomes  a  subject  of  thought  or  of  use.  If 
these  associations  are  broken,  the  result  is  a  defect  of  language 
characterized  by  the  misplacement  of  words,  and  the  patient  talks; 
jargon.  Such  a  condition  is  termed  paraphasia  or  intercortical 
aphasia. 

*  Gordinier,  American  Journal  of  the  Medical  Sciences,  September,  1903,  has  pub- 
ILsbed  such  a  case. 


140         DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

There  are  as  many  forms  of  paraphasia  as  there  are  association- 
tracts. 

Alkision  has  already  been  made  to  intercortical  sensory  aphasia. 
Another  form  is  intercortical  motor  aphasia,  in  which  the  association 
tract  between  the  temporal  convolution  and  Broca's  convolution  is 
involved.  This  tract  passes  beneath  the  island  of  Reil.  (Fig.  70,  D.) 
When  it  is  affected  the  patient  can  understand  what  is  heard  or  seen 
and  can  enunciate  words  clearly,  but  is  unable  to  repeat  after  another 
person  a  word  heard,  and  talks  jargon.  These  patients  usually  talk 
rapidly  and  constantly,  try  very  hard  to  convey  their  ideas,  but  can- 


FiG.  72. 


Hand 


Touch 


Voice 


Hearing 


Diagram  to  illustrate  aphasia.  The  cortical  sensory  and  motor  centres  are  indicated  by  the  arrows. 
The  secondary  cortical  centres  of  memories  are  indicated  by  circles.  /,  visual,  of  objects ;  //,  visual, 
of  words ;  III,  tactile ;  IV,  auditory  ;  V,  speech ;  VI,  writing.  These  are  joined  to  one  another  by 
associat4on  fibres  which  transmit  impulses  in  both  directions.  Subcortical  lesions  in  these  fibres  cause 
aphasia  as  well  as  lesions  in  the  cortex. 

not  be  understood  at  all,  for  the  mingling  of  nonsense  words  or  of  syl- 
lables with  words  which  they  had  no  intention  of  using  renders  the 
sentences  unintelligible. 

If  one  constructs  a  diagram  like  the  above  figure  (72)  and  then  hypo- 
thetically  divides  one  of  the  lines  between  the  various  centres,  one 
obtains  a  scheme  illustrating  aphasia  of  conduction  or  intercortical 
aphasia.  It  becomes  evident  that  there  are  a  great  many  possible  forms 
of  aphasia  of  conduction.  But  the  common  feature  in  all  is  the  loss 
of  power  of  association  of  the  memory  pictures  while  these  pictures  are 
preserved.  It  is  interesting  to  discover  such  cases,  and  much  care  has 
been  given  to  their  analysis.  In  fact  it  is  largely  by  their  analysis 
that  our  knowledge  of  the  existence  of  separate  memory  pictures,  of 


LOCAL  SYMPTOMS.  141 

the  localization  of  these  pictures  and  of  the  union  of  them  by  the 
association-fibres  has  been  established.  But  there  are  not  as  yet  on 
record  a  sufficient  nuhiber  of  cases  with  autopsy  to  enable  us  to  make 
an  exact  diagnosis  of  the  location  of  the  lesion  in  patients  who  present 
symptoms  of  aphasia  of  conduction. 

In  almost  all  cases  of  aphasia  which  have  been  examined  post 
mortem  some  lesion  of  these  association  fibres  has  been  found.  In  fact 
cases  of  pure  aphasia  of  cortical  origin  are  rare.  The  lesion  of  these 
fibres  causes  necessarily  great  confusion  in  the  mental  processes  of  asso- 
ciation and  hence  defects  of  intelligence  are  very  striking  in  all  cases  of 
aphasia.  P.  Marie  has  recently  called  particular  attention  to  this  side 
of  aphasia.^  He  considers  that  in  almost  all  cases  there  can  be  shown 
to  have  been  a  lesion  of  the  lenticular  zone  lying  at  the  bottom  of  the 
Sylvian  fissure  and  outside  of  the  lenticular  nucleus  —  the  zone  through 
which  the  majority  of  these  association  fibres  are  seen  to  pass  in  this 
diagram.  This  zone  he  terms  the  "  intellectual  centre  of  language.'' 
The  motor  centre  in  Broca's  convolution  he  regards  as  wholly  sub- 
sidiary and  he  considers  that  its  lesion  causes  anarthria  rather  than 
aphasia  ;  i.  e.,  a  difficulty  in  the  proper  muscular  act  of  speaking  without 
disturbance  of  intelligence.  Marie's  statements  have  produced  wide 
divergences  of  opinion  on  the  entire  question  of  aphasia  which  cannot 
be  reconciled,  and  his  conclusions  are  not  generally  accepted,  the  matters 
at  issue  being  still  under  discussion.^ 

To  examine  an  aphasic  thoroughly  it  is  necessary  to  test : 

1.  The  power  to  recognize  objects  seen,  heard,  felt,  tasted,  or  smelled, 
and  their  use. 

This  will  determine  whether  the  condition  of  apraxia  or  disturbance 
in  the  power  of  recalling  any  part  of  a  concept  is  present. 

2.  The  power  to  recall  the  spoken  name  of  objects  seen,  heard, 
handled,  tasted,  or  smelled. 

3.  The  power  to  understand  speech  and  musical  tunes. 

4.  The  power  to  call  to  mind  objects  named. 

This  will  test  the  integrity  of  the  auditory  speech  area  and  of 
the  association-tracts  between  other  sensory  areas  and  the  temporal 
convolutions. 

5.  The  power  to  understand  printed  or  written  words. 

6.  The  power  to  read  aloud  and  to  understand  what  is  read. 

7.  The  power  to  recall  objects  the  names  of  which  are  seen. 

8.  The  power  to  write  spontaneously  and  to  write  the  names  of 
objects  seen,  heard,  etc. 

9.  The  power  to  copy  and  to  write  at  dictation. 

10.  The  power  to  read  understandingly  what  has  been  written„ 
These  tests  will  determine  the  condition  of  the  visual  word  memories 

ip.  Marie,  L'Aphasie,  Semaine  Medicale,  Apr.  and  Oct.,  1906.  See  L'Aphasie  de 
Broca,  by  F.  Mouticr,  Steinheil,  Paris,  1908,  where  full  references  to  recent  French 
articles  may  be  found. 

"La  discussion  sur  I'Aphasie.  L'Encephale,  1908,  and  the  discussion  on  aphasia. 
Jour.  Nerv.  and  Ment.  Dis.,  Sept.,  Oct.,  Nov.,  1907, 


142         DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

in  the  angular  gyrus,  and  of  the  connections  between  this  area  and 
surrounding  sensory  and  motor  areas. 

11.  The  power  to  speak  voluntarily,  and  if  it  is  lost,  the  character 
of  its  defects. 

12.  The  power  of  repeating  words  after  another. 

This  will  test  the  integrity  of  Broca's  centre  and  its  association- 
tracts. 

The  following  table  shows  the  chief  defects  in  each  variety  of  aphasia, 
and  may  serve  to  aid  in  diagnosis. 

Table  IV. —  The  Symptoms  in  Different  Forms  of  Aphasia.^ 


Variety. 

Understand- 
ing of 

Power  to  re- 
peat words. 

Power  to  talk. 

Power  to  read. 

Power  to  write. 

language. 

1.    Word-deafness. 

(a)  cortical, 

Lost. 

Lost. 

Retained 
(not  to  answer). 

Eetained 
(not  aloud). 

Eetained. 

(6)  subcortical. 

Lost. 

Lost. 

Imperfect 
(not  to  answer). 

Eetained 
(not  aloud). 

Eetained, 

2,   Word-blindness. 

(a)  cortical; 

Retained. 

Eetained. 

Retained. 

Lost. 

Lost. 

(b)  subcortical. 

Eetained. 

Retained. 

Eetained. 

Lost. 

Imperfect. 

3.  Intercortical, 

between  T.  and  0. 

Imperfect. 

Retained. 

Retained, 

Imperfect 
(without  com- 

Retained 
(not  at  dicta- 

4. Motor  aphasia. 

prehension). 

tion). 

(a)  cortical, 

Retained. 

Lost. 

Lost. 

Eetained 
(not  aloud). 

Lost. 

(6)  subcortical. 

Retained. 

Lost. 

Lost. 

Imperfect 
(not  aloud). 

Lost. 

5.  Agraphia. 

(a)  cortical, 

Retained. 

Retained. 

Retained. 

Eetained. 

Lost. 

(6)  subcortical. 

Retained. 

Retained. 

Retained. 

Eetained. 

Imperfect. 

6.  Intercortical, 

between  T.  and  F. 

Imperfect. 

Lost. 

Jargon. 

Imperfect. 

Lost   or  im- 
perfect. 

The  Commissural  Fibres. —  The  second  system  of  association-fibres 
in  the  centrum  ovale  is  the  commissural  system.  This  joins  corre- 
sponding areas  of  the  two  hemispheres  with  one  another.  The  func- 
tion of  these  fibres  is  to  harmonize  the  action  of  the  two  hemispheres. 
Movements  of  like  nature  can  be  made  with  greater  facility  with  both 
upper  extremities  when  moving  simultaneously.  Movements  which 
are  difficult  when  attempted  with  the  left  hand  alone  become  easy  when 
associated  with  corresponding  movements  of  the  right  hand  —  as,  for 
example,  drawing  a  circle,  writing  one's  name.  Such  associated  motions 
are  accomplished  by  aid  of  the  commissural  fibres  between  the  two 
motor  areas. 

The  sensory  areas  are  also  necessarily  joined  by  commissural  tracts ; 
for  in  order  that  the  half  images  received  in  each  occipital  lobe  may 
be  combined,  a  large  tract  passes  from  one  cuneus  to  the  other.  In 
order  that  sensations  of  touch  may  be  correlated  the  two  parietal  lobes 

*  There  are  many  complex  varieties  of  aphasia  too  rare  and  involving  too  exhaustive 
discussion  to  be  included  in  a  general  text-book.  The  reader  is  referred  to  an  article  by 
the  author  on  Sensory  Aphasia,  Brain,  vol.  xii.,  p,  82  ;  to  Wylie,  Disorders  of  Speech, 
Edmburgh,  1894  ;  and  to  Collins,  The  Faculty  of  Speech,  New  York,  18^8,  and  to  the 
discussion  of  aphasia  before  the  Neurological  Society  of  Paris  in  1908,  published  in 
J'Fncejphale,  1908,  where  full  references  to  the  literature  of  the  subject  are  to  be  found. 


LOCAL  SYMPTOMS. 


143 


are  joined.  In  order  that  sounds  may  be  heard  the  temporal  lobes  are 
connected.  The  anterior  commissure  of  the  brain  joins  the  two  tem- 
poral lobes  ^gether.  The  commissural  fibres  between  the  greater  part 
of  the  convexity  of  the  hemispheres  pass  in  the  corpus  callosum. 

The  Association  Areas  of  Flechsig. —  The  existence  of  areas  of  the 
cortex  whose  chief  function  is  to  receive  and  transmit  association  im- 
pulses has  recently  been  proven  by  Flechsig,  and  his  researches  throw 
much  light  upon  the  function  of  those  extensive  areas  of  the  cortex 
which  have  no  known  sensory  or  motor  functions.  It  is  known  that 
the  medullary  sheath  which  surrounds  and  insulates  the  axone  of  each 
neurone  body  develops  after  the  axone.  Flechsig  has  shown  that  the 
period  at  which  this  meduUation  occurs  differs  in  different  functional 
tracts  during  embryonal  life.  In  the  earliest  stage  but  a  few  fibres  are 
meduUated.     As  the  embryo  grows,  each  system  of  neurones,  one  by 

Fig.  73. 


Flechsig's  diagram  to  show  the  order  of  development  of  the  various  areas  of  the  cortex. 
Lateral  surface.     (Lancet,  October  19,  1901.) 

one,  completely  develops,  and  by  contrasting  brains  at  different  ages 
these  functional  systems  can  be  distinguished  from  each  other. 

The  chief  sensory  systems  are  the  first  to  develop,  the  motor  system 
follows,  and  at  birth  these  tracts  which  bring  the  child  into  relation 
with  the  outer  world,  projecting  its  impressions  on  his  consciousness 
and  projecting  his  will,  as  shown  by  effort  and  act  on  the  world,  are 
complete.  Later  the  various  association-fibres  within  the  brain  are 
formed,  so  that  as  the  sensory  impressions  are  received  they  can  be 
related  to  one  another,  and  a  sensation  can  awaken  its  properly 
coordinated  motor  response.  Flechsig  now  distinguishes  thirty-six 
areas  of  the  cortex  of  the  brain,  the  neurones  in  each  area  becoming 
developed  at  a  different  time  from  those  in  other  areas.  And  he 
naturally  concludes  that  those  whose  manifest  function  is  to  associate 
the  different  parts  of  the  brain  with  one  another,  and  which  develop 
last  of  all,  are  more  closely  related  to  the  higher  mental  process  of 
reasoning  than  those  whose  function  is  merely  to  transmit  sensations  or 
motor  impulses.     In  his  diagrams  (Figs.  73  and  74)  the  order  of  this 


144        DIAGNOSIS  AND  LOCALIZATION  OF  BHAIN  DISEASES. 

cortical  development  is  indicated  by  the  numbers  on  the  various  areas. 

Mental  symptoms  consisting  of  aphasia,  disturbances  in  the  rapid 
association  of  ideas,  apraxia,  forms  of  loss  of  memory,  defect  in  the 
keen  perception  of  the  meaning  of  ideas,  defects  in  judgment  and  in 
reasoning,  in  logical  thought  and  action  —  these  are  the  symptoms  that 
we  ascribe  theoretically  to  a  disturbance  of  function  in  the  association 
areas  of  the  cortex  or  in  the  association  and  commissural  tracts.  And 
a  careful  clinical  study  of  cases  in  which  lesions  have  been  found  in 
the  so-called  latent  regions  of  the  cortex  and  in  the  centrum  ovale  and 
corpus  callosum  leads  me  to  believe  that  such  mental  symptoms  may 
always  be  elicited. 

They  will  be  noticed  more  especially  in  the  chapters  upon  apoplexy, 
cerebral  abscess,  and  cerebral  tumors. 

Fig.  74. 


Flechsig's  diagram  to  show  the  order  of  development  of  the  various  areas  of  the  cortex. 
Median  surface. 

Disturbances  in  the  Control  of  the  Emotions,  leading  either  to  undue 
excitement,  causeless  laughter,  unusual  crying,  great  depression  and  a 
lack  of  harmony  between  the  association  of  ideas  and  the  state  of 
feeling  which  they  should  awaken,  are  symptoms  produced  by  lesions 
in  the  frontal  area  of  the  cerebral  cortex  and  of  the  subjacent  white 
matter  of  the  centrum  ovale.  The  same  symptoms  are  also  noticed  in 
lesions  of  the  anterior  portion  of  the  corpus  callosum  which  unites  the 
two  frontal  lobes.  The  frontal  region  is  joined  by  a  large  tract  to  the 
optic  thalamus.  (Fig.  53,  A^.)  This  tract  passes  inward,  fills  the 
anterior  limb  of  the  internal  capsule,  and  ends  in  the  external  nucleus 
of  the  thalamus.  It  was  noticed  by  Nothnagel  that  lesions  in  the 
thalamus  interfere  with  the  automatic  facial  expression  of  emotion. 
Thus  a  patient  who  has  such  a  lesion  may  not  smile  on  the  side  oppo- 
site to  the  lesion  when  amused,  even  when  he  can  voluntarily  contract 
the  risorii  muscles,  and  thus  give  a  forced  smile.  This  is  another 
proof  of  the  intimate  relation  of  the  frontal  region  to  emotional  acts. 

These  are  the  chief  symptonis  that  are  met  with  in  cerebral  cortical 
disease  and  their  local  significance. 


LOCAL  SYMPTOMS.  145 

Symptoms  of  Subcortical  Lesions.  —  It  remains  to  consider  some 
special  symptoms  produced  by  subcortical  lesions  located  in  the  basal 
ganglia  and  in  the  cerebral  axis.  These  will  be  better  understood 
because  of  the  facts  which  have  been  already  presented  regarding  the 
anatomy  of  the  brain. 

Lesions  of  the  basal  ganglia,  viz.,  the  corpora  striata,  (made  up  of 
the  lenticular  and  caudate  nuclei,)  and  optic  thalamus,  are  very  common 
and  give  rise  to  many  symptoms.  These  symptoms  are,  however, 
to  be  ascribed  to  a  coincident  affection  of  the  motor  and  sensory  tracts 
which  pass  through  the  internal  capsule  between  the  ganglia.  Figs. 
48  and  53.)  Hence,  the  local  symptoms  of  lesions  in  the  lenticular 
or  caudate  nucleus  and  optic  thalamus  are  those  of  lesions  of  the  vari- 
ous tracts  in  the  internal  capsule  opposite  those  bodies,  viz.,  in  its  pos- 
terior division.  If  the  symptoms  are  permanent,  the  capsule  is  prob- 
ably injured.  If  the  symptoms  pass  away  the  capsule  was  incidentally 
affected.  And  the  effects  of  the  lesion  may  entirely  subside  while  the 
lesion  remains  if  it  is  entirely  limited  to  either  of  these  ganglia.  Mills 
and  Spiller  have  recently  made  a  special  study  of  lesions  of  the  len- 
ticular nucleus  and  their  conclusions  are  as  follows  : 

1.  Lesions  restricted  to  the  lenticula  apparently  do  not  cause  sen- 
sory symptoms ; 

2.  Motor  symptoms  probably  result  from  lesions  situated  in  certain 
parts  of  the  lenticula  ;  speaking  generally,  the  lenticula  may  be  regarded 
as  a  motor  organ  ; 

3.  Anarthric  or  dysarthric  speech  disorders  result  from  lesions  of 
some  portion  of  the  left  lenticula,  which  probably  contains  centres 
which  are  concerned  with  movements  which  make  speech  possible ; 

4.  Destructive  lesions  of  certain  portions  of  the  lenticula  probably 
cause  a  paresis  of  the  limbs  or  face  ; 

5.  The  paresis  or  paralysis  caused  by  destructive  lesions  of  the  len- 
ticula differs  from  that  produced  by  capsular  lesions,  the  impairment 
of  power  not  being  so  severe  and  not  being  so  characteristic  in  the 
former  as  in  the  latter  case ; 

6.  The  paresis  or  paralysis  which  is  caused  by  lenticular  lesions 
differs  from  that  produced  by  cortical  lesions  in  that  it  is  less  likely  to 
be  dissociated  ;  although  dissociated  lenticular  paresis  may  occur ; 

7.  While  the  loss  of  power  which  results  from  a  destructive  len- 
ticular lesion  is  permanent,  it  is  usually  not  intense  ; 

8.  Persistent  true  motor  aphasia,  as  this  form  of  speech  disorder  is 
generally  understood,  is  not  caused  by  a  lesion  restricted  to  the  len- 
ticula, no  matter  what  its  size  or  destructiveness  ; 

9.  The  insula,  cortex  and  subcortex  play  an  important  part  in  speech 
phenomena,  one  entirely  different  from  that  played  by  the  lenticula 
and  the  internal  capsule  ; 

10.  The  insula  is  a  part  of  the  cortical  motor  center  for  speech, 
Broca's  convolution  j^robably  forming  with  the  insula  the  entire  cor- 
tical motor  centre  for  speech  ; 

11.  Motor  aphasia  may  be  present  without  a  lesion  of  the  left  third 
frontal  convolution  ; 

10 


146         DIAGNOSIS  AND  LOCALIZATION  OF  BRAIN  DISEASES. 

12.  The  lenticula  forms  too  large  a  portion  of  the  cerebral  hemi- 
sphere to  be  regarded  merely  as  a  vestigial  organ. 

They  are  of  interest  in  view  of  the  statements  of  Marie  that  this 
region  has  more  to  do  with  speech  than  has  Broca's  centre.  The 
caudate  nucleus  is  supposed  to  have  some  relation  to  motions  of  the 
legs,  and  the  lenticular  to  those  of  the  arms,  their  relative  development 
in  the  kangaroo  and  bat  suggesting  this.  The  lenticular  nucleus  has 
been  thought  to  have  some  relation  also  to  acts  of  eating. 

Lesions  of  the  Optie  Thalamus.  —  Eoussy,  Sana,  and  JellifTe  have 
recently  outlined  a  combination  of  symptoms  which  they  term  the 


Fig.  75. 


Fig.  76. 


Shows  the  various  connections  of  the 
nuclei  of  the  optic  thalamus.  It  is  joined 
to  all  the  various  regions  of  the  cortex. 
(Compare  this  with  Fig.  76.)     (Jacob.) 


Diagram  of  the  various  nuclei  within  the  optic  thal- 
amus and  their  connections,  sci,  fibres  to  the  callosum; 
l.fr,  fibres  to  first  frontal  convolution;  jRol.  an/.,  fibres  to 
anterior  central  convolution '.  Rol.  post,  fibres  to  posterior 
central  convolution  ;  op.  Rol,  fibres  to  operculum  ;  ci,  in- 
ternal caps  ;  7!rt,  nucleus  anterior ;  dl,  dorsolateral  nu- 
cleus: dr,  dorsoventral  nucleus;  med,  median  nucleus; 
dm  dorso-median  nucleus ;  d(,  mediencephalic  nuc. ; 
cm,  centre  median  ;  I'rf,  vesicula  diencephalica ;  vl,  ven- 
trolateral ;  vb,  ventrobasal ;  ihs,  hypothalamic  fibre;  n.r, 
nucleus  ruber ;  s.n,  substantia  nigra  ;  n.  A,  nucleus  hy- 
pothalamic ;  p.c,  pes  cerebri ;  Ir.  o,  tract  optic  ;  cm, 
corpus  mammillare.     (Jacob. ) 


thalamic  syndrome.  These  are  :  (1)  A  superficial  persistent  hemian- 
esthesia of  an  organic  nature,  more  or  less  marked  for  superficial  sensi- 
bility, tactual  pain,  temperature,  but  always  very  marked  for  deep  sen- 
sibility. (2)  A  mild  hemiplegia,  usually  without  contracture,  and 
rapidly  regressive.  (3)  A  mild  hemiataxia,  and  more  or  less  complete 
astereognosis.     (4)  Severe  pains  on  the  hemiplegic  side,  persistent,  par- 


LOCAL  SYMPTOMS.  147 

oxysmal,  often  intolerable  and  not  yielding  to  any  analgesic  treatment. 
(5)  ChoreQ*-atlietoid  movements  in  the  members  of  the  paralyzed  side. 

The  hemichorea  and  hemiathetosis  which  occasionally  remain  after 
lesions  of  the  thalamus  are  to  be  regarded  as  symptoms  of  irritation 
constantly  exerted  upon  the  motor  or  sensory  tracts  passing  near  it. 
There  are  some  facts  to  support  the  assertion  that  the  optic  thalamus  is 
a  sensory  ganglion,  especially  the  fact  that  lesions  of  the  pulvinar  cause 
hemianopsia.  It  appears  from  anatomical  investigations  of  von  Mona- 
kow  that  all  the  sensory  tracts  end  in  the  thalamus,  which  in  turn  is  con- 
nected by  its  radiations  with  all  parts  of  the  cortex  (Fig.  75).  Each 
sensory  tract  is  bilateral,  hence  a  unilateral  lesion  of  the  thalamus  causes 
no  complete  loss  of  sensation,  von  Monakow  has  distinguished  seven 
separate  masses  of  neurones  in  the  thalamus,  and  traced  the  connection 
of  each  mass  to  one  or  more  distinct  regions  of  the  cortex  (Fig.  76). 
As  yet,  however,  no  practical  diagnostic  conclusions  have  followed  that 
enable  us  to  detect  a  lesion  of  the  parts  of  the  thalamus.  It  has  also  been 
supposed  that  the  thalamus  has  some  function  in  regulating  automatic 
motions.  Thus,  in  lesious  of  the  thalamus  Nothnagel  found  an  absence 
of  the  automatic  facial  expression  indicating  emotion  on  the  side  oppo- 
site to  the  lesion ;  the  patient  did  not  laugh  or  cry  on  that  side  of  the 
face,  though  the  face  was  not  paralyzed.  But  this  is  not  always 
observed.  Meynert  saw  a  case,  of  which  I  also  have  had  an  example, 
of  forced  unnatural  positions  assmned  unconsciously  by  the  arm  and 
leg  on  the  side  opposite  to  a  lesion  of  the  thalamus.  Here,  again,  the 
observations  are  not  uniform  and  are  subject  to  criticism,  as  the  capsule 
may  have  been  irritated. 

The  basal  ganglia,,  doubtless,  have  important  reflex  functions.  The 
thermic  centres  for  the  regulation  of  the  temperature  of  the  body  have 
been  located  by  physiologists  in  the  corpora  striata  and  also  in  the  optic 
thalamus,  though  pathology  does  not  support  this  assertion.  Vasomotor, 
secretory,  and  trophic  control  of  the  opposite  side  of  the  body  has  been 
a  function  assigned  to  the  thalamus.  The  effect  of  emotion  on  these 
functions  is  supposed  to  be  obtained  through  the  action  of  its  neurones. 

As  a  lesion  limited  to  any  one  of  these  ganglia  produces  no  per- 
manent symptoms  whatever,  in  many  cases  we  cannot  detect  such  a 
lesion.  As  a  matter  of  fact,  70  per  cent,  of  the  cases  of  hemiplegia 
are  due  to  a  lesion  in  the  basal  ganglia  affecting  the  internal  capsule ; 
and  from  the  symptoms  and  nature  of  the  disease  its  location  there  can 
usually  be  affirmed.  The  diagnosis  is,  however,  made  from  the  capsular 
symptoms,  as  already  detailed. 

Lesions  of  the  external  capsule  and  of  the  claustrum  cannot  be  yet 
located.  (See  Plate  XI.)  If  on  the  left  side,  they  usually  produce 
paraphasia  like  the  lesions  of  the  island  of  Reil. 

Lesions  of  the  corpora  quadrigemina  are  very  rare.  If  the  anterior 
pair  are  involved,  oculomotor  palsy,  loss  of  pupil  reflex,  strabismus, 
and  nystagmus  may  be  produced.  If  the  posterior  pair  are  involved 
disturbances  of  co5rdination  and  of  hearing  may  be  caused.  As  both 
pairs  are  usually  affected  together,  the  combination  of  these  symptoms 


148      DIAGNOSIS   AND   LOCALIZATION    OF   BEAIN    DISEASES. 

may  aid  in  diagnosis.  Blindness  is  such  a  common  symptom  of  cere- 
bral disease  that  it  is  only  when  it  is  not  due  to  choked  disk,  optic 
atrophy,  or  neuritis,  and  when  it  is  not  of  the  nature  of  hemianopsia, 
that  it  is  to  be  thought  a  local  symptom  of  quadrigeminal  lesion,  and 
some  cases  seem  to  show  that  it  may  not  occur  from  a  lesion  there.  A 
defective  action  of  the  same  branches  of  the  oculomotor  nerves  on  both 
sides  is  rather  more  characteristic  of  quadrigeminal  disease  than  the 
total  affection  of  one  nerve. 

Fig.  77. 


Jiad-jinteriores    W~ 


Position  of  the  nuclei  of  the  cranial  nerves.    The  medulla  and  pons  to  be  imagined  as  transparent. 
The  nuclei  of  origin  (motor),  black  ;  the  end  nuclei  (sensory),  red.     (Edinger.) 

Lesions  of  the  tegmentum  of  the  crura  cerebri,  which  lies  beneath  the 
corpora  quadrigemina.  Since  the  sensory  tracts  pass  through  this 
region,  anaesthesia  may  be  produced  by  such  a  lesion,  and  the  prox- 
imity of  the  corpora  quadrigemina  will  give  rise  to  indirect  local 
symptoms  of  their  affection.  Lesions  of  the  red  nucleus  cause  the 
same  state  of  incoordination  that  occurs  when  the  posterior  pair  of  the 
corpora  quadrigemina  are  involved.  They  also  cause  paralysis  of  the 
third  nerve,  which  passes  through  this  nucleus.  Lesions  of  the  foot 
of  the  crus  cerebri,  in  which  the  motor  tract  passes,  cause  hemiplegia 
of  the  opposite  side.  As  the  third  nerve  issues  through  the  foot  of 
the  crus,  a  lesion  here  causes  a  paralysis  of  this  nerve  on  the  side  of 
the  lesion.  Hence,  hemiplegia  of  one  side,  with  third  nerve  paralysis 
of  the  other  side,  indicates  a  lesion  of  the  foot  of  the  crus  cerebri  on 
the  side  of  the  third  nerve  paralysis.  Lesions  on  the  base  which  press 
upon  this  part  will  produce  the  same  combination  of  symptoms. 

Lesions  of  the  pons  Varolii  and  medulla  oblongata.  Fig.  77  shows 
the  situation  in  the  pons  and  medulla  of  the  nuclei  of  origin  of  the 
cranial  nerves.  These  nuclei  lie  either  upon  the  floor  of  the  fourth 
ventricle,  shown  in  Fig.  52  or  at  a  deeper  level  in  the  formatio  reticu- 
laris. From  the  nuclei  the  nerve  fibres  pass  through  the  pons  and 
medulla  to  make  their  exit  upon  the  base  of  the  brain,  as  shown 
in  Fig.  78.     It  is  evident,  therefore,  that  any  lesion  in  the  pons  or 


LOCAL    SYMPTOMS. 


149 


medulla  will  destroy  either  the  cranial  nerve  nuclei  or  the  nerves 
issuing  frq^n  them  and  traversing  the  cerebral  axis  at  the  level  of  the 
lesion.  The  exact  level  of  the  lesion  will  be  iudicated  by  the  nerves 
affected,  oculomotor  palsy  being  caused  by  lesions  in  the  crus  cerebri ; 
fifth,  sixth,  seventh,  and  eighth  nerve  paralysis  being  caused  by 
lesions  in  the  pons,  and  ninth,  tenth,  eleventh  and  twelfth  nerve 
paralysis  being  caused  by  lesions  of  the  medulla.     The  various  symp- 

FiG.  78. 


Floe  -^, 


ssf  rlV^-  Pyc^^^id^  Spop 


The  base  of  the  brain,  the  cranial  nerves,  and  the  cerebellum.  II,  optic  nerves ;  XII,  optic  chiasm  ; 
If,  infundlbuluin  ;  El,  lateral  part  of  tuber  cinereura  ;  Es,  tuber  cinereum  ;  Tm,  mammillary  bodies ; 
P,  crus  cerebri;  Po,  pons;  Py,  pyramid  of  medulla;  Oi,  olivary  body;  Sma,  transverse  fissure  of 
cerebellum  ;  Floe,  flocculus  ;  ssf,  subfloc.  fissure  ;  rlV4,  diverticulum  of  fourth  ventricle  ;  Pyc,  pyramid 
of  Malacarne ;  Amg,  amygdalus ;  Ldg,  digastric  lobe ;  Lgr,  slender  lobe ;  Lsli,  semilunar  lobe ;  II  to 
XII,  cranial  nerves.     (Dejerine. ) 

toms  due  to  lesions  of  the  cranial  nerves  are  discussed  in  Chapter 
XXXV.,  where  also  the  means  of  distinguishing  lesions  of  the  nuclei 
from  those  of  the  nerve  trunks  are  mentioned. 

The  facts  already  stated  regarding  the  various  tracts  passing  through 
the  pons  and  medulla,  as  shown  in  Figs.  52  to  56,  pages  112  and  116, 


150      DIAGNOSIS    AND   LOCALIZATION    OF   BBAIN    DISEASES. 

will  be  recalled.  It  is  evident  that  a  gross  lesion  like  a  hemorrhage 
or  area  of  softening  in  the  pons  or  medulla  will  cut  those  tracts  and 
produce  either  unilateral  or  bilateral  paralysis  of  motion  or  of  sensa- 
tion in  the  limbs  and  body.  This  combination  of  symptoms  of  cranial 
nerve  paralysis  and  of  paralysis  in  the  limbs  and  body  is  characteristic 
of  lesions  in  the  pons  and  medulla,  and  leads  at  once  to  the  diagnosis 
and  localization  of  such  lesions.     The  pons  also  contains  the  middle 

Fig.  79. 


The  basal  ganglia  and  the  cerehellura.  Ic,  taenia ;  NC,  caudate  nucleus  ;  Th,  thalamus ;  Ca,  Corp. 
quad,  ant.;  Cop,  posterior  coiuinissure ;  Com.  cent,  commissure;  V(Cu),  culmen ;  Lqp,  lobus  quad- 
ratus;  Sv,  fissure  of  Vicq  d'Azyr;  V(dc),  monticulus ;  Scf,  circular  fissure;  Lsli,  inferior  semilunar 
lobe ;  Lsls,  superior  semilunar  lobe ;  Ls,  superior  lobe ;  LP,  posterior  lobe ;  Bt,  vermiform  lobe,  of 
which  the  culmen  and  monticulus  are  parts.    (Dejerine.) 

peduncle  of  the  cerebellum,  hence  lesions  in  the  pons  are  likely  to 
cause  symptoms  of  cerebellar  type  already  considered. 

Cerebellar  Symptoms. — Figs.  80  and  82  show  the  connections  of 
the  cerebellum  with  the  cord,  with  the  pons,  and  with  the  cerebrum. 
Fig.  81  shows  the  structure  of  the  cerebellar  cortex. 

Lesions  of  the  cerebellum,  if  located  in  the  hemisphere  and  not  in 
the  median  or  vermiform  lobe,  and  if  of  such  a  nature  as  not  to  exert 
pressure  on  surrounding  parts,  may  not  produce  any  symptoms.     If 


LOCAL  SYMPTOMS. 


151 


the  lesion  is  in  the  vermiform  lobe,  disturbance  of  codrdination  known 
as  cerebellar  ataxia,  occurs.  This  consists  in  an  inability  to  walk 
without  staggering  like  a  drunken  man.  The  ataxia  exists  only- 
while  the  patient  is  in  an  upright  position ;  it  rarely  affects  the  motions 
of  the  arms,  and  when  it  does  it  never  interferes  with  the  fine  adjust- 
ments, but  only  with  extensive  movements  in  space  —  e.  g.,  grasp- 
ing objects  at  a  distance  —  that  involve  an  act  to  preserve  the  balance. 

Fig.  80. 


Diagram  to  show  the  connections  of  the  cerebellum.  SC,  spinal  cord ;  G,  column  of  GoU ;  B, 
column  of  Burdach  ;  MED,  medulla  oblongata  at  sensory  decussation  ;  0,  olive ;  G,  nucleus  gracilis ; 
C,  nucleus  cuneatus  ;  V,  fifth  nerve  ;  Pons,  pons  Varolii ;  VIII,  eighth  nerve  and  its  nucleus  ;  F,  fillet ; 
Py,  pyramids ;  Crus,  crus  cerebri ;  RN,  red  nucleus  of  tegmentum ;  CD,  corpus  dentatum  of  cerebel- 
lum. 1  to  13,  various  tracts  connecting  the  cerebellum  with  the  spinal  cord,  medulla,  pons,  and  crus. 
(Starr,  Atlas  of  Nerve  Cells. ) 

Closing  the  eyes  does  not  increase  the  ataxia.     In  these  respects  the 
ataxia  differs  from  that  of  posterior  sclerosis. 

A  second  characteristic  symptom  of  cerebellar  disease  located  in  the 
vermiform  lobe  is  vertigo.  This  may  be  very  severe,  but  as  it  may 
occur  without  ataxia,  and  ataxia  may  be  present  without  vertigo  (though 
rarely),  the  two  are  not  to  be  considered  as  interdependent.  Vertigo 
is  increased  by  rising  to  an  erect  position,  but  may  persist  when  the 


152       DIAGNOSIS   AND   LOCALIZATION    OF   BEAIN    DISEASES. 

patient  is  in  bed.  It  decreases  somewhat  when  the  patient  has 
remained  fixed  in  any  position  for  some  time ;  but  is  always  increased 
when  he  opens  his  eyes.  The  vertigo  is  usually  an  early  symptom 
of  cerebellar  disease.  It  is  more  constant  and  persistent  in  cases 
in  which  the  intracranial  pressure  is  increased.  It  may  gradually 
pass  off  in  other  cases.  Cerebellar  vertigo  does  not  differ  from  vertigo 
in  M6ni6re's  disease,  and  is  probably  due  to  an  affection  of  the  ter- 

FiG.  81. 


Diagrammatic  representation  of  a  section  through  the  cerebellar  cortex.  7,  molecular  layer ;  II, 
granular  layer ;  ///,  white  matter ;  P,  Purkinje  cell  with  its  neuraxone,  p,  entering  the  white  matter  ; 
S,  small  stellate  cells  of  molecular  layer ;  B,  large  stellate  cells  with  basket  fibres,  6  —  these  basket  fibres 
surround  the  body  of  the  Purkinje  cell  shown  in  dotted  outline  ;  G,  cells  of  the  granular  layer,  with 
long,  straight  neuraxone,  g,  ascending  to  molecular  layer,  and  there  bifurcating  to  become  tangential 
fibres  —  these  fibres  run  at  right  angles  to  the  plane  of  section  of  the  plate;  M,  moss-like  termination 
of  white  fibres,  m,  entering  the  cerebellum  from  without ;  If,  large  Golgi  cell  of  the  second  lype,  with 
dendrites  in  both  granular  and  molecular  layers  and  neuraxone  dividing  and  subdividing  in  the  gran- 
ular layer ;  I,  terminal  filaments  and  fibres,  f,  entering  the  cerebellum  from  without  and  ending  around 
the  branches  of  the  Purkinje  cells.     (Starr,  Atlas  of  Nerve  Cells. ) 


minal  fibres  of  the  labyrinthine  part  of  the  eighth  nerve,  from  the 
semicircular  canals  or  their  nuclei.  The  vertigo  of  Meniere's  disease 
is,  however,  usually  accompanied  by  deafness.  Ataxia  and  vertigo 
together  afford  strong  presumption  of  disease  in  the  vermiform  lobe, 
although  neither  alone  is  sufficient  for  a  diagnosis. 

Another  symptom  of  cerebellar  disease  is  a  loss  of  tone  in  the 
muscles  of  the  back  and  neck  leading  to  a  lack  of  power  to  maintain 
the  balance  and  the  erect  posture.  This  often  results  in  abnormal 
postures  of  the  body  and  in  a  tendency  for  the  head  to  be  held  away 
from  the  side  of  the  lesion.  The  relaxation  of  the  muscles  may  occur 
suddenly  from  time  to  time  and  is  not  constant.  Each  cerebellar 
hemisphere  controls  the  muscles  of  its  own  side. 


LOCAL  SYMPTOMS.  153 

The  indirect  local  symptoms  of  cerebellar  disease  may  be  numer- 
ous. The^are  due  to  ^an  affection  of  the  tracts  and  nerve  nuclei  in 
the  pons  and  medulla  (see  Fig.  53).  Various  forms  of  paralysis  and 
anesthesia,  vasomotor  disturbances,  obstinate  vomiting  of  a  projectile 
character,  general  symptoms  of  intracranial  disease, — e.  g.,  headache, 
optic  neuritis — are  usually  present  with  tumors,  abscesses,  or  hemor- 
rhages in  the  cerebellum,  especially  if  they  are  in  the  median  lobe. 


Diagram  showing  the  connections  of  the  cerebellum,  cbl,  cerebellum;  m.sp.,  direct  cerebellar 
fibres  from  spinal  cord;  oliv,  olive  ;  VIII,  labyrinthine  portion  of  auditory  nerve  ;  pchs.,  superior  pe- 
duncle to  the  red  nucleus  (re.  rub);  e.g.,  corpora  quadrigemina ;  frs.,  fibres  from  middle  cerebellar 
peduncle  ;  fd,  fibres  from  pons  nuclei  to  cerebellum.     (Jacob.) 

The  combination  of  cerebellar  ataxia  and  vertigo  with  these  and  other 
symptoms  of  pons  disease  affords  clear  evidence  of  disease  in  the 
cerebellum. 

Lesions  of  the  cerebellum  have  no  apparent  efi^ect  upon  the  mental 
powers  when  they  occur  in  adults.  A  deficient  development  of  the 
cerebellum  is,  however,  a  frequent  cause  of  congenital  idiocy.  When 
one  cerebellar  hemisphere  fails  to  develop,  the  opposite  olivary  body 
in  the  medulla,  and  sometimes  the  opposite  hemisphere  of  the  cerebrum, 
present  an  atrophic  appearance. 

Lesions  of  the  middle  peduncle  of  the  cerebellum,  the  crura  cere- 
belli  ad  pontem,  produce  characteristic  symptoms.  These  consist  in  a 
tendency  on  the  part  of  the  patient  to  assume  a  forced  position,  to  turn 
toward  or  fall  toward  one  side  in  walking,  or  even  to  revolve  constantly 
about  one  axis  of  his  body.  The  forced  movements  may  be  made  by 
the  eyes  (conjugate  deviation  in  one  direction),  by  the  head,  or  by  the 
entire  body.  In  a  case  seen  by  the  writer,  in  which  the  autopsy 
showed  a  tuberculous  tumor  in  the  left  middle  peduncle  and  in  the 
vermiform  lobe,  in  addition  to  ataxia,  vertigo,  vomiting,  and  headache, 
the  patient  lay  constantly  on  his  left  side,  and  when  he  turned  upon  his 
back  or  toward  the  right  side  the  vertigo  became  so  excessive  that  he 
was  obliged  to  resume  at  once  his  former  position.  In  walking,  this 
patient  showed  a  tendency  to  fall  toward  the  left  side,  and  found  it 
impossible  to  turn  around  toward  the  right.  In  another  case,  a  lacera- 
tion of  one  peduncle  due  to  a  fracture  of  the  base,  caused  constant 


154       DIAGNOSIS  AND  LOCALIZATION  OF  BEAIN  DISEASES. 

vigorous  movements  of  rotation  about  the  long  axis  of  the  body  toward 
the  affected  side  for  three  days  until  death  occurred.  Such  patients 
may  lose  their  balance  in  moving  in  one  direction  —  e.  g.,  forward  or 
backward,  and  in  attempting  to  regain  it  they  may  be  obliged  to  hasten 
their  movements.  This  has  been  interpreted  wrongly  as  a  tendency 
to  compulsory  walking  in  one  direction  —  e.  g.,  backward.  It  is 
really  due  to  the  vertigo.     Lesions  of  the  other  peduncles  of  the  cere- 


FiG.  83. 


^Floculies 


Med.^fi 


Diagram  showing  the  origin  and  course  of  the  fibres  of  the  peduncles  of  the  cerebellum. 

(Edinger.) 

bellum  do  not  produce  any  known  characteristic  symptoms  aside  from 
those  of  cerebellar  disease.  But  when  the  superior  peduncles  are  the 
seat  of  a  lesion  it  is  not  uncommon  to  have  oculomotor  paralysis, 
especially  paralysis  of  the  fourth  nerve,  as  an  associated  symptom. 
And  when  the  inferior  peduncle  is  affected  there  is  usually  an  alter- 
nating hemiansesthesia  from  lesion  of  the  formatio  reticularis. 


LOCAL  SYMPTOMS.  l55 

.  THE  EXAMINATION  OF  THE  CEREBRO-SPINAL  FLUID. 

The  examination  of  the  cerebrospinal  fluid,  obtained  by  lumbar 
puncture  in  accordance  with  the  method  of  Quincke,  is  of  value  in  some 
cases  of  nervous  disease.  Quincke's  puncture  should  be  done  with 
strict  care  as  to  asepsis,  but  without  an  anaesthetic.  Local  anaesthesia 
with  chlorethyl  spray  may  be  used.  It  is  performed  by  laying  the 
patient  upon  the  side  and  bending  the  body  as  far  forward  as  possible, 
the  thighs  being  flexed.  A  hollow  needle  one  millimetre  in  diameter 
is  then  thrust  between  the  third  and  fourth  lumbar  spines  in  the  middle 
line  directly  into  the  spinal  canal,  its  direction  being  a  little  upward  in 
order  to  avoid  the  lamina.  The  fourth  lumbar  spine  is  on  a  line  con- 
necting the  crests  of  the  ilium.  It  may  be  thrust  from  three  to  eight 
centimetres  before  reaching  the  spinal  canal,  according  to  the  age  and 
fatness  of  the  patient.  When  the  stylet  is  withdrawn  the  cerebro- 
spinal fluid  will  flow  out  of  the  needle,  either  in  a  gush  (if  there  is  a 
great  increase  of  pressure)  or  drop  by  drop  (if  there  is  little  pressure). 
If  it  does  not  flow  freely  the  flow  can  be  increased  by  placing  the 
patient  in  an  upright  position.  From  a  few  drops  to  fifty  cubic  centi- 
metres may  be  withdrawn  without  any  ill  effects.  The  wound  is  to  be 
closed  with  collodion. 

The  normal  cerebro-spinal  fluid  is  clear,  and  contains  no  lympho- 
cytes, or  not  more  than  two  or  three  to  the  cubic  centimetre  after  cen- 
trifugalization.  It  flows  slowly  from  the  needle  and  there  is  little 
pressure.  In  disease  it  may  be  cloudy,  containing  fibrous  shreds  and 
pus  ;  it  may  be  loaded  with  lymphocytes,  from  100  to  1000  to  the  cubic 
centimeter  and  it  may  be  so  increased  in  amount  as  to  spurt  with  force 
from  the  needle  showing  a  marked  rise  of  pressure.  It  may  also 
contain  various  microorganisms  and  leucocytes. 

In  all  cases  of  syphilis  a  considerable  number  of  lymphocytes  are 
found ;  hence  in  cases  of  syphilitic  tabes,  of  syphilitic  paresis,  of  syphilis 
of  the  brain  or  cord,  of  syphilitic  meningitis  or  gumma  the  examina- 
tion gives  positive  results.  In  meningitis  of  the  brain  or  cord  and  in 
diifuse  abscess  of  the  brain  both  lymphocytes  and  microorganisms  are 
found.  These  may  consist  of  diplococci,  or  pneumococci,  or  staphyl- 
ococci, or  streptococci .  pyogenes,  in  accordance  with  the  nature  of  the 
case.  Their  presence  is  of  therapeutic  as  well  as  of  diagnostic  signifi- 
cance, in  view  of  recent  discoveries  of  serum  therapy.  In  neuras- 
thenia, in  alcoholism  and  toxic  neuritis,  in  the  psychoses,  and  in  all 
forms  of  nervous  disease  in  which  there  is  no  syphilitic  element,  the 
fluid  is  clear  and  contains  no  lymphocytes.  The  pressure  of  the  fluid 
is  raised  in  all  the  conditions  in  which  meningitis  is  present.  It  may 
also  be  greatly  raised  in  hydrocephalus,  in  tumors  of  the  brain  or  cord, 
and  in  ursemia.  The  proteid  content  of  the  fluid  is  said  to  be  raised 
in  all  cases  where  the  lymphocytes  are  found. 


156         DIAGNOSIS  AND  LOCALIZATION  OF  BBAIN  DISEASES. 

CRANIOCEREBRAL    TOPOGRAPHY. 

The  diagnosis  of  local  lesions  in  the  brain  leads  in  many  cases  to 
surgical  treatment  for  the  removal  of  the  disease,  such  as  clots,  tumors, 
or  abscesses.  It  is,  therefore,  essential  to  know  the  exact  relation 
between  prominent  parts  of  the  skull  and  the  fissures  and  convolutions 
of  the  brain.  This  relation  has  been  carefully  determined  and  certain 
rules  have  been  laid  down. 

The  relation  of  the  brain  to  the  skull  is  shown  in  Fiir.  84. 


Fig.  84. 


^^-57Cfi^s)fanceyy^^ 


The  relation  of  the  fissures  of  Rolando  and  Sylvius  to  the  skull. 

The  rules  for  finding  the  fissures  of  Sylvius  and  Rolando  by  measur- 
ing the  skull  are  as  follows  : 

To  find  the  fissure  of  Rolando,  lay  down  a  line  from  the  root  of  the 
nose  to  the  occipital  protuberance  over  the  top  of  the  head,  and  take 
a  point  0.557  of  the  distance  back  upon  this  line.  This  point  will 
correspond  to  the  upper  end  of  the  fissure.  The  fissure  makes  an 
angle  of  67°  with  the  median  line  just  measured.  Hence  if  two  strips 
of  metal,  fixed  to  one  another  at  this  angle,  be  placed  on  the  head  with 
their  junction  upon  the  upper  end  of  the  fissure,  when  one  strip  is  on 
the  median  line  the  other  strip,  pointing  forward  and  downward,  must 
lie  over  the  fissure  of  Rolando.  In  its  lower  third  the  fissure  becomes 
a  little  more  vertical  than  the  strip.  The  fissure  is  about  three  and  a 
half  inches  long. 


LOCAL  SYMPTOMS.  157 

To  find  the  fissure  of  Sylvius,  lay  down  a  base  line  from  the  lower 
margin  of  th^orbit  to  the  auditory  meatus.  Lay  down  a  second  line 
parallel  to  the  base  line  from  the  external  angular  process  of  the  frontal 
bone  backward  one  inch  and  a  quarter,  and  then  measure  upward  one- 
quarter  of  an  inch  ;  this  gives  point  one.  Find  the  most  prominent 
part  of  the  parietal  eminence,  and  from  it  draw  a  line  downward  per- 
pendicular to  the  base  line,  and  on  this  take  a  point  three-quarters  of 
an  inch  below  the  eminence ;  this  gives  point  two.  Join  these  two 
points,  and  the  line  will  lie  over  the  fissure  of  Sylvius.  The  anterior 
limb  of  the  fissure  will  be  two  inches  behind  the  external  angular 
process.     The  fissure  of  Sylvius  is  about  four  inches  long. 

To  find  the  parieto-occipital  fissure,  continue  the  line  of  the  fissure 
of  Sylvius  to  the  median  line.     At  their  junction  lies  this  fissure. 

Since  all  areas  now  open  to  surgical  operation  can  be  located  with  a 
definite  relation  to  these  three  fissures,  no  further  rules  are  necessary. 
As  in  opening  the  skull  it  is  customary  to  make  a  fenestrum  of  at  least 
an  inch  in  diameter,  and  as  it  is  frequently  necessary  to  enlarge  the 
opening  much  more,  a  procedure  in  no  way  dangerous  under  aseptic 
conditions,  there  is  no  difficulty  In  recognizing  the  fissures  and  con- 
volutions exposed  if  the  rules  are  closely  followed.  Prior  to  the  large 
incision  of  the  scalp  it  Is  well  to  mark  certain  points  upon  the  skull 
by  the  sharp  point  of  a  chisel,  so  that  when  the  bone  is  laid  bare  sur- 
face landmarks  may  still  be  kept  in  view. 


PAET  II. 
ORGANIC  NERVOUS  DISEASES. 


SECTION  L 

INJURIES  AND  DISEASES  OF  THE  NERVES. 


CHAPTER    VI. 

INJUEIES  OF  NERVES.     NEUEITIS. 
INJURIES  OF  NERVES. 

IsTeeves  are  frequently  injured,  being  exposed  in  their  long  course. 
The  pathological  effects  of  such  injury  and  the  symptoms  produced  are 
somewhat  different  from  those  due  to  neuritis.  Hence  they  require 
a  separate  consideration.  The  pathological  effects  of  injuries  to  nerves 
have  been  ascertained  very  largely  from  experimental  division  in  ani- 
mals, but  there  is  every  reason  to  believe  that  the  process  in  man  is 
identical  with  that  in  animals.  These  effects  are  still  a  matter  of  con- 
troversy, different  observers  having  seen  different  appearances. 

After  division  of  a  nerve  trunk  a  process  of  degeneration  sets  in  at 
the  point  of  injury  and  involves  a  small  portion  of  the  central  end, 
and  the  entire  peripheral  part  of  the  nerve,  from  the  seat  of  injury 
onward.  This  process  may  be  more  or  less  complete,  and  may  or  may 
not  be  followed  by  a  second  process  of  regeneration  in  the  injured 
nerve.  It  is  necessary  to  distinguish  between  the  degenerative  and 
regenerative  processes. 

The  Process  of  Degeneration.  —  When  a  nerve  is  compressed  by  a 
ligature  or  forceps  without  sufficient  force  to  rupture  the  sheath  of 
Schwann,  the  myelin  is  driven  away  from  the  point  of  pressure  in  both 
directions  and  the  axis  cylinder  is  disintegrated  and  mingled  with  it. 
It  might  be  supposed  that  the  nodes  of  Ranvier  would  prevent  such  a 
driving  back  of  the  myelin,  but  they  seem  to  offer  but  feeble  resist- 
ance, so  that  the  entire  fibre  on  either  side  of  the  compressed  spot  is 
bulged  out  for  some  little  distance,  the  sheath  of  Schwann  between  the 
distended  portions  being  left  empty  or  containing  only  a  little  granular 
debris.  If  the  sheath  of  Schwann  is  ruptured  or  is  cut  through  the 
myelin  exudes  in  little  drops,  which  are  mingled  with  the  debris  of 
the  axis  cylinder.  In  a  short  time  changes  of  a  degenerative  character 
are  observed  in  the  nerve  on  both  sides  of  the  point  of  compression  or 

159 


160 


INJUBIES  OF  NEBVES.     NEUBITIS. 


division.  Those  on  the  central  side  are  limited  to  the  immediate 
neighborhood  of  the  injured  spot,  and,  according  to  Ranvier/  do  not 
affect  the  nerve  for  a  distance  greater  then  a  centimetre  from  the  point 
of  compression.  Recent  investigators,  however,  find  certain  minor 
grades  of  degeneration  throughout  its  entire  length  up  to  the  neurone 
body;  and  a  secondary  atrophy  of  the  body  itself  after  long  disuse, 
as  after  amputation  of  a  limb.  On  the  peripheral  side  of  the  point  of 
pressure  the  degeneration  is  complete,  involving  the  entire  nerve  down 
to  its  finest  terminations.  The  process  begins  at  once  in  the  entire 
length  of  the  nerve.     The  first  change  noticed  is  a  breaking  up  of  the 

Fig.  85. 


Degeneration  of  a  nerve  seven  days  after  injury.    The  upper  specimen  is  a  normal  nerve ; 
the  lower  is  degenerated.    Osmic  acid  stain. 

myelin  sheath  into  segments,  and  then  into  smaller  masses  and  drops 
(Fig.  85),  which  finally  undergo  further  disintegration,  either  by  a 
fatty  or  albuminoid  degeneration  or  by  a  process  of  saponification, 
until  a  finely  granular  mass  alone  remains.  Hence  the  contour  of  the 
fibre  becomes  irregular,  the  sheath  of  Schwann  bulging  at  places  with 
the  fatty  mass  and  at  other  places  being  collapsed  and  empty. 

As  the  myelin  undergoes  these  changes  the  axis  cylinder  usually 
is  split  up  into  segments  and  degenerates  into  a  granular  mass.     In 

^  Lefons  sur  I'histologie  du  systeme  nerveux,  tome  i.,  p.  115, 


INJUBIES   OF  NERVES. 


161 


very  slight  injuries  it  may  remain  intact,  and  although  deprived  of  its 
function,  it  ^s  capable  of  resuming  that  function  at  any  time  when 
regeneration  of  the  myelin  sheath  has  taken  place  (Fig.  86). 

The  sheath  of  Schwann  also  takes  part  in  the  process  of  degenerar- 
tion.  When  that  process  has  fairly  begun  numerous  nuclei  are 
observed  lining  this  sheath  in  each  interannular  segment.  They  may 
have  come  by  a  process  of  segmentation  from  the  original  nucleus  of 

Fig.  86. 


_t'»rfi*?.        '-v^  * 


•u 


^fte^f***.:.*      *      ^\ 


«••' 


*€•*■-. 


"n'iSi      ''»-   ** 


,  ■»-(!        ttigM,. 


Degeneration  of  a  nerve  fourteen  days  after  injury.    Osmic  acid  stains  the  fatty  debris  of  the 
medullary  sheath  black. 

the  segment,  as  Ranvier  holds.  But  Neumann  and  Mayer  have  shown 
that  they  appear  as  early  at  the  extremities  of  the  segment  as  they  do 
in  the  vicinity  of  the  nucleus,  and  therefore  consider  them  a  new  for- 
mation originating  in  the  granular  or  protoplasmic  mass.  Tizzoni 
thinks  them  emigrated  corpuscles,  while  Rosenheim  holds  that  they 
come  from  the  connective-tissue  cells  along  the  sheath,  which  divide 
and  multiply  and  show -powers  of  emigration  as  soon  as  the  process  of 
degeneration  begins.  When  the  granular  mass  is  absorbed  these 
nuclei  remain  scattered  along  the  sheath  of  Schwann,  and  it  has  been 
suggested  that,  when  in  an  empty  sheath  a  new  axis  cylinder  appears, 
it  owes  its  existence  to  these  nuclei  which  arrange  themselves  in  a  line 


11 


162 


INJURIES  OF  NEEVES.     NEUEITIS. 


and  develop  into  the  new  fibre  (Wolberg).  This  view,  however,  has 
not  met  with  general  acceptance,  though  recently  urged  by  Ballance 
and  Stewart/  If  no  regeneration  occurs  they  disappear  gradually, 
and  then  the  only  relic  of  the  former  nerve  fibre  is  the  empty,  col- 
lapsed sheath  of  Schwann,  which  remains  as  a  connective-tissue  strand. 
The  increase  of  nuclei  and  connective-tissue  fibrils  in  the  endo- 
neurium  and  perineurium  which  accompanies  the  process  of  degen- 
eration, aids  in  the  transformation  of  the  nerve  into  a  band  of  connec- 
tive tissue.     (Fig.  87.) 

Fig.  87. 


Cross-section  of  an  ulnar  nerve  in  a  state  of  degeneration.    The  bundles  of  nerves  on  the  left  side 
are  completely  degenerated. 

The  degeneration  that  affects  the  nerve  is  continued  to  the  terminal 
plates. upon  the  muscle.  These  are  changed  into  masses  of  granules 
and  are  finally  absorbed,  connective-tissue  plates  being  left.^  Whether 
any  changes  occur  in  the  sensory  terminal  organs,  such  as  the  tactile 
corpuscles  or  terminal  bulbs,  has  never  been  ascertained.  Those  who 
believe  that  the  individual  axis-cylinder  fibrils  terminate  in  the  epi- 
thelium of  the  skin  cite  the  trophic  changes  that  often  occur  on  the 
surface  as  evidence  that  this  covering  of  the  body  shares  in  the  nerve 


^The  Healing  of  Nerves.     The  IMacmillan  Co.,  1902. 

^  Gessler.     Die  motorisclie  Endplatte  und  ilire  Bedeutung  fiir  die  periphere  Lahmung. 
Leipzig,  1885, 


PLATE  XII 


a  J     - 


/f 


r-.  i 


■I 


^;.;;);ii- 


,      c       «;  ■  ,ff 


Cross-section  of  the  Brachial  Plexus  in  a  Case  of  Neuritis, 
(Marchi  stain.) 

The  degenerated  fibres  in  the  different  bundles   of  nerves   {i>)   are  stained  black.     In 

every  bundle  a  number  of  such  fibres  are  to  be  seen,    g,  artery  ;y,  fat. 

(Flatau,  Spec.  Pathol,  u.  Therap.,  Nothnagel,  Bd.  xi.,  Taf.  iii.) 


INJURIES   OF  NERVES. 


163 


changes.  The  process  of  degeneration  is  com- 
plete in  aboi^  two  weeks  after  the  injury. 

The  cut  end  of  the  divided  nerve  becomes 
swollen  into  a  bulbous  extremity  by  a  growth  of 
connective  tissue  and  by  the  development  of 
fine  nerve  fibres  in  process  of  regeneration. 
This  forms  a  very  sensitive  scar. 

A  true  union  of  the  divided  ends  rarely  occurs. 
All  surgeons  believe  that  function  may  be  rap- 
idly resumed  after  suture  of  freshly  divided 
nerves,  and  rapid  restoration  of  sensation,  ab- 
sence of  wasting  and  retention  of  muscular  con- 
tractility may  be  taken,  according  to  Bowlby, 
as  certain  proof  of  restoration  of  continuity  of 
a  divided  nerve.  But  experiments  upon  ani- 
mals do  not  confirm  this  view.  It  seems  prob- 
able that  under  different  circumstances  different 
processes  occur.  It  is  undoubtedly  true  that 
in  some  cases  the  degenerative  process,  so 
graphically  pictured  by  Ranvier  goes  on,  from 
the  beginning  segmentation  of  the  myelin  down 
to  the  final  result  in  the  connective-tissue  strand, 
the  relic  of  the  empty  sheath  of  Schwann  ;  while, 
in  other  cases  of  a  less  serious  nature,  the  de- 
struction is  less  complete  and  there  remains  a 
fibre  consisting  of  a  sheath  of  Schwann,  con- 
taining a  granular  mass  which  may  be  either 
an  axis  cylinder  or  a  mass  capable  of  develop- 
ing into  an  axis  cylinder  under  favorable  cir- 
cumstances. If  this  is  the  case  we  can  affirm 
that  brilliant  surgical  successes,  with  rapid 
restoration  of  nerve  function  after  suture, 
are  possible  when  only  partial  degeneration  is 
present,  but  are  impossible  when  total  and 
extensive  destruction  of  the  nerve  fibre  has 
occurred. 

The  Process  of  Regeneration.  — The  proc- 
ess of  regeneration  begins  about  two  weeks  after 
an  injury  or  experimental  section.  With  regard 
to  the  method  of  this  process  two  divergent 
views  are  held.  Ranvier  and  his  followers 
affirm  that  the  new  nerve  is  wholly  a  product 
of  the  central  end  of  the  injured  nerve,  grow- 
ing out  from  it  and  making  its  way  along 
the  track  of  the  peripheral  end,  which  takes 
no  active  part  in  the  process.  The  central 
end  of  an  individual  nerve  fibre  becomes  hy- 
pertrophied,  and  from  this  swollen  part  a  sin- 


FlG. 


-sc/twz 


.-.>-vxa 


-M tr^s 


Regeneration.  Longitu- 
dinal section  of  a  nerve  fibre 
from  a  case  of  lead-neuritis  in 
a  guinea  pig.  Biclschowsky 
stain,  ax,  axis  cylinder  ;  ax, 
newly  formed  axis  cylinder 
branch  dividing  into  many 
new  axones;  r.  sohw.,  nucleus 
of  Schwann  sheath  ;  ms,  my- 
elin ;  m.t,  degenerated  myelin. 
(Doinikow,  Beitragezur  histo- 
path.  d.  peri  ph.  nerven.,Nissl'S 
Arbciteu,  IV.,  4^15,  1911.) 


164  INJUBIES  OF  NEBVES.     NEUBITIS. 

gle  new  fibre  starts  out  already  medullated  and  grows  onward  into  the 
old  sheath,  which  it  follows  down  until  it  reaches  its  end.  By  the  aid 
of  the  nerve  callus  or  cicatricial  tissue,  that  usually  joins  the  central  end 
with  the  degenerated  peripheral  end,  the  new  fibres  are  directed  out- 
ward toward  the  periphery.  And  when  they  reach  the  peripheral  end 
of  the  cut  nerve  they  insinuate  themselves  into  the  old  remaining 
sheaths  of  Schwann  or  between  those  sheaths,  and  grow  on  and  out- 
ward until  at  last  they  reach  the  termination  of  the  peripheral  end,  and 
the  regeneration  is  complete.  The  terminal  plates  upon  the  muscles 
are  renewed  by  a  reproduction  of  protoplasm  in  the  plate.  The 
process  thus  described  is  in  accordance  with  the  process  of  original 
development  of  nerve  fibres  from  nerve  cells  in  embryonal  life  as  has 
been  recently  proven  by  Harrison.  ^  Neumann  and  Mayer,  and  more 
recently  Ballance  and  Stewart,  have  affirmed,  on  the  contrary,  that  the 
regeneration  goes  on  in  the  peripheral  end  of  the  cut  nerve,  segment 
by  segment  being  formed  successively  or  simultaneously,  the  new  nerve 
being  built  up  by  the  union  of  each  distal  segment  with  the  one  lying 
centrally  to  it,  until  the  process  is  complete.  They  hold  that  the 
process  of  regeneration  begins  in  the  granular  mass  left  in  the  sheath 
of  Schwann.  Within  it  they  have  seen  a  narrow  band  of  fine  homo- 
geneous substance  appear,  which  has  the  structure  of  a  rudimentary 
axis  cylinder.  This  does  not  fill  the  sheath  of  Schwann  and  is  often 
pressed  aside  by  the  nuclei  which  lie  in  that  sheath.  It  is  not  at  first  con- 
tinuous with  the  end  of  the  old  axis  cylinder,  remaining  in  the  central 
part  of  the  compressed  or  divided  segment,  but  as  it  increases  in  definite 
structure  it  approaches  this  old  axis  cylinder,  and  finally  unites 
with  it.  At  the  point  of  union  a  ring  of  Ranvier  is  formed.  As 
this  axis  cylinder  develops  a  substance  is  gradually  formed  around 
it,  which  is  stained  by  osmic  acid.  This  increases  in  thickness  as 
the  protoplasmic  mass  and  the  nuclei  diminish,  until  it  finally  forms 
a  new  myelin  sheath.  The  new  myelin  sheath  is  never  continuous 
with  the  old  one  in  the  central  end  of  the  nerve,  since  it  is  sep- 
arated from  that  by  the  ring  of  Ranvier ;  but  often  at  first  the  old 
sheath  seems  to  bulge  out  and  encircle  the  new  sheath,  though  this 
appearance  is  never  permanent.  At  the  point  of  union  of  the  new 
fibre  with  the  old  one  nuclei  are  often  found,  but  these,  like  the  others, 
gradually  disappear.  Lastly,  a  new  sheath  of  Schwann  is  produced 
around  the  new  myelin  sheath  and  within  the  old  sheath  of  Schwann. 
It  presses  aside  the  old  sheath,  together  with  such  masses  of  protoplasm, 
drops  of  myelin  and  nuclei  as  may  remain,  leaving  them  thus  wholly 
outside  of  the  new-made  fibre,  so  that  they  coalesce  with  and  make  part 
of  the  endoneurium.  The  new  sheath  of  Schwann  has  but  one  nucleus 
in  each  segment  and  presents  the  nodes  of  Ranvier  at  regular  intervals. 
Neumann  has  shown  that  this  process  goes  on  in  every  individual 
segment  of  the  nerve  sheath,  so  that  in  segment  by  segment,  proceed- 
ing toward  the  periphery,  the  regenerative  changes  occur,  and  as  each 
segment  approaches  completion  it  joins  itself  to  the  preceding  one, 

^  Johns  Hopkins  Hospital  Eeports,  January,  1906. 


INJUBIES   OF  NEBVES.  165 

until  finally  the  nerve  is  reestablished  in  its  entire  length.  As  the 
degenerative*  process  begins  in  the  segment  nearest  to  the  point  of 
compression,  so  does  the  process  of  regeneration,  and  in  some  nerves 
the  two  processes  may  be  seen  going  on  together,  the  segments  near 
the  seat  of  injury  being  renewed,  while  those  at  the  periphery  are  still 
in  a  process  of  degeneration.  The  new  fibres  are  at  first  somewhat 
smaller  in  calibre  than  the  old  ones,  but  they  gradually  attain  a  normal 
size,  and  then  the  process  may  be  said  to  be  completed. 

Ballance  and  Stewart  afl&rm  that  the  regeneration  originates  from  the 
nuclei  of  the  old  sheath,  or  even  from  connective-tissue  cells  and  neu- 
rilemma nuclei  remaining  in  the  connective-tissue  strand.  The  most 
important  evidence  of  such  regeneration  is  offered  by  Bowlby.^  In 
three  cases  of  division  of  nerves  in  which  union  was  attempted  by  opera- 
tion some  months  after  the  injury,  he  found  regenerating  nerves  in  the 
peripheral  portion.  There  were  new  fine  fibres  much  smaller  than 
natural,  and  in  some  the  myelin  sheath  was  scarcely  perceptible.  In 
some,  however,  the  myelin  sheath  was  fully  developed,  including  the 
nodes  of  Ranvier.  The  origin  of  these  new  fibres  was  clearly  from 
nuclei  which  seemed  to  be  identical  with  the  nuclei  of  the  sheath  of 
Schwann.  These  had  arranged  themselves  in  bundles  with  their  long 
axis  parallel  to  that  of  the  nerve  trunk.  Then  the  nuclei  had  elon- 
gated and  finally  been  transformed  into  fibres  around  which  subsequently 
a  myelin  sheath  was  formed.  A  similar  process  has  been  fully  de- 
scribed by  Ballance  and  Stewart. 

It  is  evident,  therefore,  that  the  process  of  regeneration  varies  in  dif- 
ferent conditions  according  to  the  exact  stage  of  degeneration  reached 
before  it  begins.  If  the  final  product  of  degeneration  is  a  band  of 
simple  connective  tissue,  it  seems  probable  that  the  nerve  fibre  will 
have  to  grow  into  it  from  a  central  origin,  as  in  its  original  develop- 
ment in  foetal  life.  If,  however,  the  connective-tissue  cells  recently 
discovered  are  neuroplastic  cells  and  have  the  power  of  producing  new 
nerves,  just  as  cells  of  periosteum  may  produce  a  new  bone,  and  if, 
when  degeneration  ceases,  there  remains  a  sheath  of  Schwann  contain- 
ing a  granular  protoplasmic  mass,  it  is  not  at  all  improbable  that  that 
mass  may  be  differentiated  into  an  axis  cylinder  and  a  medullary  sheath 
and  joined  to  the  old  nerve  fibre  — -  a  process  which  has  its  analogy  in 
the  meduUation  of  nerves  in  the  embryonal  state.  If  we  admit,  with 
Wolberg,  that  in  some  cases  the  axis  cylinder  is  not  destroyed,  the 
formation  of  new  myelin  is  a  rapid  matter.  That  some  such  process 
as  the  one  described  by  Bowlby  and  Stewart  must  occur  in  many  cases 
is  certain,  when  the  rapid  recovery  after  minor  injuries  is  considered  and 
when  the  results  of  nerve  suture  are  taken  into  account,  for  in  both 
these  conditions  the  return  of  function  occurs  long  before  a  new  nerve 
fil)re  starting  out  from  the  old  one  could  have  reached  the  periphery. 

It  is  affirmed  by  Mayer  that  individual  nerve  fibres  in  normal  nerves 
are  constantly  undergoing  these  processes  of  degeneration  and  regenera- 
tion, either  because  the  necessary  renewal  of  worn-out  tissue  takes 

^  Injuries  of  Nerves,  page  25. 


166  INJUBIES  OF  NEBrES.     NEUBITIS. 

place  in  this  manner,  or  beeause  slight  injuries  from  pressure  or  over- 
strain are  sufficient  to  start  up  degeneration  in  single  fibres.  Such 
changes  are  more  apparent  in  old  age  than  in  youth,  and  in  cachectic 
conditions  than  in  healthy  states.  In  all  persons  dying  of  infectious 
diseases  they  can  be  found  well  marked. 

The  process  of  degeneration  in  the  nerves  consequent  upon  the 
destruction  of  the  ganglion  cells  from  which  they  arise  —  the  so-called 
Wallerian  degeneration  —  which  is  best  seen  in  cases  of  anterior  polio- 
myelitis, diifers  in  no  respect  from  that  ensuing  upon  compression  or 
division. 

NEURITIS. 

A  nerve  may  be  inflamed  in  a  short  portion  of  its  course  ^ —  localized 
neuritis ;  or  it  may  be  aflPected  at  many  different  parts  —  disseminated 
neuritis ;  or  it  may  be  diseased  in  its  entire  length  —  general  neuritis. 
The  process  may  be  described  as  ascending  or  descending,  according  to 
the  direction  in  which  the  disease  makes  progress.  Even  when  the 
lesion  is  a  strictly  local  one,  limited  to  a  short  portion  of  a  nerve, 
extensive  secondary  changes  occur  from  the  part  aifected  outward  ;  and, 
as  these  may  involve  the  entire  length  of  the  nerve,  regeneration  and 
repair  may  require  a  much  longer  time  than  is  taken  by  the  healing  of 
the  original  lesion.  General  constitutional  states  may  produce  a  simul- 
taneous neuritis  in  many  nerves  —  multiple  neuritis  —  and  as  this  con- 
dition develops  usually  in  the  distal  parts  of  the  nerves,  it  is  often 
termed  peripheral  neuritis.  Neuritis  may  also  occur  secondarily  to 
inflammatory  changes  in  other  parts,  as  with  periostitis  or  abscesses. 
Syphilitic  deposits  in  the  nerves,  tubercles  in  the  nerves,  cancer  or 
other  neoplasms  along  the  nerves  may  cause  a  proliferation  of  the  con- 
nective-tissue elements  or  a  true  diffuse  inflammation. 

After  wounds  and  injuries  of  the  nerves  a  condition  has  occasionally 
been  seen  which  is  known  as  ascending  neuritis  or  migratory  neuritis. 
Tender  spots  along  the  course  of  the  nerve  above  the  point  of  injury 
and  pain  in  the  course  of  the  nerve  as  high  as  its  root  in  the  plexus 
have  been  observed  in  a  few  cases.  This  has  been  chiefly  in  patients 
in  whom  there  has  been  an  open  wound  at  the  point  of  primary  injury 
and  where  there  has  been  a  suspicion  of  an  ascending  septic  process  in 
the  nerve.  The  neuritis,  however,  is  not  always  a  continuous  one  from 
the  point  of  injury  upward,  but  in  a  few  cases  tender  spots  have  been 
found  at  some  distance  above,  without  any  change  in  the  intermediate 
space.  Hence  the  term  migratory  neuritis.  The  migratory  form  is 
supposed  to  be  characteristic  of  septic  infection,  the  sepsis  extending 
along  the  connective-tissue  sheaths  of  the  nerve.  When  there  is  no 
open  wound  to  account  for  this  sepsis,  a  septic  condition  of  internal 
origin,  associated  with  obliteration  or  plugging  of  the  bloodvessels  and 
with  the  production  of  a  gangrenous  area,  has  been  recorded  as  a  cause. 
While  cases  of  ascending  neuritis  in  the  continuity  of  the  nerve  have 
been  produced  experimentally,  it  has  not  been  possible  to  produce 
migratory  neuritis  when  the  wound  has  been  kept  aseptic.     In  some 


NEUBITIS.  167 

cases  of  traumatic  neuritis  a  very  extreme  condition  of  tenderness  of 
the  limb  aboye  the  point  of  injury,  together  with  inability  to  move  the 
joints  and  a  general  hypersensitive  state  with  pain  in  the  joints,  occa- 
sionally develops.  This  condition,  however,  must  be  considered  as 
probably  hysterical,  and  usually  develops  only  in  hypersensitive  or 
neurasthenic  individuals.  It  is  not  necessarily  attended  with  a  distinct 
localizable  tenderness  along  the  course  of  the  nerve,  though  this  may 
also  be  present.  The  number  of  cases  of  ascending  neuritis  recorded 
in  the  literature  of  the  past  few  years  is  very  few.  I  have  never  seen 
a  case  of  true  ascending  neuritis,  though  I  have  seen  many  cases  of 
painful  aifection  of  the  extremities  above  the  level  of  the  neuritis  that 
were  hysterical  in  their  nature.  There  are  some  cases  which  are  not 
hysterical.  In  these  the  explanation  of  the  condition  is  found  in  the 
fact,  well  known  to  psychologists,  that  mild  irritation  long  continued 
produces  in  a  nerve  centre  a  hypersensitive  state  by  what  is  known  as 
a  summation  of  impulses.  In  this  state  slight  impressions  are  believed 
to  be  intense  and  local  impressions  become  generalized  and  widely 
referred.  This  state  may  be  induced  by  neuritis  long  continued  and 
has  been  mistaken  for  an  ascending  neuritis. 

Pathology. — A  nerve  which  is  inflamed  is  red  and  swollen,  is  lack- 
ing in  its  natural  surface  lustre,  and  is  no  longer  firm  and  smooth  to 
the  touch.  Its  vessels  are  congested  and  there  may  be  hemorrhages 
within  its  sheath.  If  the  process  has  been  in  progress  for  some  time 
there  may  be  bulbous  swellings  on  the  nerve  the  result  of  connective- 
tissue  infiltration,  or  the  nerve  may  be  markedly  atrophied.  Such 
bulbous  thickenings  are  very  common  after  injuries  and  always  occur 
after  a  division  of  a  nerve  on  the  central  end.  A  section  of  the  nerve 
viewed  by  the  microscope  shows  a  distention  of  the  vessels,  an  infiltra- 
tion of  endoneurium  with  small  cells,  and  a  thickening  of  the  connec- 
tive-tissue elements. 

The  microscopic  changes  are  similar  to  those  produced  by  injuries  of 
the  nerves.  Yet  it  is  possible  to  distinguish  between  cases  in  which 
the  lesions  are  chiefly  limited  to  the  ax  one  and  medullary  sheath  —  the 
parenchymatous  form  of  neuritis,  and  cases  in  which  the  lesion  affects 
more  especially  the  connective  tissue  about  the  fine  fibres  and  the  endo- 
neurium and  perineurium  —  the  interstitial  form  of  neuritis.  In  the 
latter  the  bloodvessels  and  lymphatics  take  a  more  active  part  in  the 
inflammatory  changes. 

In  parenchymatous  neuritis  at  the  outset  the  myelin  sheath  appears 
slightly  swollen,  is  less  homogeneous,  and,  from  a  difference  of  refrac- 
tive power,  is  less  translucent.  It  then  becomes  split  up  into  segments 
of  different  length  and  form,  the  segmentation  occurring  preferably  at 
the  incisures  of  Schmitt,  while  the  incisures  at  other  parts  disappear. 
Between  these  segments  of  myelin  a  finely  granular  protoplasm  is  seen  in 
which  new  nuclei  are  found.  In  some  fibres  the  axis  cylinder  may  still 
be  preserved.     In  others  it  is  broken  at  the  same  places  as  the  myelin. 

At  the  next  stage  of  the  process  the  changes  are  more  marked.  The 
myelin  is  now  reduced  to  a  series  of  small  globules  surrounded  every- 


168  INJUBIES  OF  NEBVES.     NEUBITIS. 

where  by  granular  jirotoplasm,  and  in  this  protoplasm  the  nuclei  are 
now  very  numerous.  The  axone  cannot  be  distinguished  in  the  mass, 
as  a  rule,  but  occasionally  a  fine  line  is  seen  passing  through  the  mass, 
which  may  be  a  remaining  axone.     (See  Fig.  86.) 

The  succeeding  stage  presents  a  different  picture.  While  up  to  this 
time  the  size  of  the  nerve  fibre  has  remained  about  normal  and  uni- 
form, it  is  now  seen  to  vary.  At  places  the  fibre  is  still  wide  and 
filled  with  a  granular  mass ;  at  other  places  it  is  narrow,  the  mass 
having  disappeared,  leaving  either  a  collapsed  sheath  or  a  sheath  con- 
taining only  nuclei  here  and  there.  In  a  few  such  narrow  fibres  there 
seems  to  be  an  axone  lying  directly  within  the  sheath  of  Schwann,  and 
occasionally  separated  from  it  at  various  places  by  nuclei ;  but,  as  a 
rule,  no  trace  of  the  axone  remains.  As  any  single  fibre  may  show 
constrictions  at  some  places,  dilatations  at  others,  the  variation  in  its 
calibre  is  the  most  striking  feature  of  this  stage.  In  the  terminal 
stage  the  calibre  is  uniform  again,  but  is  now  everywhere  reduced. 
The  sheath  of  Schwann  is  empty  or  contains  only  a  little  granular 
substance  and  the  nuclei  are  now  less  numerous  than  before.  There 
is,  in  fact,  only  an  atrophied  tube  with  none  of  its  original  contents. 
These  tubes  lying  side  by  side  are  folded  and  undulating,  and  appear 
like  a  strand  of  connective  tissue. 

These  various  stages  of  parenchymatous  inflammation  are  to  be  seen 
in  different  fibres  in  the  same  specimen.  They  are  present  not  only 
at  the  seat  of  inflammation,  but  they  are  present  from  this  point  onward 
to  the  end  of  the  nerve,  constituting  the  change  known  as  secondary 
degeneration.  Their  appearance  is  identical  with  that  observed  in  the 
course  of  degeneration  of  a  nerve  after  compression  or  after  destruc- 
tion of  the  neurone  body  in  the  spinal  cord,  as  in  anterior  poliomye- 
litis. This  had  led  such  an  accurate  observer  as  Erb  ^  to  advance  the 
hypothesis  that  in  cases  of  multiple  neuritis  due  to  toxic  agents  some 
slight  changes  in  cells  in  the  spinal  cord,  not  visible  to  the  microscope, 
are  present  primarily,  and  that  the  changes  in  the  nerves  are  secondary, 
the  nutrient  power  of  the  cell  being  incapable  of  supporting  the  entire 
axone,  which  thus  shows  changes  in  its  most  distal  part.  It  has  lately 
been  shown  by  means  of  the  Nissl  stain  that  after  any  nerve  lesion  in 
the  periphery  a  change  occurs  in  the  entire  length  of  the  axone  and 
also  in  the  central  cell  body,  of  which  that  nerve  is  the  axone.  (See 
Plate  II.,  G.)  This  change  is  a  degeneration ;  but  at  the  end  of  a 
month  it  becomes  stationary,  and  then  gradually  the  cell  body  regains 
its  original  appearance,  even  though  the  axone  may  remain  degener- 
ated. Such  cell  change,  therefore,  is  not  necessarily  the  primary  con- 
dition in  neuritis.  Striimpell  urges  that  parenchymatous  neuritis  has 
its  parallel  in  other  parenchymatous  inflammations,  and  therefore  does 
not  need  to  be  traced  to  any  primary  affection  in  the  cells ;  and  hence 
Erb's  hypothesis  has  not  met  with  general  acceptation. 

Interstitial  neuritis  presents  a  diflPerent  appearance.  In  this  condi- 
tion inspection  shows  the  nerve  to  be  congested,  swollen,  thicker  than 

^Neurol.  Centralbl.,  1883,  p.  481. 


NEURITIS.  169 

normal,  and  lacking  in  lustre,  or  to  be  yellow  and  irregularly  swollen 
by  the  accupaulation  of  pus  and  serum,  or  to  be  reduced  to  a  mere  con- 
nective-tissue strand.  Upon  teasing  the  nerve  it  is  at  once  evident 
from  its  brittleness,  that  individual  fibres  are  lacking  in  continuity  and 
are  changed  in  structure ;  and  if  it  is  examined  under  the  microscope 
the  exudation  of  serum  and  of  inflammatory  corpuscles,  the  great  in- 
crease in  the  number  of  connective-tissue  nuclei,  the  distended  condi- 
tion of  the  vessels,  as  well  as  the  various  appearances  characteristic  of 
nerve  degeneration,  are  clearly  seen.  The  thickening  and  hyperplasia 
of  endoneurium  and  perineurimn  are  well  marked  in  the  older  cases. 
Here  the  inflammation  is  originally  an  interstitial  inflammation,  though 
later  it  becomes  a  diffuse  one.  It  is  possible  that  the  degenerative 
processes  in  the  nerves  may  have  been  due  to  the  compression  by  the 
products  of  inflammation  exuded  within  the  nerve  siieath.  In  one  or 
two  cases  where  the  patient  died  early  in  the  disease  the  nerve  fibres 
which  lay  near  the  vessels  were  affected  to  a  greater  degree  than  those 
lying  deeper,  and  from  this  fact  it  was  concluded  that  their  degenera- 
tion was  secondary.  In  other  cases,  however,  all  the  fibres  in  a  bundle 
were  equally  involved.  It  is  probable  that  in  some  cases  the  inflam- 
mation is  diffuse  from  the  outset,  parenchyma  and  interstitial  tissue 
being  affected  simultaneously.  One  marked  feature  in  these  cases  is 
the  large  amount  of  fatty  deposit  found  in  the  altered  nerves,  as  shown 
by  the  Marchi  stain.  This  is  to  be  ascribed  to  the  fact  that  myelin  in 
undergoing  degeneration  breaks  down  into  globules  and  undergoes  a 
fatty  change.  It  is  itself  allied  to  fat,  and  making  up,  as  it  does,  a 
large  part  of  the  bulk  of  the  fibre,  it  would  be  noticeable  in  the  product 
of  degeneration  if  it  were  not  absorbed.  Sometimes  the  absorption 
seems  to  be  interfered  with  by  the  vascular  condition,  and  hence  the 
residual  amount  of  fat  is  increased.     (See  Fig.  85.) 

One  additional  pathological  form  must  be  mentioned,  since  it  has 
been  described  by  such  a  careful  observer  as  Gombault.^  It  is  the  so- 
called  segmental  periaxillary  neuritis.  In  toxic  neuritis  from  lead  and 
alcohol  poisoning,  Gombault  found  that  the  degenerative  process  was 
not  uniform  in  the  entire  length  of  the  nerve  fibre.  On  the  contrary, 
entirely  normal  segments  alternated  with  the  degenerated  segments  in 
the  nerve.  Mayer  has  noticed  a  somewhat  similar  condition,  and  it 
has  been  described  as  occurring  in  senility.  The  changes  already  de- 
scribed take  place  in  the  myelin  sheath  of  the  affected  segment,  even 
to  its  entire  absorption,  leaving  the  axis  cylinder  in  the  sheath  of 
Schwann  ;  but,  as  the  adjacent  segments  are  not  involved,  regeneration 
is  thought  to  be  more  easily  accomplished.  Pitres  and  Vaillard  ^  have 
noticed  a  somewhat  similar  condition  in  the  neuritis  occurring  after 
diphtheria,  although  in  their  case  the  axis  cylinder  as  well  as  the 
myelin  sheath  was  totally  destroyed  in  many  segments. 

Occasionally  simple  atrophy  of  nerve  fibre  has  been  observed  ;  a  mere 
gradual  reduction  of  all  the  elements  without  any  degenerative  process. 

'  Archives  de  Phys.,  1873,  p.  692  ;  also  Arch,  de  Neurol.,  I.,  1. 
^De  la  n^vrite  segmentaire,  Arch,  do  Neurol.,  xi.,  337. 


170  INJUEIES   OF  NEBVES.     NEVEITIS. 

While  it  is,  of  course,  impossible  to  describe  definitely  the  process 
of  regeneration  that  goes  on  after  neuritis,  there  is  no  reason  to  sup- 
pose that  it  differs  in  any  way  from  that  observed  in  experimental 
lesions.  The  time  required  for  the  completion  of  the  process  will  de- 
pend upon  the  severity  and  extent  of  the  degeneration.  When  that  is 
slight  the  recovery  may  be  rapid,  cases  having  been  reported  where  a 
total  restoration  of  function  took  place  in  two  months.  As  a  rule, 
however,  it  is  a  slow  process.  The  large  majority  of  the  cases  require 
over  six  months  for  the  complete  regeneration,  and  in  not  a  few  cases 
from  ten  to  sixteen  months  elapse  before  the  condition  of  the  nerves  is 
proved  to  be  normal  by  the  total  disappearance  of  all  symptoms. 

Symptoms.  —  The  symptoms  of  neuritis  or  of  injuries  of  the  nerves 
have  been  described  on  pages  39  to  46.  The  special  symptoms  de- 
pending on  the  nerve  which  is  affected  are  considered  separately  in  the 
next  chapter. 

Prognosis.  —  The  prognosis  in  neuritis,  as  a  rule,  is  a  good  one. 
We  have  seen  that  there  is  a  spontaneous  tendency  to  regeneration  in 
a  nerve  that  is  injured  or  that  has  been  affected  by  inflammation,  and 
while  this  progress  toward  recovery  is  usually  slow,  yet  eventually  it 
becomes  complete  and  all  the  functions  of  the  nerve  are  restored.  The 
only  factor  in  preventing  a  recovery  is  the  impossibility  of  a  union  be- 
tween the  severed  ends  of  the  nerve,  or  the  interposition  of  callus,  or 
the  development  of  a  connective-tissue  scar  which  prevents  a  reestab- 
lishmeut  of  continuity ;  but,  after  such  obstructions  are  remedied  by 
surgical  treatment,  regeneration  takes  place,  even  though  the  obstruc- 
tion may  have  persisted  for  many  months.  Therefore,  the  eventual 
prognosis  of  recovery  in  neuritis  is  good.  Spontaneous  recovery,  un- 
less the  nerve  is  put  in  a  normal  condition  by  being  freed  from  scar 
tissue,  may  not  occur.  I  have  seen  a  man,  aged  fifty-two  years,  who 
had  suffered  since  the  age  of  six  years  from  paralysis  and  atrophy  of 
the  muscles  controlled  by  the  ulnar  nerve.  At  that  time  his  elbow 
was  fractured  and  dislocated  and  the  nerve  displaced,  so  that  it  passed 
over  the  olecranon,  where  it  could  be  felt.  Until  the  age  of  fifty  years 
he  suffered  from  partial  paralysis  and  ansesthesia,  and  then,  from  un- 
known cause,  his  symptoms,  pain,  numbness,  and  atrophy  increased 
rapidly,  and  he  suffered  much.  Complete  recovery  of  power  and  sen- 
sation followed  an  operation  that  repaired  the  nerve,  though  so  many 
years  had  elapsed  since  the  injury. 

Bruns  ^  has  recently  called  attention  to  the  ultimate  results  in  injuries 
of  the  nerves  and  of  the  plexuses,  and  has  shown  that  while  two-thirds 
of  his  cases  of  nerve  injury  recovered,  only  about  one-quarter  of  the 
cases  of  injuries  of  the  plexuses  were  cured. ^ 

Treatment.  —  In  the  treatment  of  injuries  and  wounds  of  nerves  or 
in  spontaneous  neuritis  the  first  and  most  important  object  is  to  secure 
the  possibility  of  regeneration  by  establishing  the  continuity  of  the  in- 
jured nerve.     If  the  injury  is  from  a  stab  wound  it  is  important  that 

'L.  Bruns.     Keurol.  Cent.,  November,  1902. 
"Kennedy.     Brit.  Med.  Joui-.,  February,  1903. 


NEURITIS.  171 

the  severed  ends  of  the  nerve  should  be  brought  together  and  carefully 
united,  or  if  laceration ^has  taken  place,  so  that  there  is  a  loss  of  con- 
tinuity, a  flap  can  be  made  from  both  ends  and  these  elongated  nerves 
then  united ;  or  it  is  possible  to  insert  the  ends  within  a  tube  of  decal- 
cified bone  to  serve  to  direct  the  regenerating  fibres  outward  toward 
the  distal  end.     Even  those  who  teach  that  regeneration  occurs  in  the 
severed  end  admit  that  it  is  only  rapid  and  perfect  when  such  union  is 
established.     If  the  nerve  has  been  lacerated  by  a  fracture  of  bone  or 
by  a  strain  it  is  usually  sufficient  to  set  the  fracture  or  to  place  the 
strained  part  in  splints,  thus  securing  its  immobility  for  a  time  and 
allowing  nature  to  effect  the  proper  repair.     Occasionally,  however, 
when  the  fracture  has  united,  or  when  the  local  swelling  and  pain  of 
the  strain  have  gone  down  and  the  bony  thickening  or  the  induration 
about  the  strained  part  has  disappeared,  the  nerve  will  still  be  found 
to  have  lost  its  function.     If  after  six  weeks'  treatment  by  electricity 
and  massage  it  is  still  evident  that  there  is  no  progress  toward  recovery, 
it  is  advisable  to  expose  the  point  at  which  the  nerve  was  injured.    It 
is  sometimes  found  that  regeneration  has  been  prevented  by  the  growth 
of  a  connective-tissue  mass,  and  occasionally  it  is  found  that  a  mass  of 
bony  callus  has  formed  about  the  nerve  in  such  a  way  as  to  keep  up 
pressure.     Thus,  in  a  case  of  ulnar  neuritis  under  my  observation,  pro- 
duced by  fracture  at  the  elbow,  the  repair  of  the  fracture  and  the 
restoration  of  motion  in  the  joint  were  not  attended  by  a  recovery  from 
the  neuritis.     It  was  evident,  by  palpation,  that  a  bony  callus  still 
existed  about  the  nerve  at  the  elbow,  and  surgical  exploration  showed 
the  nerve  to  be  completely  embedded  in  this  mass.     When  this  was 
chiselled  away  and  a  groove  formed  in  which  the  nerve  could  lie  freely, 
and  the  two  ends  were  approximated  by  a  flap,  regeneration  and  re- 
covery ensued.     In  a  case  of  an  infant  in  whom  brachial    neuritis 
from  pressure  above  the  clavicle  during  delivery  had  occurred,  and  a 
permanent  paralysis  of  the  arm  had  remained  for  seven  years,  surgical 
exploration  discovered  a  mass  of  scar  tissue  lying  upon  the  nerve  trunk 
and  about  twice  its  diameter,  which  had  prevented  regeneration,  and 
when  this  was  removed  and  the  ends  of  the  nerve  approximated  re- 
covery ensued.     It  is  evident,  therefore,  that  the  first  requisite  in  the 
treatment  of  injuries  of  nerves  is  to  restore,  if  possible,  the  original 
position  of  the  nerve,  so  that  nature  may  produce  regeneration. 

In  cases  of  spontaneous  neuritis  from  cold,  etc.,  the  only  thing  nec- 
essary is  to  maintain  the  parts  in  a  perfectly  quiet  position,  and,  if 
possible,  to  reduce  any  congestion  in  the  inflamed  nerve.  This  may  be 
done  by  the  use  of  counter-irritants,  of  which  the  actual  cautery  is 
probably  the  best.  Light  touching  with  a  Paquelin  cautery  along  the 
course  of  the  inflamed  nerve  will  often  give  relief  to  intense  pain  and 
will  reduce  the  congestion  which  attends  spontaneous  neuritis.  Small 
mustard  plasters  are  also  of  service. 

liocal  applications  of  heat  are  often  very  grateful  in  painful  condi- 
tions of  neuritis ;  in  fac!t  are  much  more  agrceal)le  to  the  patient  than 
cold,  ]icn(;e  poultices  or  packing  the  affected  liml>  in  cotton-wool  cov- 


172  1NJVMIE8  OF  NE:KVES.     NEVmTIS. 

ered  with  oiled  silk  may  be  of  service.  In  the  more  severe  cases, 
where  pain  is  frequently  intense  and  keeps  the  patient  awake  at  night, 
it  is  necessary  to  use  medicines.  The  newer  analgesics  seem  to  have 
the  power  of  diminishing  painful  sensations  in  peripheral  nerves,  and 
hence  pheuacetin,  5  grains;  antipyrine,  10  grains;  acetanilid,  3  grains; 
exalgin,  3  grains,  or  salophen,  10  grains,  may  be  used  every  two  or 
three  hours.  It  is  sometimes  possible  to  obtain  a  better  action  by  a 
combination  of  these,  to  which  a  small  amount  of  codeine  may  be 
added  if  the  pain  is  very  persistent.  The  following  formula  is  a 
favorite  and  very  efficacious  : 


-Phenacetin 

gr. 

vj 

Acetanilid 

gr. 

iij 

Codeine 

gr. 

i- 

Caffeine 

gr. 

ij- 

Sacch.  lactis 

gr. 

V. 

Triturate.     S 

ig. — One  s 

uch  p 

owder  every  three  hours. 

M. 

Codeine  is  a  useful  remedy,  but  in  many  cases  it  is  necessary  to  re- 
sort to  hypodermic  injections  of  morphine.  These  may  be  used  freely 
and  with  less  danger  of  producing  a  habit  in  neuritis  than  in  any  other 
affection,  inasmuch  as  the  gradual  recovery  will  insure  a  diminution 
of  the  pain  and  the  eventual  cessation  of  the  use  of  the  drug.  The 
tenderness  of  the  nerve  to  any  manipulation  and  the  production  of 
pain  by  any  movement  are  sufficient  to  induce  the  patient  to  keep  the 
part  in  perfect  rest,  which  is  essential  to  recovery.  The  anjesthesia  in 
the  skin  within  the  domain  of  the  affected  nerve  can  be  speedily  re- 
duced and  in  many  cases  entirely  removed  by  the  application  of  the 
faradic  brush.  One  pole  of  the  battery  should  be  placed  high  up 
upon  the  limb  that  is  affected,  or  upon  the  trunk,  and  the  brush  should 
be  applied  in  the  anaesthetic  area.  The  strokes  should  be  from  the 
adjacent  sensitive  skin  into  the  ansesthetic  region,  and  the  strength 
of  the  battery  should  be  moderate,  yet  just  sufficient  to  produce  a  dis- 
tinct sensation  of  the  electric  current.  In  this  way  it  seems  as  if  it 
were  possible  to  produce  an  extension  of  sensibility  from  the  sensitive 
area  into  the  adjacent  insensitive  region,  and  thus  to  open  up  anasto- 
motic paths  through  the  skin. 

The  paralysis  following  a  nerve  injury  or  neuritis  requires  electrical 
treatment.  In  the  majority  of  such  cases  the  faradic  contractility  in 
the  muscle  is  lost  and  it  is  useless  to  apply  faradism.  The  galvanic 
contractility,  however,  is  always  preserved,  even  though  it  may  be 
diminished.  For,  by  a  strong  galvanic  current,  even  in  the  worst 
cases,  a  fair  contraction  can  be  produced  in  the  paralyzed  muscle  when 
the  current  applied  to  the  muscle  is  interrupted  by  means  of  the  inter- 
ruj)ting  electrode  held  in  the  hand.  The  pole  to  be  used  over  the 
muscle  should  be  that  to  which  the  muscle  responds  most  quickly. 
If  a  complete  reaction  of  degeneration  is  present  the  positive  closure 
contractility  will  be  greater,  and  then  the  positive  pole  should  be 
placed  upon  the  muscle.  If  a  partial  reaction  of  degeneration  is  pres- 
ent the  negative  closure  contractility  will  be  greater,  and  then  the 


NEUBITIS.  .  173 

negative  pole  should  be  used.  The  other  pole  is  to  be  applied  to  the 
limb  higher  up  in  the  jcourse  of  the  nerve.  The  application  should  be 
made  daily  and  should  last  about  five  minutes  to  each  muscle.  The 
object  of  such  applications  is  to  exercise  the  paralyzed  muscle.  It  is 
not  probable  that  the  effect  of  the  electricity  is  in  any  way  to  increase 
the  repair  of  the  nerve  or  to  aid  its  regeneration,  hence  there  is  no 
object  in  giving  a  continuous  current  to  the  nerve,  as  some  authors 
advise.  The  electrodes  should  be  of  sponge  and  covered  with  clean 
gauze,  and  wet  with  warm  salt  water. 

The  paralysis  may  be  also  aided  by  massage  and  careful  manipula- 
tion of  the  joint  which  the  paralyzed  muscles  should  move.  In  cases 
of  spontaneous  neuritis  where  the  nerve  is  very  tender,  massage  may 
be  so  painful  as  to  be  injurious,  and  it  should  always  be  remembered 
that  massage,  to  be  useful,  should  be  painless.  I  have  seen  severe 
injuries  of  nerves  follow  the  painful  manipulation  of  unintelligent 
masseurs  and  osteopaths,  and  the  statement  which  such  individuals 
often  make,  that  if  their  manipulations  cause  pain  they  are  thereby 
doing  good,  is  absolutely  false.  The  object  of  massage  in  paralysis  is 
to  increase  the  nutrition  and  circulation  in  the  paralyzed  muscles.  It 
should  be  done  gently  for  about  one-half  hour  daily,  and  should  be 
followed  by  a  sense  of  warmth  and  comfort,  and  not  by  fatigue. 

A  secondary  result  of  the  paralysis  of  certain  muscles  is  the  contrac- 
ture of  their  healthy  opponents,  producing  forced  positions  in  a  joint 
and  very  often  quite  serious  deformities.  The  joint  may  even  become 
stiff  or  the  bones  may  become  displaced,.  Thus  in  wrist-drop  which 
follows  musculospiral  palsy  the  intrinsic  bones  of  the  hand  are  not 
uncommonly  displaced,  so  that  a  distinct  protuberance  upon  the  back 
of  the  hand  becomes  visible.  Manipulations  of  such  displaced  or  stiff- 
ened joints  are  very  valuable,  and  should  be  urged  from  the  very 
beginning,  in  order  to  prevent  deformity  ©r  stiffness. 

The  trophic  symptoms  which  occur  in  neuritis  usually  yield  at  once 
to  a  careful  disinfection  of  the  skin  and  protection  of  the  skin  from 
extraneous  injury  or  infection.  This  can  be  attained  by  washing  the 
parts  in  a  solution  of  bichloride  or  of  carbolic  acid,  applying  carbolized 
vaseline  to  the  surface,  and  wrapping  in  absorbent  cotton.  It  is  very 
important  to  prevent  the  occurrence  of  these  trophic  symptoms  by 
proper  care  of  the  skin  from  the  very  beginning,  and  patients  suffering 
from  neuritis  or  having  any  ansesthetic  regions  should  be  warned 
against  the  possibility  of  unnoticed  injuries  or  burns. 


CHAPTER  VII. 

INJUEIES  OF  THE    SPINAL    NERVES  AND  SPECIAL  FOEMS  OF 

NEURITIS. 

The  affections  of  the  cranial  nerves  will  be  considered  in  connection 
with  diseases  of  the  brain  ;  see  Chapter  XXXV. 

There  are  thirty-one  pairs  of  spinal  nerves  which  are  named  after  the 
segments  of  the  spinal  cord  from  which  they  arise,  there  being  eight 
cervical,  twelve  dorsal,  five  lumbar,  five  sacral,  and  one  coccygeal. 
The  origin,  course,  and  branching  of  these  nerves  are  shown  in  Plates 
XIII.  and  XV.  It  is  not  needful  to  describe  the  anatomy  of  these 
nerves  as  their  course  and  destination  is  a  matter  of  general  anatomical 
knowledge  and  is  shown  in  the  plates,  but  it  is  necessary  to  consider 
certain  special  types  of  neuritis  and  the  results  of  injuries  to  certain 
nerves  which  are  frequently  met  with  in  practice. 

CERVICO-BRACHIAL  NEURITIS. 

The  anatomy  of  the  cervico-brachial  plexus  is  shown  in  Plate  XIII. 

Etiology.  —  A  neuritis  of  the  cervico-brachial  plexus  may  occur  in 
the  adult  as  the  result  of  injuries  to  the  neck  and  shoulder,  especially 
after  dislocations,  as  the  result  of  spinal  caries,  or  of  tumors,  or  aneu- 
risms growing  in  the  neck  ;  as  the  result  of  long-continued  pressure  of 
the  plexus  upon  an  abnormal  cervical  rib,  as  in  cases  described  by 
Keen,^  where  full  references  to  -this  subject  are  given  ;  as  a  sequel  of 
any  of  the  infectious  diseases,  especially  grippe,  or  of  toxic  conditions  ; 
from  rheumatic  and  gouty  states,  and  also  from  taking  cold.  It  is 
more  frequently  met  with  in  women,  and  persons  above  the  age  of 
forty  years  are  more  liable  than  young  persons. 

Symptoms.  —  If  the  neuritis  is  limited  to  the  upper  four  cervical 
nerves  a  very  intense  occipital  neuralgia  is  produced.  The  pain  runs 
up  the  back  of  the  neck  and  over  the  head  as  high  as  the  vertex,  and 
is  attended  by  extreme  sensitiveness  of  the  great  occipital  nerve  to 
pressure  ;  by  tingling  and  numbness,  and  by  difficulty  in  the  movement 
of  the  head  because  of  the  pain  produced  by  motion.  The  head  is 
often  held  stiffly  on  this  account.  Occasionally  the  hair  of  the  scalp 
comes  out.  There  is  sometimes  a  weakness  or  paralysis  of  the  deep 
cervical  muscles. 

If  the  neuritis  is  limited  to  the  fifth  and  sixth  cervical  nerves  or 
their  branches  in  the  plexus,  pain  is  very  intense  in  the  neck  above  the 
clavicle,  and  is  felt  over  the  shoulder  and  in  the  axilla  and  down  the 
back  of  the  arm,  and  even  into  the  forearm,  and  the  paralysis  affects 
the  deltoid,  biceps,  coracobrachialis  and  supinator  muscles, 

iyV,  W,  Keen,  Amer.  Jour.  Med.  Sci,,  Feb.,  1907, 
174 


Neclc  muscles 


CEEVICO-BBACHIAL    NEUEITIS.  175 

If  the  lower  cervical  nerves  and  the  first  dorsal  nerve  are  the  seat 
of  the  neuritis  the  pain  ^nd  tenderness  are  very  intense  over  the  clav- 
icle, and  the  pain  is  felt  down  the  front  of  the  arm  over  the  entire 
forearm  and  in  the  hand  and  fingers,  and  the  paralysis  aifects  the 
muscles  of  the  forearm  and  hand. 

It  is  to  be  remembered  that  the  spinal  origin  of  the  sympathetic 
nerve  in  the  neck  is  in  the  last  cervical  and  first  dorsal  segments 
of  the  spinal  cord,  and  the  anterior  nerve  roots  coming  from  these 
segments  contain  the  fibres  passing  to  the  cervical  sympathetic. 
These  nerve  fibres  leave  the  main  cervical  nerves  soon  after  their  exit 
from  the  vertebral  foramina  and  ascend,  lying  deeply  beneath  the 
muscles.  In  the  majority  of  cases  of  neuritis  of  the  lower  part  of  the 
brachial  plexus  they  are  not  involved,  but  occasionally,  if  the  inflam- 
mation extends  deeply  through  the  spinal  nerve  roots,  or  if  these  nerve 
roots  are  compressed  by  tumor,  or  are  involved  in  caries  of  the  spine, 
or  are  lacerated  within  the  spinal  canal,  the  symptoms  of  paralysis  of 
the  sympathetic  appear.  These  symptoms  are  a  slight  retraction  of  the 
eyeball  and  narrowing  of  the  palpebral  fissure,  the  eyelids  appearing 
partly  closed  and  the  outer  angle  of  the  eye  being  slightly  drooping. 
The  pupil  is  somewhat  contracted  and  does  not  dilate  fully  when  the 
eye  is  shaded.  There  is  a  slight  pallor  of  the  side  of  the  face  and 
neck,  an  unusual  dryness  of  the  nostril  and  mouth  on  the  side  of  the 
injury,  and  a  diminution  in  the  secretion  of  sweat  on  the  neck,  arm, 
and  chest  of  the  injured  side.  If  the  patient  is  subjected  to  heat  the 
injured  side  does  not  flush  and  perspire  in  the  normal  manner. 

The  existence  of  these  symptoms  of  injury  of  the  sympathetic,  to 
which  attention  was  first  directed  by  Klumpke,  is  sometimes  of  much 
importance  in  diagnosis,  as  an  indication  that  an  injury  of  the 
plexus  is  present.  Thus  in  a  patient  seen  with  Hartley  at  the  New 
York  Hospital,  who  had  fallen  several  stories  and  sustained  a  fracture 
of  the  right  side  of  the  cranium  and  a  manifest  strain  of  the  left  arm, 
it  was  important  to  determine  whether  the  paralysis  of  the  left  arm  was 
due  to  a  laceration  of  the  brachial  plexus  or  to  an  injury  of  the  brain. 
The  existence  of  great  pain  on  pressure  over  the  brachial  plexus  and 
on  motion  of  the  arm,  with  almost  total  anaesthesia  of  the  hand  and 
arm  below  the  elbow,  and  the  presence  of  the  symptoms  indicative  of 
injury  of  the  sympathetic  nerve  in  the  neck,  made  it  evident  that  the 
paralysis  of  the  arm  was  due  to  a  laceration  of  the  nerves  of  the 
brachial  plexus  and  not  to  a  cerebral  injury.  The  development  of  the 
reaction  of  degeneration  in  the  muscles  in  the  course  of  ten  days  con- 
firmed this  diagnosis.  Usually  in  brachial  neuritis  several  cords  of 
the  plexus  are  involved  simultaneously,  and  then  the  pain  radiates 
over  all  the  branches  of  the  nerves  which  arise  below  this  plexus. 
Plate  XIII.  shows  the  formation  of  this  plexus  from  the  nerve  roots 
and  the  distribution  of  the  cords  of  the  plexus  to  the  various  nerves  of 
the  arm.  Plate  XIV.  demonstrates  the  distribution  of  the  cutaneous 
nerves  in  the  skin  of  the  arm. 

A  comparison  of  the  symptoms  developing  in  any  case,  namely,  thQ 


176  INJUBIES   OF  SPINAL  NEBVES  AND  NEUBITIS. 

distribution  of  the  pain,  of  the  lines  of  tenderness,  of  the  areas  of  ting- 
ling, numbness,  and  anaesthesia,  with  these  diagrams  will  serve  to 
indicate  what  branches  of  the  brachial  plexus  are  involved  in  the  neu- 
ritis. As  cases  differ  widely  from  one  another  in  the  extent  of  impli- 
cation of  the  nerves^  it  is  only  by  such  a  comparison  of  each  case  with 
a  diagram  that  a  definite  diagnosis  can  be  reached. 

Severe  injuries  of  the  brachial  plexus  may  result  in  a  tearing  of  the 
nerve  roots  within  the  spinal  canal,  as  well  as  in  a  direct  injury  to  the 
plexus  itself.  Such  lacerations  of  the  nerve  roots  produce  symptoms 
which  are  difficult  to  differentiate  from  the  symptoms  caused  by  an 
injury  of  the  cords  of  the  plexus.  The  diagnosis  can  sometimes  be 
reached,  however,  if  all  the  muscles  that  are  paralyzed  be  listed,  and 
their  nervous  supply  be  exactly  determined  by  a  comparison  with  a 
diagram  such  as  is  shown  in  Plate  XIII.  In  a  case  recently  under  my 
observation,  a  comparison  of  the  muscles  paralyzed,  with  this  diagram, 
made  it  evident  that  the  fifth  and  sixth  nerve  roots  were  implicated 
rather  than  the  upper  cord  of  the  brachial  plexus,  the  long  posterior 
thoracic  nerve  to  the  serratus  magnus  muscle  being  involved,  which 
would  have  escaped  had  the  cord  alone  been  injured.  In  another  case, 
the  seventh,  eighth  and  first  dorsal  nerve  roots  were  implicated  rather 
than  the  lower  cords  of  the  brachial  plexus.  In  both  these  cases  the 
diagnosis  was  confirmed  at  the  operation,  which  was  undertaken  to 
explore  and  repair  the  plexus  in  case  that  was  found  to  be  injured. 
In  these  cases  the  distribution  of  anaesthesia  in  the  skin  corresponded 
to  the  anaesthesia  which  would  have  been  produced  by  a  lesion  of  the 
segments  of  the  cord,  and  not  to  the  distribution  that  would  have  been 
produced  by  a  lesion  of  either  the  individual  nerves  making  up  the 
plexus,  or  of  the  cords  of  the  plexus.  In  cases  where  the  roots  of  the 
nerves  are  torn,  there  appears  to  be  no  possibility  of  repair,  as  a  union 
is  not  possible  between  the  distal  end,  outside  of  the  vertebra,  and  the 
central,  inside  of  the  vertebra.  These  cases,  therefore,  have  a  bad 
prognosis.^ 

Brachial  neuritis  of  the  ordinary  form  in  the  adult,  due  to  exposure 
to  cold,  is  an  extremely  common  disease,  almost  as  common  as  sciatica. 
It  is  an  exceedingly  painful  affection,  coming  on  sometimes  with  great 
suddenness,  as  in  a  case  described  by  Klumpke  ^  where  the  autopsy 
showed  an  extensive  hemorrhage  in  the  sheath  of  the  nerves.  Hence 
it  has  been  called  "  apoplectic  neuritis."  Its  onset  may  be  gradual 
through  several  days,  under  which  circumstances  an  extreme  conges- 
tion with  interstitial  neuritis  and  secondary  implications  of  the  nerve 
fibres  are  present.  A  pain  in  the  location  of  the  plexus  and  pain  radi- 
ating outward  are  the  most  distressing  symptoms,  and  are  so  intense 
and  agonizing  as  to  deprive  the  patient  not  only  of  all  use  of  the  limb, 
but  also  of  sleep.  Soon  after  the  onset  of  the  pain  any  motion  be- 
comes so  uncomfortable  as  to  make  rest  of  the  limb  imperative.  The 
numbness  and  tingling  which  attend  the  pain  are  extremely  disagree- 
able and  are  more  intense  after  the  disease  is  well  established  at  the 

1  Frailer.     Jour.  Amen  Med.  Assoc,  Dec.  16,  1911.      2  Rev,  de  M^d.,  1885. 


PLATE  XIV 


,4roc'i[ect>- 


Superficial  Perineal  of\ 
Piidic  and  Inferior    I 
Pudendal  of  small     I 
Sciatic 


The  Distribution  of  Sensory  Nerves  in  the  Skin.     (After  Flower.) 

The  areas  of  the  skin  supplied  by  the  cutaneous  nerves  are  shown  in  finely  dotted 
outline.  The  circles  on  the  trunk  show  areas  occasionally  ansesthetic  in  hysteria.  The 
lines  across  the  limbs  at  ankle,  knee  and  thigh,  wrist,  elbow  and  shoulder  show  the  upper 
limits  of  anaesthesia  in  multiple  neuritis  of  varying  degrees  of  severity. 


CEBVICO-BBACHIAL    NEUBITIS.  177 

end  of  a  week.  The  paralysis  of  the  muscles  supplied  by  the  affected 
nerves  develops  within  three  or  four  days  of  the  onset,  and  these 
muscles  becdme  atrophied  as  the  case  goes  on,  and  usually  after  ten 
days  show  a  reaction  of  degeneration.  In  brachial  neuritis  trophic 
disturbances  of  the  skin,  glossy  skin,  herpes,  eruptions  of  various 
kinds,  and  interference  with  the  growth  of  the  nails  are  very  common 
occurrences.  The  skin  is  manifestly  thin,  becomes  very  shiny  and 
smooth,  appears  as  if  tightly  stretched  over  the  fingers,  hand,  or  arm, 
and  is  exceedingly  tender  and  hypersensitive  to  touch,  to  heat,  and  to 
cold.  Sensations  caused  by  moving  the  fine  hair  on  the  skin  are 
peculiarly  disagreeable.  The  limb  is  very  frequently  covered  with 
perspiration,  which  may  be  of  a  fetid  odor.  Such  a  neuritis  usually 
persists  for  several  weeks  or  even  months.  The  average  duration  in 
ten  such  cases  under  my  observation  was  four  months.  The  symptoms 
gradually  subside,  but  the  weakness  often  persists  for  some  weeks  after 
the  pain  has  disappeared.  In  cases  where  the  presence  of  a  cervical 
rib  is  suspected  an  aj-ray  will  demonstrate  its  existence. 

Pathology.  —  Pathological  observations  in  cases  of  neuritis  of  the 
brachial  plexus  are  comparatively  rare,  as  the  patients  usually  recover. 
Klumpke's  case  of  hemorrhage  within  the  sheath  of  the  plexus  has 
already  been  mentioned.  Jacobson  reports  a  case  following  carcinoma 
of  the  breast  of  nine  months'  duration.  A  microscopic  examination 
showed  an  almost  total  disappearance  of  the  nerves  of  the  brachial 
plexus,  though  there  was  no  infiltration  with  carcinomatous  masses. 
The  process  of  degeneration  was  present  in  many  bundles,  with  a 
manifest  disappearance  of  nerve  fibres,  and  an  atrophic  condition  was 
found  in  the  peripheral  nerves  of  the  arm.  The  cells  of  the  posterior 
spinal  ganglia  in  the  cervical  region  showed  degenerative  changes  by 
the  Nissl  method  of  staining,  and  by  the  Marchi  method  there  was 
discovered  a  manifest  degenerative  change  in  the  posterior  root  zone 
of  the  spinal  cord  corresponding  to  the  entrance  of  the  nerve  roots 
from  which  the  brachial  plexus  was  made  up.  Degeneration  ascended 
in  the  column  of  Burdach,  and  also  descended  in  the  comma-shaped 
bundle  of  Schultze  as  far  down  as  the  fifth  dorsal  segment.  This 
observation  proves  that  in  some  cases  when  the  sensory  neurone  is 
involved  in  one  of  its  branches  the  cell  body  and  the  other  branch 
may  also  degenerate. 

Prognosis.  —  The  prognosis  is  fairly  good,  as  recovery  eventually 
occurs  in  the  majority  of  the  cases.  But  the  progress  is  slow  and  is  at- 
tended by  very  great  discomfort.  It  is  to  be  remembered  that  nerves 
in  regenerating  grow  but  one  centimetre  a  week,  and  hence  the  repair 
in  the  affected  nerves  requires  a  long  time,  especially  if  those  nerves 
are  the  long  ones  to  the  fingers.  In  the  traumatic  cases  the  prognosis 
is  less  favorable  than  in  injuries  of  single  nerves.  Thus  in  a  case  seen 
with  McBurney,  a  calcareous  cicatrix  had  involved  the  three  cords  of 
the  plexus  and  destroyed  the  nerves  so  completely  that  nothing  could 
be  done  to  induce  repair. 

Treatment.  —  Treatment  consists  of  absolute  rest^  the  arm  being 
12 


178  INJURIES   OF  SPINAL  NEBVES  AND  NEUBITIS. 

carried  in  a  sling  and  held  to  the  side  by  bandages,  constant  applica- 
tion of  heat,  either  by  packing  the  shoulder  and  arm  with  cotton,  which 
is  to  be  covered  with  oiled  silk,  hot  bottles  being  applied  outside  of  the 
bandage,  or  by  the  application  of  poultices.  In  my  experience  cold 
applications  —  ice  packs,  or  spraying  with  ether,  or  chloride  of  ethyl, 
which  are  recommended  by  the  Germans — produce  great  discomfort 
and  are  voluntarily  discarded  by  the  patients  in  favor  of  hot  applica- 
tions. The  use  of  the  actual  cautery  in  producing  counter-irritation 
over  the  painful  nerve  trunks  is  urged  by  all  authorities,  and  I  have 
certainly  known  it  to  alleviate  the  pain.  It  may  be  done  daily  if  the 
touch  made  is  very  light,  so  as  not  to  break  the  skin,  merely  produc- 
ing a  red  line  upon  it.  Electricity  may  be  used,  and  in  some  patients 
appears  to  give  considerable  relief.  A  very  mild  galvanic  current, 
from  four  to  six  milliamp&res  only,  should  be  applied  with  the  positive 
pole  over  the  painful  region,  and  the  negative  pole  over  the  back  of 
the  neck.  The  current  should  not  be  broken  and  should  be  allowed 
to  pass  for  ten  minutes  through  the  painful  region,  the  pole  being 
shifted  in  order  to  bring  all  the  painful  parts  within  the  influence  of 
the  current.  In  applying  the  electricity  to  the  arm,  Remak  recom- 
mends that  a  current  be  used  diagonally  through  the  painful  nerve, 
the  positive  pole  being  over  the  most  painful  point  and  the  negative 
upon  the  other  side  of  the  limb.  During  the  early  and  painful  stage 
of  the  disease  any  electrical  application  producing  a  contraction  of  the 
muscles  is  too  painful  to  be  endured,  but  later  in  the  course  of  the 
case,  when  the  pain  is  subsiding,  it  is  well  to  exercise  the  muscles  by 
electricity  in  order  to  keep  up  their  tone  until  the  nerve  has  regener- 
ated. The  application  of  electricity  has  no  effect  upon  the  course  of 
the  case  or  upon  the  regeneration  of  the  nerve  trunk. 

Massage  of  the  limb  is  advisable  when  the  parts  are  not  too  tender 
to  prevent  its  use,  and  as  soon  as  the  tenderness  subsides  sufficiently  to 
allow  of  it,  it  should  be  begun  and  continued  until  recovery.  Some 
relief  is  often  obtained  by  douching  the  limb  with  hot  water  from  a 
spray  or  by  alternate  douches  of  hot  and  cold  water.  Patients  differ 
in  their  susceptibility  to  cold  in  this  condition.  Many  patients  are 
hypersensitive  to  cold  in  conditions  of  neuritis,  and  under  these  circum- 
stances cold  water  should  be  avoided. 

The  pain  in  brachial  neuritis  is  so  intense  as  to  require  medical 
relief.  The  various  analgesic  preparations  —  acetanilid,  antipyrine, 
phenacetin,  alone  or  combined  —  should  be  tried  before  codeine  or 
opium  are  resorted  to.  But  in  the  majority  of  cases  in  this  condition 
it  is  necessary  to  use  hypodermic  injections  of  morphine,  and  these 
may  be  used  very  freely  in  sufficient  amount  and  with  sufficient  fre- 
quency to  secure  relief  from  the  intense  agony  which  otherwise  exhausts 
the  patient.  Care  should  be  taken  to  keep  up  the  general  nutrition  of 
the  patient  during  the  course  of  the  disease.  Good  food,  adjuvants  to 
digestion  and  laxatives  which  counteract  the  action  of  the  morphine 
should  be  used  freely,  and  it  is  well  to  insist  upon  the  use  of  fatty 
foods  in  excess,  as  these  appear  to  have  a  beneficial  effect  in  increasing 


OBSTETEICAL   PABALTSIS.  179 

the  rate  of  regeneration  in  the  nerves.  Alcoholic  drinks  should  be 
avoided  or^sed  in  great  moderation.  Strychnine  and  arsenic  are  of 
benefit  in  the  stage  of  recovery,  but  are  not  to  be  used  while  the  pain 
is  intense.  The  glycero-phosphates  of  lime  and  soda  in  1 5-grain  doses, 
three  times  a  day,  are  of  much  use. 

Surgical  treatment  is  demanded  in  traumatic  cases  which  show  no 
tendency  to  recover  after  three  months.  The  plexus  should  be  exposed, 
cicatricial  tissue  removed,  and  a  union  of  the  separated  nerves  secured 
by  a  nerve  flap,  or  by  inserting  the  ends  in  a  tube  of  decalcified  bone. 

OBSTETRICAL    PARALYSIS,    OR   BIRTH    PALSY   OF   DUCHENNE. 

This  form  of  paralysis  is  noticed  within  a  few  hours  or  days  of  the 
birth  of  an  infant.  It  is  usually  limited  to  the  deltoid,  biceps,  brachi- 
alis  anticus,  supraspinatus  and  infraspinatus,  and  supinator  longus 
muscles,  but  occasionally  involves  the  extensor  muscles  of  the  wrist 
and  fingers.  The  arm  hangs  loose  at  the  side  of  the  body,  its  position 
as  a  whole  being  governed  by  gravitation,  with  the  forearm  extended 
and  pronated,  and  the  wrist  and  fingers  flexed.  (See  Fig.  89.)  If  the 
arm  be  lifted  and  then  let  go,  it  falls  into  this  position.  The  muscles 
which  are  paralyzed  are  relaxed  and  their  opponents  are  not  rigid,  so  that 
all  joints  are  freely  movable  and  motion  does  not  give  the  child  pain. 
The  only  movements  in  the  paralyzed  arm  are  those  of  extension  of 
the  forearm  and  motions  of  the  wrist  and  fingers.  Sometimes  when 
the  extensors  of  the  wrist  are  involved  there  is  slight  extension  of  the 
two  distal  phalanges  only.  If  the  paralyzed  muscles  are  examined 
electrically  the  reaction  of  degeneration  can  be  demonstrated,  but  this 
is  often  difficult  in.  an  infant  on  account  of  the  undeveloped  condition 
of  the  muscles  and  the  thick  layers  of  fat.  A  very  strong  current  is 
necessary  to  produce  the  reaction,  and  this  is,  of  course,  attended  by 
pain ;  hence  electrical  examinations  are  difficult  in  infants.  If  such 
tests  are  made  the  normal  arm  should  be  taken  as  a  standard  for  com- 
parison. If  the  sensibility  is  tested  by  a  needle  it  will  usually  be  found 
to  be  considerably  impaired  over  the  area  of  the -arm  corresponding  to 
the  cutaneous  branches  of  the  circumflex  nerve — a  heart-shaped  area 
whose  point  corresponds  to  the  insertion  of  the  deltoid  muscle,  and 
sometimes,  also,  along  the  outer  side  of  the  arm  and  forearm  in  the  dis- 
tribution of  the  musculocutaneous  nerve.  After  this  condition  has 
remained  for  several  weeks  or  months  without  much  change,  as  it 
often  does,  the  muscles  which  are  paralyzed  become  considerably  atro- 
phied and  feel  unduly  soft  and  yielding.  (Fig.  90  )  The  actual  size 
of  the  limb,  however,  may  not  be  very  perceptibly  reduced,  for  the  fat 
in  a  thoroughly  healthy  baby  is  much  greater  than  the  substance  of 
muscular  tissue,  and,  therefore,  there  is  not  the  rapid  wasting  of  the  arm 
and  forearm  which  is  noticed  in  adults  suffering  from  paralysis  of  the 
brachial  plexus.  When  the  condition  has  lasted  three  or  four  months 
there  sometimes  appears  a  slight  stiffiiess  of  the  unparalyzed  muscles, 
so  that  bending  the  forearm  and  wrist  or  opening  the  little  closed  hand 
is  not  as  easy  as  at  the  beginning.     A  permanent  rigidity^  however, 


180 


INJURIES   OF  SPINAL  NEBVES  AND  NEUEITIS. 


rarely  if  ever  develops.  Many  of  these  cases  go  on  to  spontaneous 
recovery,  which  ensues  within  six  months  after  birth.  Others  remain 
longer  and  do  not  recover  within  the  first  year.  The  condition  may 
remain  for  three  or  four  years  and  pass  away  only  when  the  child  is 
old  enough  to  be  taught  systematic  gymnastic  exercises.  I  have  seen 
one  case  where  no  recovery  had  ensued  after  seven  years,  and  then  an 
operation  by  Dowd  demonstrated  the  existence  of  a  cicatricial  mass  at 
the  junction  of  the  roots  of  the  fifth  and  sixth  cervical  nerves  in  the 


Fig.  89. 


Fig.  90. 


Boy,  aged  seven,  whose  right  arm  had  been  par-       Atrophy  of  left  shoulder  and  arm  due  to  birth 
alyzed  since  birth.     The  position  of  the  arm  and    palsy.     (W.  N.  Bullard.) 
hand  and  the  atrophy  are  typical. 

neck,  which  had  prevented  regeneration.  (Fig.  91.)  This  was  too 
extensive  to  warrant  removal.  I  have  seen  one  girl  of  fourteen  who 
still  had  complete  disability  of  movement  at  the  shoulder-joint  and  in- 
ability to  supiuate  the  arm,  due  to  the  occurrence  of  birth  palsy. 

The  course  of  the  disease  and  the  final  condition  depend  upon  the 
severity  of  the  lesion. 

The  lesion  in  all  cases  of  birth  palsy  involves  the  brachial  plexus. 


obsteTbical  pjbalysts. 


181 


When  the  upper  arm  type  of  paralysis  is  present  the  roots  of  the  fifth 
and  sixth  c^vical  neryes  are  involved.  When  the  lower  arm  type  is 
present  the  seventh  and  eighth  cervical  nerves  are  affected.  Duchenne 
showed  that  pressure  backward  upon  the  side  of  the  neck  is  liable  to 
compress  these  two  nerves  against  the  lamina  of  the  sixth  cervical 
vertebra.  Erb  has  shown  that  it  is  possible  by  a  careful  examination 
to  find  a  spot  two  centimetres  above  the  clavicle,  back  of  the  outer 
edge  of  the  sternomastoid  muscle,  corresponding  to  the  point  of 
emergence  of  the  sixth  cervical  nerve  between  the  scaleni,  at  which 
point  irritation  by  the  faradic  current  will  produce  a  contraction  in  the 
deltoid,  biceps,  brachialis  anticus,  and  supinator  longus  muscles ;  and 
if  the  irritation  be  increased  the  extensors  of  the  wrist  will  also  con- 
tract. Pressure  upon  this  particular  region  is  often  made  during 
delivery  either  by  the  clavicle  or  by  forceps  or  by  the  fingers  of  the 
obstetrician.  This  is  more  common  when  there  is  a  breech  presenta- 
tion and  the  after-coming  head  is  extracted  in  the  common  method. 

Fig.  91. 


Thickened  cord  of  brachial  plexus  at  the  junction  of  the  5th  and  6th  cervical  nerves  found  at  operation 
on  boy  shown  in  Fig.  89.     (Natural  size,  from  a  sketch  by  Dowd.) 


The  index  and  middle  fingers  of  the  left  hand  being  open  like  a  fork 
over  the  shoulders  of  the  child,  traction  is  commonly  made  upon  the 
shoulders,  and  the  pressure  of  the  obstetrician's  finger  in  the  neck  often 
produces  injury  of  the  plexus.  In  some  cases  injury  of  the  plexus 
is  produced  by  attempts  to  bring  down  the  hand  or  arm  in  breech 
presentations  or  to  replace  these  when  the  head  presents.  In  these 
cases  it  is  probable  that  the  roots  of  the  nerves  are  torn  or  a  portion  of 
the  upper  cord  of  the  plexus  is  torn.^  Forceps  applications  in  an 
awkward  position  may  also  produce  this  injury.  Children  do  not 
appear  to  be  very  liable  to  injury  of  the  nerves  in  spite  of  the  many 
accidents  which  befall  them.  I  have  not  seen  a  case  of  traumatic 
neuritis  in  the  plexus  of  a  child  below  the  age  of  fourteen  years,  ex- 
cepting the  form  of  brachial  neuritis  just  described. 

Treatment.  —  The  arm  should  be  kept  in  a  sling,  with  the  elbow 
flexed,  and  should  not  be  allowed  to  hang  down,  since  its  weight  often 
suffices  to  overstretch  the  ligaments  of  the  shoulder  which  are  no  longer 
1 W.  N.  Bullard.     Amer.  Jour.  Med.  Sci.,  July,  1907. 


182  INJURIES   OF  SPINAL  NEilVES  AND   NEUEITlS. 

assisted  by  the  action  of  the  deltoid.  The  entire  arm  should  be  rubbed 
daily,  the  mother  being  taught  the  kneading  process  of  massage.  It 
should  be  bathed  freely  night  and  morning  with  hot  and  cold  water, 
so  as  to  increase  the  general  circulation  and  nutrition.  Unintelligent 
people  will  be  more  likely  to  keep  up  the  necessary  rubbing  if  some 
simple  lotion  cr  ointment  be  ordered.  As  soon  as  any  voluntary 
motion  can  be  made  the  child  should  be  encouraged  to  make  it,  even 
if  it  be  necessary  to  bind  the  unaffected  limb  to  the  side  of  the  body. 
As  the  child  grows  older  systematic  exercises  of  a  gymnastic  kind 
should  be  insisted  upon  daily.  Galvanic  treatment  in  accordance  with 
the  regular  methods  described  on  page  172,  should  be  begun  early  and 
used  until  recovery  has  occurred.  The  mildest  current  which  will 
produce  contraction  in  the  muscles  should  be  used,  that  the  treatment 
may  not  be  too  painful,  and  an  intelligent  nurse  or  mother  can  be 
instructed  to  carry  out  this  treatment  at  home.  If  there  is  no  im- 
provement after  two  years  an  exploratory  operation  should  be  under- 
taken. The  upper  part  of  the  brachial  plexus  should  be  exposed,  the 
nerves  should  be  freed  from  cicatricial  tissue  if  possible ;  and  if  not 
possible  the  cicatrix  should  be  cut  out  and  the  ends  of  the  divided 
nerves  be  sutured.  The  shoulder  may  be  elevated  and  the  head  drawn 
over  to  the  side  and  held  in  this  position  by  a  stiif  plaster-of- Paris 
cast  during  the  time  of  union  in  order  to  prevent  traction  upon  the 
united  nerves.  A  number  of  successful  instances  of  this  operation 
have  been  recently  reported.^ 

PARALYSIS  OF  THE  CIRCUMFLEX  NERVE. 

Injuries  of  the  shoulder  and  dislocations  of  the  shoulder  sometimes 
produce  a  paralysis  of  the  circumflex  nerve.  The  symptoms  consist 
of  pain  and  tenderness  in  the  course  of  the  nerve ;  paralysis  and 
atrophy  of  the  deltoid  muscle,  which  prevents  abduction  of  the  arm,  a 
relaxation  of  the  ligaments  of  the  shoulder-joint,  as  the  deltoid  muscle 
keeps  the  humerus  in  apposition  to  the  socket ;  and  an  area  of  anaes- 
thesia, triangular  in  shape,  with  the  apex  of  the  triangle  downward 
corresponding  to  the  insertion  of  the  deltoid.     See  Plate  XIV. 

A  patient  under  my  care  had  been  in  the  habit  of  standing  in 
his  office  and  resting  his  shoulder  against  the  sharp  edge  of  a  mantel. 
About  the  first  of  May  he  began  to  suffer  from  pain  in  the  back  of  the 
neck  and  about  the  shoulder,  and  after  ten  days  his  deltoid  became  sud- 
denly paralyzed.  This  paralysis  remained,  in  spite  of  treatment  by 
electricity  and  massage,  until  the  first  of  November,  though  by  the 
middle  of  July  he  had  recovered  power  enough  to  raise  the  arm  above 
the  head.  The  anaesthesia  over  the  shoulder  disappeared  within  the 
first  month. 

^  See  British  Medical  Journal,  February  3,  1903. 


NEUBITIS    OF    THE    SHOULDEB-JOINT.  183 

PARALYSIS  OF  THE  SUPRASCAPULAR  NERVE. 
Paralysis  juif  the  suprascapular  nerve  occasionally  occurs  after  dis- 
location of  the  humerus,  or  may  be  produced  by  falls  upon  the  shoulder 
or  on  the  hand,  causing  contusion  of  the  shoulder.  The  symptoms 
are  chiefly  referable  to  paralysis  of  the  supraspinatus  and  infraspinatus 
muscles.  I  have  seen  this  following  the  removal  of  deep  cervical 
glands  in  the  neck  by  accidental  division  of  the  nerve  trunk.  '  The 
shoulder  is  very  much  hampered  in  its  movement,  as  is  also  the  arm. 
When  the  arm  is  abducted  and  raised  the  head  of  the  humerus  falls 
from  lack  of  support  by  the  supraspinatus  muscle  ;  hence  the  arm  can- 
not be  held  up.  Movements  of  outward  rotation  are  also  impaired  by 
the  paralysis  of  the  infraspinatus.  The  scapula  is  slightly  rotated,  its 
lower  angle  being  moved  upward  and  inward,  and  its  upper  angle  pro- 
jecting on  the  side  of  the  neck.  The  defective  action  is  somewhat 
supplemented  by  the  deltoid  and  the  teres  minor,  which  muscles  occa- 
sionally become  a  little  hypertrophied  in  the  course  of  the  case.  Some- 
times a  small  region  of  anaesthesia  is  found  over  the  scapula. 

NEURITIS  OF  THE  SHOULDER-JOINT.  PAINFUL  SHOULDER. 

A  condition  of  extreme  pain  and  stiffness  at  the  shoulder-joint,  due 
to  a  neuritis  of  the  nerves  within  the  joint,  is  not  an  uncommon  affec- 
tion. It  occurs,  as  a  rule,  in  women  of  middle  age,  but  men  are  not 
exempt.  It  occurs  in  gouty  and  in  anaemic  and  cachectic  individuals, 
and  is  often  seen  in  old  age.  It  sometimes  appears  to  follow  an  ex- 
posure to  cold,  and  sometimes  it  can  be  traced  to  a  slight  injury.  It 
begins  rather  suddenly  with  pain  in  the  shoulder-joint,  but  without  any 
swelling  or  exudation  within  the  joint  and  without  the  appearances  of 
rheumatism  or  arthritis.  Very  soon  any  movement  becomes  extremely 
painful,  and  as  a  result  the  muscles  about  the  joint  are  thrown  into  a  state 
of  tonic  spasm.  The  arm  is  adducted  tightly  to  the  chest,  and  while 
slight  forward  or  backward  motions  are  possible,  any  attempt  at  ab- 
duction is  followed  by  an  immediate  contraction  in  the  pectorals  and 
latissimus  dorsi.  There  are  tender  points  in  front  and  at  the  back  of 
the  joint,  and  pressure  of  the  head  of  the  humerus  into  its  socket  is 
very  painful.  The  condition  remains  for  many  weeks,  and  as  the 
result  of  the  immobility  the  deltoid  frequently  becomes  atrophied. 
Occasionally  I  have  seen  an  anaesthesia  in  the  circumflex  area  of  the 
skin  and  such  a  rapid  atrophy  of  the  deltoid  as  to  suggest  a  neuritis 
of  the  circumflex  nerve,  but  this  is  not  an  invariable  accompaniment 
of  the  affection.  When  there  are  no  objective  signs  of  neuritis  in  the 
shoulder,  or  of  tenderness  about  the  nerves  in  the  neck,  the  affection 
may  be  thought  to  be  hysterical,  as  neurotic  individuals  appear  to  be 
more  subject  to  it  than  others.  But  the  lack  of  remissions  in  the  dis- 
ease and  the  fact  that  the  spasm  of  the  muscles  does  not  appear  to 
relax  under  chloroform  —  a  fact  that  I  observed  in  a  patient  who  had 
an  operation  for  cancer  of  the  breast  while  this  affection  was  in  prog- 
ress —  leads  me  to  believe  that  the  spasm  is  a  reflex  one  from  the 
nerves  within  the  joint  and  that  the  origin  of  the  trouble  is  a  neuritis 


184  INJURIES   OF   SPINAL  NEBVES  AND  NEURITJS. 

of  these  nerves.  As  any  motion  is  extremely  painful,  patients  guard 
the  arm  by  carrying  it  in  a  sling  and  by  bandaging  it  to  the  chest. 
They  find  hot  applications  and  packing  in  cotton  agreeable,  and  they 
usually  obtain  considerable  relief  from  the  application  of  a  Paquelin 
cautery  to  the  front  and  back  of  the  shoulder.  It  seems  to  have  less 
effect  when  applied  over  the  region  of  the  deltoid  muscle.  The  treat- 
ment is  rest,  cautery,  and  the  use  of  sedatives,  including  codeine,  if 
necessary,  for  the  pain.  When  the  acute  symptoms  are  over,  massage 
of  the  muscles  may  be  attempted,  but  movement  of  the  joint  is  to  be 
avoided,  as  it  is  liable  to  produce  a  return  of  the  spasm  in  the  muscles. 
In  all  cases  that  I  have  seen,  recovery  has  eventually  occurred,  but 
from  four  to  six  months  have  elapsed  before  the  full  use  of  the  arm 
has  been  restored. 

PARALYSIS  OF  THE  POSTERIOR  THORACIC  NERVE. 

Injuries  to  the  side  of  the  neck  sometimes  bruise  the  long  posterior 
thoracic  nerve  which  passes  to  the  serratus  magnus  muscle.  I  have 
seen  two  cases  of  this  kind  occurring  in  porters  who  had  carried  a 
piano  upon  the  shoulder  ;  and  several  cases  from  injuries  to  the  side  of 
the  neck.  I  have  seen  a  case  resulting  from  the  effort  of  swinging  a 
hammer.  Falls  and  blows  upon  the  neck  or  shoulder  which  cause 
extreme  extension  of  the  head  may  also  produce  this.  Pressure  on  the 
shoulder  in  a  woman  placed  in  the  Trendelenberg  position  on  the  oper- 
ating table  caused  it  in  one  of  my  cases.  And  many  cases  have  been 
noticed  after  exposure  to  cold. 

The  symptoms  of  the  disease  are  severe  pains  about  the  shoulder 
and  neck  and  total  paralysis  of  the  serratus  magnus  muscle.  It  will 
be  remembered  that  the  function  of  this  muscle  is  to  hold  the  posterior 
edge  of  the  shoulder-blade  against  the  chest  and  to  rotate  the  shoulder- 
blade  in  the  act  of  raising  the  arm.  When  it  is  paralyzed  the  shoul- 
der-blade projects  from  the  chest  like  a  wing  in  a  very  characteristic 
manner  (Figs.  92,  93,  94);  and  the  arm  cannot  be  lifted  much  above 
the  horizontal  level  when  the  forearm  is  extended.  Motions  of  the 
forearm  forward  and  backward  are  also  somewhat  impaired.  If  the 
arms  be  abducted,  forcible  inspiration  shows  a  defective  expansion  of 
the  chest  on  the  paralyzed  side.  An  electrical  examination  usually 
demonstrates  a  reaction  of  degeneration  in  the  serratus  magnus  muscle. 
The  motor  points  of  this  muscle  are  on  the  side  of  the  chest  beneath 
the  axilla.  A  slow  recovery  usually  ensues,  provided  the  nerve  is  not 
entangled  in  the  scar  at  the  point  of  injury.  If  recovery  fails  to  occur 
after  several  months,  a  surgical  exploration  is  justifiable. 

PARALYSIS  OF  THE  MUSCULOSPIRAL  NERVE. 

This  nerve  is  injured  more  often  than  any  other  of  the  nerves  of  the 
arm  inasmuch  as  it  is  exposed  in  its  passage  around  the  humerus  just 
below  the  insertion  of  the  deltoid.  Pressure  of  the  head  lying  upon  the 
arm,  which  frequently  occurs  in  the  sleep  of  intoxication,  is  sufficient 


PABALYSIS  OF  THE  MUSCVLOSPIBAL  NEHVE. 


185 


to  cause  musculospiral  palsy,  or  the  compression  of  nerve  in  the  axilla, 
if  during  sleep  the  arm  hangs  over  a  sharp  edge  of  a  bench  or  bed. 
The  pressure  of  a  crutch  may  also  produce  this  form  of  paralysis.     In 


Fig.  93. 


Paralysis  of  the  serratus  magnus  muscle, 
causing  displacement  of  the  scapula  when 
the  arm  is  held  forward.  (Icon,  de  la 
Salpetri6re. ) 


Paralysis  of  the  serratus  magnus  muscle, 
causing  displacement  of  the  scapula  when 
the  arm  is  held  forward. 


Fig.  94. 


Paralysis  of  the  serratus  magnus  from  injury  of  the  posterior  thoracic  nerve.  Position  of  the 
scapula  when  the  arm  is  abducted.  Further  elevation  of  the  arm  is  impossible,  as  rotation  of  the 
scapula  is  impossible. 

Germany  this  is  called  "  police  paralysis,"  because  of  the  method  used 
in  securing  the  arms  of  a  prisoner  by  placing  a  bar  across  his  back 
and  beneath  his  two  elbows  and  tying  the  forearms  firmly  to  the  arms. 


186 


INJVnitS  OF  SPINAL  NSBVES  AND  NEVBITIS. 


Gowers  describes  three  cases  which  have  followed  violent  contraction 
of  the  triceps  —  one  in  pulling  on  a  pair  of  tight  boots  ;  one  in  throw- 
ing a  stone,  and  one  in  grasping  a  su})port  to  prevent  falling.  I  have 
seen  cases  which  have  developed  from  pressure  during  a  long-continued 
anaesthesia,  the  patient  coming  out  of  the  anaesthetic  to  find  his  arm 
paralyzed.  The  nerve  may  be  injured  in  fractures  of  the  middle  third 
of  the  humerus.  In  one  patient  the  administration  of  a  hypodermic 
injection  in  this  region  was  followed  by  paralysis. 

The  symptoms  produced  by  musculospiral  palsy  are  drop-wrist,  with 
pronation,  due  to  paralysis  of  the  long  extensors  of  the  wrist  and  long 

Fig.  95. 


Drop-wrist  due  to  musculosiiiral  paralysis. 

extensors  of  the  fingers  and  thmnb,  and  weakness  in  flexion  of  the 
elbow  from  paralysis  of  the  supinators.  If  the  nerve  is  injured  in  the 
axilla  the  triceps  is  also  paralyzed.  Sometimes  in  very  slight  cases 
the  supinator  longus  is  not  paralyzed.  The  position  of  the  hand  in 
drop-wrist  is  shown  in  Fig.  95.  The  action  of  the  flexors  of  the  hand 
is  apparently  weak  in  grasping  objects,  but  if  the  wrist  be  extended  by 
the  examiner  and  held  firmly  in  this  position,  the  flexors  are  found  to 
act  in  a  normal  manner.  The  awkward  action  of  the  forearm  gives 
the  patient  considerable  discomfort,  especially  the  loss  of  power  of 
supination.  After  the  drop-wrist  has  continued  for  some  days  a 
prominence  appears  upon  the  back  of  the  hand  as  a  result  of  the  over- 
flexion  of  the  wrist,  due  to  a  partial  dislocation  of  one  of  the  bones  of 
the  hand.  But  this  subsides  when  recovery  has  taken  place.  The 
muscles  paralyzed  show  the  reaction  of  degeneration,  excepting  in  very 
light  cases.  In  the  early  stage  of  the  aflection  some  tingling  and 
numbness  may  be  felt  over  the  back  of  the  hand  at  the  base  of  the 
index  finger  and  thumb,  and  an  impairment  of  sensibility  may  often 
be  demonstrated  in  this  region.     (See  Plate  XIV.)     This,  however, 


PaMLTsis  of  the  ULNAB  nebve.  187 

rapidly  subsides.  Musculospiral  paralysis  can  hardly  be  mistaken  for 
any  other  disease,  though  drop-wrist  may  develop  in  any  form  of  mul- 
tiple neuritis,  especially  in  lead  palsy.  It  is  then,  however,  bilateral, 
and  the  supinator  longus  usually  escapes. 

The  prognosis  is  good  for  recovery,  but  this  usually  requires  from 
three  to  five  months. 

The  treatment  is  that  for  ordinary  traumatic  neuritis. 

PARALYSIS  OF  THE  MEDIAN  NERVE. 

This  may  occur  from  wounds  or  injuries  of  the  forearm  or  of  the 
brachial  plexus,  also  from  pressure  of  a  crutch.  The  median  nerve 
supplies  the  pronators,  the  flexor  carpi  radialis,  the  flexors  of  the 
fingers,  and  the  muscles  that  abduct  and  flex  and  oppose  the  thumb 
and  tlie  two  radial  lumbricales,  which  flex  the  first  phalanx  ;  hence 
this  injury  produces  paralysis  in  these  muscles,  resulting  in  supination 
of  the  forearm  and  inability  to  flex  the  fingers  firmly  or  to  use  the 
hand.  The  thumb  cannot  be  abducted  or  opposed  to  the  tips  of  the 
fingers.  The  anaesthesia  produced  is  shown  in  Figs.  96  and  97.  Pain 
is  sometimes  felt  in  the  hand.  When  the  nerve  is  injured  at  the  wrist 
the  action  of  the  long  flexors  is  not  interfered  with,  hence  the  paralysis 
is  limited  to  the  fingers  alone.  There  is  usually  some  atrophy  of  the 
thenar  muscles.     Trophic  changes  on  the  skin  and  nails  often  occur. 

The  prognosis  and  treatment  are  in  accordance  with  ordinary  trau- 
matic neuritis. 

PARALYSIS  OF  THE  ULNAR  NERVE. 

The  ulnar  nerve  is  very  frequently  injured,  as  its  course  is  an  ex- 
posed one  at  the  elbow  and  in  the  forearm.  It  may  be  affected  by 
pressure  in  the  axilla  by  a  crutch,  but  this  is  rare.  Exposure  to  wet 
and  cold  often  causes  ulnar  neuritis.  Long-continued  pressure  in  a 
condition  of  anaesthesia,  or  even  in  the  heavy  sleep  of  intoxication,  may 
cause  paralysis  of  the  muscles  supplied  by  the  ulnar  nerve,  and  after 
dislocation  and  fractures  of  the  arm  or  forearm  it  has  been  frequently 
observed.  Callus,  after  fractures  about  the  elbow,  occasionally  presses 
on  and  involves  the  nerve.  The  ulnar  nerve  supplies  the  flexor  carpi 
ulnaris,  the  ulnar  half  of  the  deep  flexors  of  the  flngers,  the  muscles 
of  the  little  finger,  the  interossei,  the  inner  two  lumbricales,  and  the 
adductors  of  the  thumb. 

The  result  of  paralysis  of  the  ulnar  flexor  of  the  wrist  is  not  very 
manifest,  as  other  muscles  are  capable  of  doing  the  work  of  the 
flexor  carpi  ulnaris.  A  paralysis  of  the  flexor  profundus  digitorum 
makes  the  patient  incapable  of  flexing  the  first  phalanges  of  the  little 
and  ring  fingers,  hence  there  is  no  opposition  to  their  extreme  exten- 
sion, and  part  of  the  deformity  of  the  hand  resulting  from  ulnar  nerve 
palsy  (Figs.  98  and  99)  is  due  to  this  cause. 

The  chief  disability,  however,  produced  by  ulnar  nerve  lesions  is  the 
paralysis  of  all  the  intrinsic  muscles  of  the  hand  excepting  the  first  and 


188  INJUmES  OF  SPINAL  NERVES  AND  NEVmflS. 

second  lumbricales,  which  are  supplied  by  the  median  nerve.  As  a 
result  of  this  paralysis  the  use  of  the  thumb  and  fingers  is  very  much 
impaired,  the  thenar  and  hypothenar  eminences  become  flat  and  flabby, 


Fig.  96. 


Fig.  97. 


Showing  areas  of  sensory  loss  in  injuries  of  the  median  nerve.     Horizontal  lines  show  total  anaesthesia. 
Vertical  lines  show  partial  anaesthesia.     ( Bowlby. ) 


Fig.  98. 


Fig.  99. 


Showing  sensory  loss  and  abnormal  position  after  injuries  of  the  ulnar  nerve.     (Bowlby. ) 

the  interossei  of  the  hand  are  also  atrophied,  so  that  the  bones  and 
long  tendons  of  the  fingers  stand  out  beneath  tlie  skin  ;  and  the  little 
and  ring  fingers  are  flexed  in  their  second  and  third  phalanges  because 


Muscles  of  back 


NEURIT  IS   OF  THE  LUMBAB  NERVES.  189 

of  the  inability  to  extend  them,  due  to  the  complete  paralysis  of  the 
interossei.  Tjais  is  the  second  cause  of  the  deformity  produced  in  the 
hand.  The  extension  of  the  middle  and  end  phalanges  of  the  other 
two  fingers  is  also  considerably  impaired.  The  adduction  and  abduc- 
tion of  the  fingers  is  completely  abolished  and  the  thumb  cannot  be 
brought  forward  because  of  overextension  of  its  first  phalanx  by  the 
extensor  longus  pollicis. 

The  sensory  disturbances  produced  by  ulnar  palsy  are  tingling  and 
numbness  and  possibly  pain  in  the  inner  half  of  the  hand,  in  the  little 
finger  and  one-half  of  the  ring  finger,  and  these  parsesthesia  are  usually 
attended  by  anaesthesia,  which  may  be  total  in  the  little  finger,  but  is 
only  partial  in  the  hand  and  ring  fingers. 

The  sensory  disturbance  is  not  always  a  total  one,  sensations  of  tem- 
perature and  pain  being  very  often  retained  when  the  sensation  of 
touch  is  lost.  Trophic  disturbances  are  very  liable  to  occur  in  the 
anaesthetic  region  of  the  skin.      (See  Fig.  104,  page  203.) 

The  prognosis  and  treatment  are  those  of  general  neuritis. 

NEURITIS  OF  THE  INTERCOSTAL  OR  DORSAL  NERVES. 

This  occasionally  occurs  after  injuries  in  which  ribs  are  broken  or 
from  pressure  by  small  tumors.  In  one  case  such  a  tumor  caused  in- 
termittent pressure  as  it  occasionally  got  caught  between  the  ribs  and 
then  produced  excruciating  pain.  It  was  successfully  removed.  The 
nerve  aifected  will  be  indicated  by  the  distribution  of  the  pain  or  of 
the  ansesthesia,  as  shown  on  Plate  VII.  There  is  no  paralysis  caused 
by  intercostal  neuritis. 

NEURITIS  OF  THE  LUMBAR  NERVES. 

The  anatomy  of  the  lumbar  and  sacral  plexus  is  shown  in  Plate 
XV. 

The  lumbar  plexus  supplies  the  flexors  and  abductors  of  the  thigh, 
the  extensors  of  the  knee  and  the  cremaster  muscle.  Its  sensory  dis- 
tribution is  shown  in  Plate  XIV. 

The  lumbar  plexus  rarely  suifers  from  neuritis,  as  it  is  well  pro- 
tected from  cold  or  from  external  injury.  But  tumors  within  the 
spinal  canal,  tumors  in  the  abdomen,  cancer  of  the  spine,  and  psoas 
abscesses  occasionally  set  up  a  neuritis  or  cause  pressure  on  the  nerves 
of  the  plexus.  I  have  seen  several  cases  following  operations  for 
appendicitis  where  filaments  of  the  nerves  were  divided. 

The  symptoms  may  be  limited  to  one  or  two  muscles,  such  as  those 
supplied  by  the  obturator  nerve,  or  the  anterior  crural  nerve,  or  may 
be  widespread.  In  the  first  case  the  legs  cannot  be  crossed,  as  the 
abductors  of  the  thigh  are  paralyzed  and  rotation  of  the  leg  is  impaired. 
In  the  second  case  the  extensors  of  the  knee  are  weak  and  atrophied 
and  the  knee-jerk  is  lost.  In  both  cases  the  inability  is  marked  in 
walking,  and  the  patients  move  with  difficulty.  There  is  usually  pain  in 
the  groin  and  thigh,  and  sometimes  this  shoots  downw^ird  into  the  genital 


190  INJURIES   OF  SPINAL  NEBVES  AND  NEUBITIS. 

region.  There  may  be  tenderness  along  the  course  of  the  anterior  crural 
nerve.  The  degree  of  anaesthesia  in  the  skin  is  slight,  but  tingling  and 
numbness  are  felt  in  the  sensory  distribution  of  the  plexus. 

PARALYSIS  OF    THE  EXTERNAL  CUTANEOUS   NERVE. 

The  external  cutaneous  nerve  of  the  thigh  supplies  the  skin  over  the 
outer  portion  of  the  thigh,  from  the  line  of  the  trochanter  downward 
to  the  knee.  Occasionally  this  nerve  is  diseased  and  then,  as  a  rule, 
there  is  a  sensation  of  pain  in  its  distribution,  which  is  attended  by 
parsesthesia  and  by  diminution  of  sensibility  in  the  skin.  The  affection 
runs  the  ordinary  course  of  a  neuritis,  and  should  be  treated  as  a 
peripheral  neuritis.  Attention  was  first  called  to  it  by  Bernhardt,  and 
hence  it  has  been  termed  "  Bernhardt's  Paralysis." 

NEURITIS  OF  THE  SACRAL  PLEXUS. 

Neuritis  of  the  sacral  plexus  is  a  rare  affection  and  is  usually  due  to 
injury  of  the  sacral  plexus  during  a  difficult  labor.  Every  obstetrician 
has  observed  a  number  of  cases  in  which  during  a  delayed  or  difficult 
labor,  the  woman  has  been  seized  with  sudden  severe  pains  in  one  leg, 
usually  in  the  back  of  the  leg,  and  often  below  the  knee,  and  has 
suffered  from  a  flexor  spasm  of  the  leg  for  some  hours  during  labor. 
A  day  or  two  after  delivery  paralysis  of  the  muscles  of  the  leg  below 
the  knee  develops,  and  also  a  condition  of  anaesthesia  extending 
down  the  back  of  the  thigh  and  around  the  outer  side  of  the  leg 
and  into  the  foot.  In  some  cases  the  bruising  of  the  nerve  is  a  slight 
one  and  all  the  symptoms  subside  within  three  or  four  weeks.  In 
other  cases  the  injury  is  more  severe,  and  months  elapse  before  the 
paralysis  subsides  and  the  patient  is  able  to  w^alk.  It  is  to  be  remem- 
bered that  conditions  of  paralysis  may  follow  labor  from  anaemic  states 
of  the  spinal  cord  or  from  an  infectious  multiple  neuritis.  These  con- 
ditions, however,  produce  paralysis  in  both  legs,  while  the  disease 
under  discussion  is  always  unilateral.  In  sacral  plexus  paralysis  the 
functions  of  the  bladder  and  rectum  are  usually  normal,  and  the  symp- 
toms of  neuritis,  pain,  tenderness,  paralysis,  reaction  of  degeneration, 
are  limited  to  the  distribution  of  the  sciatic  nerve.  Tumors  in  the 
pelvis  and  caries  of  the  sacrum  produce  the  same  condition. 

NEURITIS  OF  THE  SCIATIC  NERVE. 

Sciatica,  or  neuralgia  of  the  sciatic  nerve,  is  considered  in  Chapter 
XLII,  but  there  are  many  cases  of  so-called  neuralgia  which  are  actually 
due  to  neuritis,  and  there  are  cases  of  neuritis  of  the  sciatic  nerve  which 
develop  without  any  preceding  neuralgia.  The  disease  may  be  pro- 
duced by  any  one  of  the  many  causes  of  neuritis  and  neuralgia,  but 
seems  to  be  more  common  as  a  sequel  of  the  grippe  than  of  any  other 
infectious  disease,  and  it  also  occurs  with  great  frequency  in  gouty  and 
anaemic  individuals. 

Symptoms.  —  The  symptoms  are  pain  in  the  entire  domain  of  the 


NEUEITIS  OF  THE  SCIATIC  NEEFE.  191 

sciatic  nerve  and  tenderness  along  the  course  of  the  nerve,  which 
is  not  to  be  confounded  "vv^ith  the  existence  of  tender  spots  at  Valleix's 
points  present  in  sciatica.  The  nerve  is  found  to  be  tender  in  its 
entire  length  and  not  infrequently  localized  swellings  can  be  detected 
by  palpation.  The  pain  is  usually  constant  as  contrasted  with  the 
paroxysmal  attacks  of  neuralgia.  The  patient  is  never  able  to  get  into 
an  easy  position  so  as  to  be  free  from  pain.  There  is  great  restless- 
ness and  uneasiness  in  the  entire  limb,  and  all  movement  is  extremely 
distressing.  Walking  can  only  be  done  with  difficulty  and  pain  and 
has  to  be  avoided.  Very  often  an  actual  paralysis  develops  in  the 
muscles  supplied  by  the  sciatic  nerve,  namely,  those  below  the  knee, 
and  an  atrophy  with  reaction  of  degeneration  may  develop.  Not 
infrequently  the  pain  of  the  neuritis  gives  rise  to  reflex  spasms  in  the 
muscles,  and  such  twitchings  of  the  muscles  of  the  leg  are  liable  to  be 
exceedingly  distressing  and  painful.  The  leg  is  frequently  hot  and 
the  secretion  of  perspiration  may  be  increased.  I  have  occasionally 
seen  glossy  skin  upon  the  outside  of  the  leg  and  upon  the  foot  and 
irregular  growth  of  the  toe-nails.  The  disease  is  very  slow  in  its 
progress,  and  often  remains  for  months  before  recovery  follows. 

Treatment.  —  Treatment  consists  of  hot  applications  to  the  leg, 
which  must  be  kept  at  rest,  the  patient  being  confined  to  thebed  and 
the  position  of  the  limb  changed  frequently  by  the  aid  of  numerous 
small  pillows.  It  is  a  mistake,  in  my  opinion,  to  apply  a  long  splint 
to  the  limb,  inasmuch  as  the  strained  position  produces  very  great 
disturbance  and  discomfort.  But  a  tight  strapping  about  the  hips  by 
adhesive  plaster,  holding  the  pelvic  bones  and  the  sacrum  together 
sometimes  is  useful.  The  tenderness  of  the  limb  secures  a  sufficient 
amount  of  rest  to  the  part  without  this  enforced  bandaging.  Hot 
applications  may  be  kept  up  by  poultices  or  by  the  use  of  hot  bottles, 
but  great  caution  is  to  be  used  in  applying  the  latter,  to  avoid  blisters 
or  serious  burns,  inasmuch  as  the  skin  may  be  insensitive  below  the 
knee.  Some  patients  experience  relief  from  the  use  of  electricity,  the 
continuous  galvanic  current  being  employed,  with  the  positive  pole 
over  the  painful  nerve,  the  negative  pole  being  placed  upon  the  back. 
The  best  method  of  treatment,  however,  is  by  means  of  the  actual 
cautery,  which  often  gives  relief  from  the  pain  for  several  hours,  so 
that  the  patients  are  glad  to  have  the  application  repeated,  even  though 
it  is  painful.  The  various  forms  of  hydrotherapy,  especially  a  hot 
douche  in  a  bath,  are  of  service.  As  in  the  treatment  of  brachial  neuritis, 
sedatives  may  be  used  freely.  Hypodermics  of  cocaine  in  the  upper 
part  of  the  nerve  are  often  better  than  morphine.  When  the  condition 
is  not  improved  by  such  treatment,  puncture  of  the  nerve  by  a  needle 
has  been  known  to  give  relief,  probably  by  allowing  fluid  to  escape 
from  the  sheath.  Several  needles  may  be  passed  into  the  nerve  and 
allowed  to  remain  for  an  hour.  This  must  be  done  with  great  care  so 
as  to  be  aseptic.  In  chronic  cases  stretching  the  nerve  has  been  done 
with  some  good  results,  but  too  much  should  not  be  promised,  as  the 
operation  is  uncertain.     The  sciatic  may  be  stretched  without  an  opera- 


192  INJUBIES   OF  SPINAL  NEBVES  AND  NEUBITIS. 

tion,  the  leg  being  extended  and  the  thigh  forcibly  flexed  on  the  trunk. 
This  is  usually  a  very  painful  procedure,  requiring  an  ansesthetic.  It 
is  occasionally  of  benefit  in  those  cases  where  an  interstitial  neuritis  has 
caused  adhesions  between  the  nerve  and  its  sheath. 

PARALYSIS   OF  THE  EXTERNAL  POPLITEAL  NERVE. 

The  external  popliteal  branch  of  the  sciatic  nerve  in  its  passage  around 
the  fibula  is  exposed  to  injury  either  directly  from  pressure,  as  by  a 
tight  garter,  or  from  external  wounds,  or  from  dislocation  or  fracture  of 
the  fibula.  The  result  of  such  injury  is  a  paralysis  of  the  tibialis  anticus, 
of  the  long  peronei  muscles  in  the  leg  and  of  the  extensors  of  the  toes, 
causing  a  dropping  and  adduction  of  the  foot  and  toes  and  lame- 
ness in  walking.  The  entire  leg  is  raivsed  so  that  the  toes  do  not  trip 
the  walker.  Atrophy  soon  follows  with  reaction  of  degeneration. 
There  may  be  a  loss  of  sensation  in  the  outer  side  of  the  leg  and  on 
the  back  of  the  foot.  Prognosis  and  treatment  are  the  same  as  in 
other  forms  of  traumatic  neuritis. 


PARALYSIS  OF  THE  INTERNAL  POPLITEAL  NERVE. 

Paral^^sis  of  the  internal  popliteal  nerve  is  much  more  rare,  as  it  is 
protected  by  its  deep  position  under  the  knee.  Occasionally  it  is 
strained  or  wounded.  Then  paralysis  of  the  muscles  of  the  calf  of  the 
leg  and  of  the  sole  of  the  foot  causes  much  difficulty  in  walking.  The 
patient  cannot  rise  on  the  toes  or  flex  the  ankle  or  toes.  There  may 
be  pain,  numbness,  or  anaesthesia  in  the  outer  part  of  the  foot  and  in 
the  sole. 

NEURITIS  OF  THE  PLANTAR  NERVES. 

The  finer  branches  of  the  plantar  nerves  are  not  infrequently  affected 
by  neuritis.  Such  neuritis  may  be  traumatic  from  walking  in  ill-fitting 
shoes,  (3r  upon  very  rough  ground  for  an  unusual  length  of  time.  It 
may  be  due  also  to  taking  cold,  as  when  a  person  stands  for  some  hours 
in  the  wet,  or  has  the  feet  chilled  and  frozen.  The  symptoms  are 
pain  along  the  course  of  the  nerves,  between  the  long  bones  of  the  foot, 
great  tenderness  to  pressure,  and  inability  to  endure  the  pressure  of 
the  shoe  and  inability  to  wallc  on  account  of  the  pain.  Gowers  pointed 
out  the  fact  that  propulsion  of  the  body  forward  is  done  by  pressure 
on  the  toes,  and  that  when  they  are  paralyzed  the  act  of  walking  is 
seriously  impeded.  Not  infrequently  irregular  areas  of  anaesthesia 
may  be  found  in  the  sole,  and  the  patient  suffers  from  prickling  and 
burning  pain,  as  well  as  from  sharp  neuralgic  pains.  The  sensory 
symptoms  may  be  associated  with  motor  symptoms  of  paralysis  of  the 
intrinsic  muscles  of  the  foot,  so  that  the  toes  cannot  be  moved  freely 
and  the  foot  may  become  quite  thin  in  consequence  of  the  atrophy. 
Reaction  of  degeneration  is  not  infrequently  present.  The  affection 
must  be  treated  by  rest  in  bed,  the  patient  not  being  allowed  to  walk 
about,  by  local  applications  of  heat  and  by  the  general  measures  recpm' 
mended  for  the  treatment  of  neuritis. 


TUMOBS   OF   THE  NERVES.  193 

MORTON'S  TOE. 

In  some  persons  the  second  digital  branch  of  the  internal  plantar 
nerve  in  its  passage  between  the  enlarged  ends  of  the  first  and  second 
metatarsal  bones  is  compressed  by  anything  which  brings  these  two 
bones  in  close  apposition.  In  others,  any  one  of  the  digital  nerves 
is  similarly  liable  to  be  compressed.  It  seems  probable  that  a  con- 
genitally  abnormal  position  of  the  nerve  is  the  predisposing  cause  of 
this  affection.  The  disease  develops  in  adults,  and  is  usually  the 
consequence  of  wearing  shoes  that  are  too  narrow.  The  patient  will 
be  seized  with  a  sudden  sharp  pain  between  the  great  and  second 
toes,  or  between  other  toes,  which  is  so  agonizing  in  character  as  to 
prevent  his  taking  another  step,  and  which  lasts  until  the  shoe  is  re- 
moved and  by  a  certain  amount  of  manipulation  the  bones  are  separated 
and  the  pressure  removed.  But  such  attacks  are  liable  to  recur  with 
great  frequency,  and  to  give  rise  to  such  distress  and  inability  to  walk 
as  to  require  treatment.  The  disease  is  purely  mechanical  in  its  origin, 
and,  therefore,  anything  which  removes  the  pressure  or  which  prevents 
the  recurrence  of  the  pressure  will  cure.  In  some  cases  it  is  sufficient 
to  prescribe  a  very  wide  shoe.  In  others  it  is  necessary  to  insert  pads 
and  strap  them  with  adhesive  plaster  to  the  foot  in  such  a  position  as 
to  keep  the  metatarsal  bones  separated.  Flatfoot  may  be  the  cause, 
and  if  corrected  the  pain  may  be  obviated.  But  in  some  cases  all 
these  mechanical  contrivances  fail  to  give  relief,  and  then  resort  to 
surgical  treatment  may  be  necessary.  Morton  was  the  first  to  operate 
for  this  condition,  and  hence  it  has  been  named  after  him.  The  opera- 
tion may  consist  in  a  division  of  the  nerve  above  the  point  of  pressure, 
by  an  exsection  of  a  portion  of  the  nerve,  but  inasmuch  as  regeneration 
is  sure  to  occur,  this  operation  is  unsatisfactory.  The  operation  de- 
vised by  Morton  was  exsection  of  the  end  of  the  metatarsal  bone  caus- 
ing the  pressure,  and  this  has  uniformly  given  relief. 

TUMORS    OF    THE    NERVES. 

Neuroma.  —  Any  form  of  tumor  —  fibroma,  angioma,  sarcoma,  etc., 
—  may  develop  within  a  nerve  sheath  or  upon  it  and  thus  produce 
an  apparent  tumor  of  the  nerve.  In  such  cases  the  nerve  fibres  may 
pass  through  the  tumor  or  may  be  dissected  apart  by  it  and  pass 
around  it.  Such  tumors  have  been  termed  false  neuromata,  for  they  are 
not  made  up  of  nerve  cells  or  fibres.  But  the  fibres  may  become  pressed 
by  the  new-growth  and  undergo  degeneration.  And  the  symptoms  and 
course  of  the  case  under  such  conditions  will  be  exactly  such  as  have  been 
already  considered  under  compression  of  the  nerves.  Fig.  100  shows 
such  false  neuromata,  which  are  very  often  multiple,  as  in  this  case. 

Neuroma,  or  a  tumor  consisting  of  nerve  fibres,  first  described  by 
Virchow,  is  a  rare  form  of  tumor.  The  fibres  of  which  it  consists 
may  be  medullated  or  non-medullated ;  nerve  cells  are  not  found  in 
such  tumors ;  connective  tissue  exists  in  greater  or  less  degree,  so  that 

13 


194 


INJUEIES   OF  SPINAL  NEBVES  AND  NEURITIS. 


some  tumors  are  neuro-fibromata.  There  appears  to  be  a  tendency  for 
such  tumors  to  be  multiple,  and  in  the  large  majority  of  the  recorded 
cases  hundreds  of  minute  tumors  have  developed.  Occasionally  the 
tumors  have  been  large,  but  usually  they  are  the  size  of  a  pea.  When 
they  appear  on  the  sensory  nerves  of  the  skin  they  are  easily  felt  and 
are  usually  tender.     Under  these  circumstances  they  have  been  called 

riG.  100, 


Multiple  neuromata.     Nerves  of  the  arm,  the  lumbar  plexus,  and  the  sciatic  nerve.    Specimen  from 
the  Museum  of  the  College  of  Physicians  and  Surgeons. 

"  tubercula  dolorosa."  I  have  seen  a  patient  with  more  than  a  hun- 
dred such  tumors  distributed  over  the  entire  body.  Hoggan  has 
shown  that  some  tumors  of  this  description  were  adenomata  of  the 
sweat  glands.  That  some  congenital  tendency  to  the  multiplication  of 
nerve  fibres  is  at  the  basis  of  this  affection  is  proved  by  the  fact  that 
many  of  the  cases  reported  have  been  in  children.  Thus  in  the  so- 
called  plexiform  neuroma  of  the  fifth  nerve  the  disease  is  usually  con- 
genital and  other  nerves  have  been  affected  later  in  life.  The  tumors 
attain  a  certain  size  and  then,  as  a  rule,  cease  to  grow ;  the  condition 
remains  permanent  until  death  from  some  other  cause. 

In  the  majority  of  cases  no  symptoms  are  caused  by  neuromata,  as 


ISCHEMIC   PARALYSIS.  195 

the  nerves  on  which  the  tumors  develop  are  not  afFected  by  the  growth. 
The  tumors  riiay  be  felt  'and  may  be  tender  to  pressure,  but  are  not 
subject  to  treatment  and  cannot  safely  be  removed,  as  they  are  so 
numerous  and  as  the  nerves  may  be  cut.  Occasionally  pain,  hyperses- 
thesia,  and  numbness  are  caused  by  neuromata. 

There  is  one  form  of  neuroma  which,  however,  requires  special  men- 
tion. It  is  the  neuroma  which  develops  on  the  cut  end  of  a  divided 
nerve,  either  after  an  amputation  or  after  an  injury  without  union. 
Such  a  neuroma  may  be  extremely  painful  and  tender  and  may  cause 
pains  referred  to  the  peripheral  termination  of  the  nerve,  and  also 
spasms  in  the  muscles  related  to  these  terminations.  In  such  cases 
excision  is  imperative,  but  recurrence  is  not  infrequent. 

In  one  patient  under  my  observation,  who  suffered  from  great  pain 
referred  to  the  toes,  for  many  years  after  an  amputation  of  the  leg 
above  the  knee,  excision  of  the  neuroma  on  the  sciatic  failed  to  give 
relief.  A  portion  of  the  sciatic  nerve  in  the  thigh  was  then  excised, 
but  this  also  failed  to  stop  the  pain.  In  this  case  it  seemed  probable 
that  atrophic  changes  had  occurred  in  the  spinal  cord,  such  as  are 
known  to  follow  amputations,  and  that  these  had  involved  not  only 
the  anterior  horns,  but  also  the  sensory  columns. 

ISCHEMIC  PARALYSIS. 

There  is  a  condition  of  paralysis  which  occurs  as  a  result  of  tight 
bandaging  of  a  limb  (usually  after  fractures)  termed  ischsemic  paralysis. 
The  bandage  compresses  the  muscles  and  vessels  and  nerves  and 
causes  very  great  pain.  This  pain  is  ascribed,  erroneously,  to  the 
fracture,  and  the  bandage  may  be  allowed  to  remain  on  the  limb  for 
several  days  in  spite  of  the  pain.  Then  when  it  is  removed  the  limb 
is  found  to  be  paralyzed,  the  muscles  are  hard,  indurated,  rigid  and 
cannot  be  moved  voluntarily  and  often  any  attempted  passive  motion 
is  found  to  be  very  difficult  and  to  cause  pain.  Thus,  if  the  limb 
affected  be  the  arm,  it  may  be  impossible  to  flex  or  to  extend  the 
fingers  without  meeting  with  resistance,  and  causing  pain  in  the  muscles 
of  the  forearm.  The  mucles  are  swollen,  are  hard  to  the  touch,  do 
not  pit  on  pressure  but  are  tense;  their  mechanical  excitability  is  lost 
and  the  electrical  excitability  is  diminished.  The  limb  itself  is  cold, 
its  circulation  is  defective.  There  may  be  numbness  and  anaesthesia 
to  all  sensations  in  some  cases;  in  other  cases  there  is  no  evidence  of 
neuritis  in  the  limb.  The  skin  may  be  mottled  or  glossy  or  rough. 
Sometimes  ulcerations  due  to  the  pressure  are  found. 

This  condition  may  be  ascribed  to  neuritis  in  consequence  of  the 
fracture,  by  the  surgeon  who  is  responsible  for  the  tight  bandaging. 
But  it  is  easily  differentiated  from  neuritis,  for  that  condition  never 
causes  the  brawny  infiltration  and  rigidity  of  the  mucles  present  in 
ischsemic  paralysis;  and  the  anaesthesia  is  limited  to  the  domain  of 
definite  nerves  in  neuritis  while  in  this  condition  it  is  rarely  present 
or  if  so  it  is  distributed  over  the  entire  limb. 


196  INJUBIES   OF  SPINAL  NEBFES  AND  NEUBITIS. 

The  prognosis  in  ischsemic  paraly>sis  is  grave.  Months  and  even 
years  may  elapse  before  nature  restores  the  circulation  and  nutrition  of 
the  muscles  which  are  primarily  affected. 

The  treatment  consists  in  daily  massage,  in  very  hot  baths  twice  or 
three  times  a  day  kept  up  for  half  an  hour  and  followed  by  cool  spong- 
ing and  in  passive  movements  performed  after  the  hot  baths. 

Surgeons  have  sometimes  cut  down  upon  the  tendons  of  the  wrist  or 
ankle  when  these  appear  to  be  matted  together  and  adherent  to  their 
sheaths  and  have  broken  up  adhesions  with  good  results. 


CHAPTER   VIII. 

MULTIPLE   NEUEITIS. 

Varieties.     Etiology.      Symptoms.      Course.      Duration.      Diagnosis.      Prognosis  and 

Treatment. 

Multiple  neuritis  or  polyneuritis  is  a  general  disseminated  inflam- 
mation or  degeneration  of  the  nerves,  symmetrical  in  its  distribution 
upon  both  sides  of  the  body  and  generally  aifecting  all  the  nerves  of 
the  limbs,  particularly  in  their  terminal  branches.  It  is  hence  called 
peripheral  neuritis.  The  affection  rarely,  if  ever,  extends  as  high  as 
the  nerve  plexuses. 

Varieties.  — There  are  a  number  of  forms  of  multiple  neuritis  which 
difi'er  from  one  another  somewhat  in  their  pathology  and  in  their  symp- 
tomatology. The  following  etiological  classification  of  these  various 
forms  is  of  practical  use. 

1.  Toxic  cases  due  to  the  action  of  a  poison  derived  from  without 
the  body.  These  poisons  are  alcohol,  carbonic  oxide  gas,  bisulphide 
of  carbon,  the  coal-tar  products,  especially  sulphonal  and  trional ;  and 
nitrobenzol ;  also  arsenic,  lead,  mercury,  copper,  phosphorus  and  silver. 

2.  Infectious  cases  due  to  some  agent  acquired  or  developed  within 
the  body  as  an  accompaniment  or  sequel  of  diphtheria,  grippe,  typhoid, 
typhus,  malaria,  scarlet  fever,  measles,  whooping-cough,  smallpox,  ery- 
sipelas, and  septicsemic  conditions,  including  gonorrhoea  and  puerperal 
fever,  epidemic  forms  of  beriberi  or  kakke,  and  leprous  neuritis. 

3.  Cases  due  to  general  diseased  states  of  the  body  whose  origin  is 
undetermined,  such  as  rheumatism,  gout,  diabetes,  anaemia,  marasmus, 
general  malnutrition  consequent  upon  tuberculosis,  syphilis,  and  senility, 
carcinoma,  and  local  malnutrition  produced  by  arterial  sclerosis. 

4.  Cases  due  to  exposure  to  cold  and  developing  spontaneously 
without  known  cause. 

Etiology. — Multiple  neuritis  is  more  common  in  males  than  in 
females  in  all  varieties.  All  ages  are  liable  to  be  aifected.  The  diph- 
theritic type  is  of  course  more  commonly  seen  in  children  than  in 
adults,  and  if  these  cases  are  included  in  a  general  list  it  would  appear 
as  if  children  were  more  subject  to  the  disease  than  adults.  In  154 
miscellaneous  cases,  not  diphtheritic,  16  were  between  the  ages  of  six- 
teen and  twenty  years,  34  were  between  the  ages  of  twenty  and  thirty 
years,  54  were  between  the  ages  of  thirty  and  forty  years,  35  were  be- 
tween the  ages  of  forty  and  fifty  years,  19  were  between  the  ages  of 
fifty  and  sixty  years,  and  10  were  over  sixty  years  of  age.  Heredity 
appears  to  play  little  or  no  part  in  the  causation  of  multiple  neuritis, 
excepting  in  so  far  as  the  general  tendencies  to  gout,  rheumatism,  dia- 

197 


198  MULTIPLE    NEUEITIS. 

betes,  carcinoma,  arterial  sclerosis,  and  tuberculosis  may  be  said  to  be 
hereditary. 

It  seems  probable  that  in  many  cases  a  number  of  different  causes 
combine  to  start  the  neuritis.  Thus  in  a  person  who  has  used  alcohol 
for  some  years  the  disease  may  not  develop  until  some  acute  illness, 
like  an  attack  of  the  grippe,  or  of  rheumatism,  or  an  exposure  to  cold 
occurs,  when  a  typical  alcoholic  neuritis  may  manifest  itself.  The  par- 
ticular elements  of  causation  will  be  more  fully  discussed  in  connec- 
tion with  the  various  types  of  the  affection. 

Symptoms.  —  A  general  statement  of  the  symptoms  occurring  in 
any  or  all  forms  of  multiple  neuritis  will  be  made,  and  then  the  special 
combination  of  these  symptoms,  together  with  the  common  course  of 
the  disease  in  the  different  classes  of  cases,  will  be  presented.  Some 
authors  have  attempted  a  symptomatic  classification  of  the  forms  of 
neuritis,  and  distinguish  between  sensory,  motor,  and  ataxic  types  of 
the  disease.^  As  many  cases,  however,  present  a  combination  of  these 
symptoms  such  a  division  does  not  appear  to  me  to  be  scientifically 
justifiable,  however  convenient  from  the  clinical  standpoint. 

The  sensory  symptoms  of  multiple  neuritis  are  the  earliest  to  appear 
and  the  last  to  pass  away.  In  the  majority  of  the  cases  on  record, 
from  whatever  cause,  numbness,  tingling,  or  formication  ushers  in  the 
disease.  These  forms  of  parsesthesise  begin  in  the  feet  and  hands  and 
extend  to  the  knees  and  elbows.  They  may  be  associated  with  burn- 
ing, stretching,  boring,  or  tearing  sensations  M^hich  distress  the  patient 
especially  during  the  onset,  and  such  sensations  usually  increase  as  the 
affection  reaches  its  height.  Their  subsidence,  as  the  case  goes  on, 
may  be  regarded  as  a  favorable  symptom,  but  they  are  among  the  last 
evidences  of  the  disease  to  disappear.  Pain  is  usually  present  as  well 
as  parsesthesise.  It  is  sharp  in  character  and  is  usually  intermittent. 
At  times  it  may  be  lancinating  and  so  severe  as  to  necessitate  the  use 
of  morphine.  It  is  fully  as  distressing  as  in  cases  of  locomotor  ataxia. 
Tenderness  in  the  nerves  and  muscles  is  a  constant  symptom.  It  may 
be  so  extreme  that  the  limbs  cannot  be  moved  or  handled,  and  thus  it 
may  interfere  with  the  application  of  electricity  and  massage.  When 
the  tenderness  and  pain  are  referred  to  the  joints,  as  not  infrequently 
occurs  in  the  early  stage  of  the  disease  and  in  the  spontaneous  and  in- 
fectious cases,  the  case  may  be  mistaken  for  one  of  acute  articular 
rheumatism ;  and  if  the  joints  are  swollen  or  the  limbs  oedematous  the 
difficulty  of  diagnosis  is  greatly  increased. 

In  addition  to  these  subjective  feelings  some  demonstrable  distur- 
bance of  the  various  sensations  is  usually  present.  Hypersesthesia  to 
touch  and  also  to  electricity  is  not  infrequently  observed  during  the  first 
two  weeks.  It  is  usually  followed  by  some  ansesthesia,  although  this 
rarely  becomes  complete.  In  some  cases  the  loss  of  tactile  sense  is 
quite  evident  from  the  onset,  either  limited  to  the  cutaneous  distribu- 
tion of  some  special  nerve,  in  which  case  oddly-shaped  areas  of  insen- 
sibility will  be  found,  as  in  lepra,  or,  as  is  most  often  the  case,  about 

^  Judson  Burj.     Clifford  AUbutt's  System  of  Medicine,  vol.  vi. 


SYMPTOMS. 


199 


uniformly  distributed  over  the  distal  parts  of  the  extremities  and  fad- 
ing off  inta  normal  conditions  higher  up  upon  the  limb.  When  the 
ansesthesia  is  at  its  height  the  patient  has  difficulty  in  locating  a  touch 
upon  the  hands  and  feet,  even  though  he  feels  it.  The  distribution  of 
the  ansesthesia  when  it  is  fully  developed  corresponds  to  the  parts  of 
the  extremities  covered  by  gloves  and  stockings,  and  hence  has  been 
named  the  glove-shaped  and  stocking-shaped  area  of  anaesthesia. 
(Fig.lOl.)     This  distribution  of  anaesthesia,  contrasting  sharply,  as  it 


Fig.  101. 


N.  cut.postr.- 


N.  occip.maj. 
N.  occip.min. 
N.  Auric.magn. 

NN.  supraclavic. 

R.  Dors.  N.  spin. 
N.  axill. 

N.  cut  medialis. 
N.  intercost. 
N.  rad. 
N.  cut.medius. 

N.  tnusccut. 
NN.  cut.clunium 

^N.perf  lig.sacro-tub. 
^ — N  rad. 
N.  uln. 
—N.  median. 


K.  cut.lat. 

N.peron. 
N.  sapfc:- 


N.  suralis ^| 

N.plant.lat.- 
N.  plant. medr- 
The  distribution  of  anesthesia  in  multiple  neuritis.    The  dotted  areas  are  anesthetic. 


These  do  not 


correspond  with  the  distribution  of  the  various  nerves. 


does,  with  that  due  to  spinal-cord  disease,  or  to  that  occurring  in  injuries 
of  single  nerves,  and  being  symmetrical  upon  the  two  limbs,  is  quite 
diagnostic  of  multiple  neuritis.  The  transmission  of  pain  and  temper- 
ature sensations  is  sometimes  delayed,  but  these  impressions  are  usually 
felt  quite  acutely.  The  sense  of  pressure  is  less  commonly  unpaired. 
The  sense  of  vibration  as  tested  with  the  tuning  fork  is  often  lost  early. 
The  muscular  sense  escapes  any  affection  in  some  cases,  but  in  others 
is  the  most  profoundly  disturbed  of  all  the  senses.  When  it  is  involved 
the  incoordination  and  ataxia  are  well-marked  symptoms,  and  some 
cases  have  been  mistaken  for  locomotor  ataxia  because  of  the  pre- 
dominance of  the  disturbance  of  muscular  sense. 


200  MULTIPLE   NEURITIS. 

The  ataxia  is  very  pronounced  in  diphtheritic  cases,  in  cases  due  to 
arsenical  poisoning,  and  in  some  cases  due  to  alcoholic  poisoning.  The 
French  and  Germans  distinguish  a  class  of  cases  of  neuritis  which 
they  term  neurotabes  peripherique  or  pseudo-tabes,  because  of  the  close 
resemblance  to  locomotor  ataxia. 

These  sensory  symptoms  are  usually  limited  to  the  forearms  and 
hands  and  to  the  legs  and  feet.  In  no  case  have  they  involved  the 
entire  extremities  or  the  trunk,  and  but  one  case  of  facial  tingling  with 
anaesthesia  has  been  recorded.*  The  skin  reflexes  are  usually  pre- 
served and  are  occasionally  exaggerated. 

The  special  senses  are  rarely  involved  in  multiple  neuritis.  It  is 
true  that  optic  neuritis  has  occurred  in  a  few  cases,  especially  in  cases 
due  to  alcoholic  poisoning,  and  in  some  cases  hearing  as  well  as  sight 
has  been  aifected.  These  cases  prove  that  no  nerve  can  be  said  to  be 
exempt  from  implication  in  this  disease,  but  the  liability  to  affection 
seems  to  be  slight  in  the  case  of  the  nerves  of  special  sense.  In  alco- 
holic cases  toxic  amblyopia  has  been  found. 

The  motor  symptoms  are  as  marked  and  as  important  as  the  sensory. 
Paralysis,  beginning  as  simple  weakness,  with  a  feeling  of  fatigue  on 
any  exertion,  gradually  increases  in  severity  until  at  the  height  of  the 
disease  it  becomes  complete.  It  usually  comes  on  rapidly,  so  that 
within  two  weeks  the  patient  is  helpless ;  but  it  may  be  less  sudden 
and  not  deprive  him  of  the  power  of  walking  and  of  using  his  hands 
for  two  or  three  months.  It  may  be  arrested  in  the  slighter  cases  and 
not  go  beyond  a  condition  of  general  feebleness  in  the  extremities.  In 
a  few  cases  a  very  acute  onset  is  recorded,  all  the  symptoms  develop- 
ing within  three  or  four  hours. 

The  distribution  of  the  paralysis  is  not  uniform  at  the  outset.  It 
may  begin  in  the  muscles  of  the  legs  and  then  involve  those  of  the 
forearms ;  it  may  commence  in  all  four  extremities  at  once.  It  is 
always  more  severe  in  the  muscles  which  move  the  joints  of  the  feet 
and  hands  and  the  ankles  and  wrists.  It  rarely  invades  those  which 
move  the  knees  and  elbows.  The  extensors  of  the  wrists  and  fingers 
and  the  peronei  and  anterior  tibial  muscles  in  the  legs  are  the  muscles 
first  affected,  and  it  is  usual  for  the  paralysis  to  become  more  complete 
and  to  last  longer  in  these  muscles  than  in  the  flexors  of  the  wrist  or 
in  the  posterior  tibial  muscles  of  the  legs.  A  marked  tremor  in  the 
hands  is  not  at  all  uncommon  in  the  early  stage  of  paralysis,  especially 
in  the  alcoholic  form,  and  usually  the  weakness  is  attended  by  slight 
incoordination  and  unsteadiness  of  gait  before  the  patient  is  finally 
incapacitated  from  walking.  When  the  disease  is  fully  developed  all 
the  muscles  below  the  knees  and  elbows  are  much  weakened  or  totally 
paralyzed.  Occasionally  those  of  the  thighs  and  arms  are  involved 
also,  and  the  muscles  of  the  trunk  and  those  of  respiration  may  become 
affected,  and  then  the  patient  usually  dies. 

In  two  cases  which  eventually  recovered  I  have  seen  a  total 
paralysis  of  the  diaphragm  lasting  several  days.     In  both  these  cases 

^Lowenfeld.     Neurol.  Centralbl.,  1885,  No.  7. 


SYMPTOMS.  201 

the  patient's  respiration  ceased  as  soon  as  he  fell  asleep,  and  the  ex- 
haustion from  the  long  wakefulness  and  the  voluntary  efforts  of  breath- 
ing was  very  great. 

In  some  cases  of  multiple  neuritis  the  cranial  motor  nerves  become 
involved,  those  of  the  eye  and  of  the  face  being  most  liable  to  inva- 
sion.    It  is  only  in  fatal  cases  that  the  action  of  deglutition  has  been 

Fig.  102. 


Dropped  wrists  in  multiple  neuritis. 

affected,  and  when  the  pneumogastric  is  invaded  and  the  heart  be- 
comes rapid  and  irregular  the  prognosis  is  always  grave,  though  not 
absolutely  bad. 

The  paralyzed  muscles  are  relaxed,  flabby,  and  atrophied  ;  they 
may  or  may  not  lose  their  mechanical  irritability,  but  their  normal 
tone  is  always  lost,  and  hence  their  tendon  reflexes  are  abolished. 
The  loss  of  knee-jerk  is  an  early  symptom  in  the  disease,  though  some 
cases  have  been  observed  in  which  it  has  been  preserved.  These  were 
cases  in  which  the  neuritis  did  not  advance  up  the  legs  sufficiently  high 
to  affect  the  nerves  above  the  knee.  The  knee-jerk  always  disappears 
early  in  diphtheritic  cases. 

To  the  electric  current  their  excitability  is  very  rapidly  and  mark- 
edly changed  ;  but  the  conditions  which  have  been  observed  are  quite 
various.  Sometimes  there  is  a  simple  diminution  of  excitability,  and 
then  a  very  strong  faradic  or  galvanic  current  is  needed  to  produce 
contractions.  Frequently  all  faradic  excitability  is  lost,  and  then  the 
muscles  react  to  a  galvanic  current  only.  In  this  condition  it  may  re- 
quire a  very  strong  galvanic  current  to  produce  contraction,  and  this 
fact  is  quite  patli()gnornonic  of  ncui'itis,  for  in  anterior  poliomyelitis, 
where  the  muscles  respond  to  galvanism  only,  it  does  not  require  a 


202 


MULTIPLE  NEVniTlS. 


strong  current  to  cause  a  motion  until  some  months  after  the  invasion. 
The  action  of  the  different  poles  is  not  uniform.  In  many  cases  the 
contraction  of  the  muscle  when  stimulated  with  the  positive  pole  is 
greater  than  when  stimulated  with  the  negative  pole,  and  the  contrac- 
tions may  be  sluggish.  Then  the  reaction  of  degeneration  is  present; 
but  in  some  cases  the  normal  condition  is  found,  and  the  negative  pole 
produces  stronger  contractions  than  the  positive  pole.  If  the  muscles 
which  are  not  paralyzed  be  tested  the  same  electrical  changes  may 
often  be  discovered  in  them.     A  loss  of  faradic  irritability  and  a 

Fig.  103. 


Dropped  feet  in  multiple  neuritis. 

marked  decrease  in  the  galvanic  irritability  of  the  muscle  and  nerve 
are,  therefore,  important  symptoms  of  multiple  neuritis,  and,  as  the 
disease  goes  on  to  recovery,  a  gradual  increase  in  the  galvanic  irrita- 
bility occurs,  a  fact  which  is  often  of  much  aid  in  prognosis  if  care- 
ful measurements  of  the  strength  of  current  used  be  made  by  the 
galvanometer. 

As  a  result  and  accompaniment  of  the  paralysis  abnormal  positions 
are  assumed  by  the  limbs.  The  dropped  wrist  and  dropped  foot  are 
quite  characteristic  of  multiple  neuritis.  (Figs.  102  and  103.)  They 
are  in  part  due  to  the  action  of  gravitation  and  in  part  to  the  unop- 
posed action  of  muscles  which  are  not  very  weak.  But  other  deformi- 
ties may  be  present.  In  a  few  cases  there  have  been  extreme  contrac- 
tures of  all  the  extremities  in  flexed  position.  When  the  legs  are  thus 
flexed  the  posture  is  at  first  voluntarily  assumed  to  relieve  the  pain, 
and  later  the  flexor  muscles  become  permanently  contractured  and 
shortened.  These  contractures  may  be  exceedingly  painful.  In  the 
severe  cases  a  typical  claw-hand  and  talipes  equiuus  are  seen.  These 
deformities  usually  subside  as  the  power  returns,  or,  if  they  do  not, 
they  can  be  corrected  by  proper  manipulation  and  by  apparatus.     In 


SYMPTOMS.  203 

a  few  cases  it  has  been  necessary  to  resort  to  tenotomy,  but  a  perma- 
nent defornyty  has  not  been  recorded. 

The  vasomotor  and  trophic  symptoms  are  less  constant  than  those 
already  described.  In  some  cases  marked  oedema  has  been  an  early 
and  a  permanent  symptom.  This  may  develop  in  the  feet  and  hands, 
or  may  appear  about  the  joints.  It  is  usually  temporary.  The  circu- 
lation is  not  impaired  to  any  greater  degree  than  is  customary  in  a 
limb  whose  muscles  are  inactive,  and  coldness  and  cyanosis  are  rarely 
sufficient  to  attract  attention.  Sometimes  profuse  perspiration  is  a 
noticeable  symptom,  being  limited  to  the  paralyzed  parts.  This  is 
often  seen  in  alcoholic  cases.  It  may  be  oifensive,  and  by  its  evapora- 
tion always  causes  a  complaint  of  coldness.  In  other  cases  glossy  skin 
makes  its  appearance  early  and  remains  until  the  regeneration  of  the 
nerves  is  complete.  Its  disappearance  in  one  of  my  own  cases  was 
the  first  sign  of  recovery  in  the  lower  extremities.  A  change  in  the 
appearance  and  growth  of  the  nails  is  very  common,  and  a  high  ridge 
across  the  nail  becomes  evident  as  recovery  begins,  indicating  the 
difference  between  the  normal  and  abnormal  nail  formations.  This  is 
shown  in  Fig.  104.    Bed-sores  never  appear.     Other  forms  of  trophic 

Fig.  104. 


Ridged  appearance  of  the  nails  in  traumatic  neuritis.    The  hand  is  also  atrophied,  and  the  thenar 
eminence  flat.    The  thumb  cannot  be  opposed  to  the  fingers.    The  scar  over  the  ukiar  nerve  is  visible. 

disturbance  are  rarely  met  with  in  multiple  neuritis,  and  this  is  quite 
remarkable  in  view  of  the  fact  that  it  has  been  the  tendency  of  late  to 
refer  such  trophic  affections  as  ulcerations,  bed-sores,  gangrene,  pem- 
phigus, and  various  eruptions  to  lesions  of  the  nerves.  It  is  true  that 
inflammations  of  the  joints  resembling  those  appearing  in  acute  rheu- 
matism sometimes  occur  at  the  onset  of  neuritis ;  but  as  they  disappear 
quickly  while  other  symptoms  remain,  it  is  improbable  that  they  are 
to  be  traced  to  the  changes  in  the  nerves.  They  may  be  due  to  the 
infectious  agent  or  to  the  same  obscure  cause  which  sets  up  the 
neuritis,  or  they  may  be  evidence  of  an  attack  of  acute  articular 
rheumatism,  which  is  in  turn  followed  by  neuritis,  but  they  cannot  be 
described  as  trophic  symptoms  of  the  disease,  otherwise  they  would 
be  more  constant  in  their  occurrence  and  more  permanent  in  their 
duration. 

A  negative  symptom  of  great  importance  is  the  absence  of  any  inter- 
ference with  the  automatic  action  of  the  bladder  or  rectum.     Retention 


204  MULTIPLE    NEUEiriS. 

or  incontinence  of  urine  does  not  occur,  and  impaction  within  or  a  re- 
laxation of  the  rectum  is  not  observed  in  the  course  of  the  disease. 
Constipation  may  ensue  upon  the  long-continued  rest  in  bed  that  is 
necessitated  by  the  paralysis ;  but  it  is  by  no  means  as  obstinate  or  as 
difficult  to  relieve  as  it  is  in  spinal-cord  aflPections. 

Another  negative  symptom  of  some  importance  is  the  absence  of 
pain  or  of  anaesthesia  upon  the  trunk.  In  the  various  forms  of  spinal- 
cord  disease  which  are  likely  to  be  confounded  with  neuritis,  zones  of 
anaesthesia  upon  the  body  are  not  at  all  uncommon.  It  is  well  known 
that  in  locomotor  ataxia  bands  of  anaesthesia  about  the  trunk  and  in 
the  axillae  are  almost  constant  signs  of  the  affection.  It  is  well  to  rec- 
ognize the  absence  of  this  symptom  in  those  cases  of  multiple  neuritis 
which  simulate  locomotor  ataxia. 

There  are  certain  mental  symptoms  which  develop  during  the  course 
of  alcoholic  multiple  neuritis,  but  as  they  do  not  appear  in  the  other 
types  they  will  be  discussed  in  connection  with  the  toxic  cases. 

Course.  —  The  course  of  the  disease  in  multiple  neuritis  varies  so 
exceedingly  that  no  general  statement  can  be  made.  In  the  next  chap- 
ters in  which  the  various  forms  of  the  affection  are  considered  the 
course  of  the  case  in  each  form  will  be  described.  General  constitu- 
tional disturbances,  however,  are  not  infrequent  in  the  course  of  mul- 
tiple neuritis,  and  may  be  mentioned  here.  The  onset  is  often  sudden 
and  accompanied  by  a  marked  febrile  movement  with  chill  and  tempera- 
ture of  103°  or  104°  F.  The  fever  may  persist  for  several  days,  but 
usually  subsides  spontaneously  and  does  not  recur.  In  a  few  cases 
there  has  been  a  constant  elevation  of  temperature  of  about  one  degree, 
persisting  for  several  weeks.  The  general  symptoms  accompanying 
fever,  viz.,  malaise,  loss  of  appetite,  nausea,  disturbances  of  digestion, 
constipation,  flatulence,  occasional  diarrhoea,  febrile  condition  of  the 
urine,  general  pains  all  over  the  body,  headache,  and  discomfort 
usually  pass  off  as  this  subsides.  An  enlargement  of  the  spleen  and 
a  condition  of  leucocytosis  has  been  discovered  by  examination  during 
the  onset  in  some  cases,  especially  in  those  following  an  infectious 
disease  and  in  cases  that  are  not  due  to  poisoning.  These  facts  have 
led  to  the  theory  that  neuritis  may  be,  under  some  circumstances,  a 
primary  infectious  disease. 

Increased  rapidity  of  the  pulse  is  very  common  in  all  forms  of 
neuritis,  and  may  persist  during  the  entire  course  of  the  disease,  the 
pulse  ranging  from  80  to  100.  In  some  cases  it  becomes  exceedingly 
rapid,  reaching  140  or  160,  being  feeble  and  small.  In  these  cases 
the  neuritis  has  extended  to  the  pneumogastric  nerve.  If  this  condi- 
tion persists  for  several  days  oedema  of  the  extremities  and  finally  of 
the  lungs  may  result,  and  heart  failure  may  be  the  primary  cause  of 
death. 

The  duration  of  a  condition  of  multiple  neuritis  is  so  different  in  dif- 
ferent types  of  cases  that  no  general  statement  is  warranted.  In  the 
diphtheritic  cases  recovery  is  quite  rapid,  and  I  have  seen  patients  who 
had  been  completely  paralyzed  quite  well  within  two  months.     In 


DIAGNOSIS.  205 

cases  of  alcoholic  neuritis  of  a  mild  type,  where  no  actual  paralysis 
developed,  r^overy  was  usually  complete  in  six  months.  In  the  severer 
cases,  where  drop-foot  and  drop-wrist  had  developed,  at  least  a  year, 
and  in  several  cases  two  years  elapsed  before  their  health  and  power 
were  restored.  Arsenic  and  lead  cases  are  also  slow  in  their  progress, 
from  eight  to  twelve  months  being  the  usual  duration.  The  duration 
is  in  exact  ratio  with  the  degree  of  degeneration  present  at  the  maxi- 
mum of  the  symptoms. 

Diagnosis.  —  While  the  individual  symptoms  occurring  in  the  course 
of  multiple  neuritis  are  not  different  in  character  from  those  found  in 
spinal-cord  diseases,  the  diagnosis  can  usually  be  reached  with  very 
little  difficulty  when  their  combination,  the  causation,  and  the  course 
of  the  case  under  examination  are  considered.  There  are  three  com- 
binations of  symptoms  in  neuritis  which  resemble  very  closely,  respec- 
tively, anterior  poliomyelitis,  locomotor  ataxia,  and  diffuse  myelitis, 
and  to  these  attention  must  be  directed. 

Atrophic  paralysis,  with  reaction  of  degeneration  and  loss  of  reflexes, 
is  common  to  anterior  poliomyelitis  and  some  cases  of  multiple  neu- 
ritis. Poliomyelitis  attacks  healthy  children,  and  is  known  to  be  due 
to  an  infection  in  many  cases.  In  neuritis  it  is  often  possible  to  ascer- 
tain some  previous  condition  of  ill  health  or  some  infectious  disease  or 
constitutional  state  which  has  produced  the  affection.  In  neuritis,  a 
more  gradual  onset,  preceded  and  attended  by  numbness  and  pain, 
tenderness  in  the  course  of  the  nerves,  tenderness  in  the  muscles,  and 
the  persistence  of  sensory  symptoms  after  the  invasion  will  remove  all 
doubt  regarding  the  diagnosis.  When  these  symptoms  are  not  clearly 
marked  the  distribution  of  the  paralysis  in  symmetrically  situated 
muscles,  especially  if  these  muscles  are  supplied  by  single  nerves,  and 
the  further  extension  to  muscles  in  other  nerve  domains,  rather  than  the 
affection  simultaneously  of  muscles  which  are  grouped  physiologically 
{i.  e.,  act  together  to  perform  one  function)  will  point  to  neuritis.  In 
neuritis  the  paralysis  advances  more  or  less  gradually,  while  in  acute 
poliomyelitis  there  is,  after  the  onset,  a  subsidence  of  the  paralysis  in 
some  of  the  muscles  first  involved.  Ataxia,  which  is  a  common  symp- 
tom in  neuritis,  never  appears  in  infantile  paralysis.  Cramps  in  the 
muscles  are  complained  of  in  neuritis,  but  not  in  poliomyelitis.  In  the 
latter  fibrillary  twitchings  occur  in  the  muscles  which  are  paralyzed, 
but  never  in  the  former.  Glossy  skin  never  appears  in  poliomyelitis. 
Lastly,  as  the  case  goes  on,  a  gradual  complete  recovery  will  be  far 
more  frequent  if  it  was  originally  a  case  of  multiple  neuritis.  Gowers 
has  described  a  number  of  cases  in  which,  he  believes,  the  two  diseases 
have  occurred  together,  probably  being  produced  by  the  same  cause. 
This  has  been  seen  also  in  epidemics  of  infantile  paralysis.  Under 
these  circumstances  a  mingling  of  the  symptoms  is  to  be  expected,  and 
no  sharp  differentiation  can  be  made. 

Ataxia,  loss  of  knee-jerk,  pain,  and  sensory  disturbances,  including 
a  loss  of  muscular  sense,  Romberg's  symptom,  that  is,  swaying  when 


206  MULTIPLE   NEUBITIS. 

standing  with  closed  eyes,  and  optic  neuritis,  are  common  to  locomotor 
ataxia  and  to  multiple  neuritis.  This  form  of  neuritis  has  been  called 
acute  polyneuritic  ataxia  or  neurotabes  peripherica  by  Dejerine/  who 
was  the  first  to  point  out  clearly  its  resemblance  to  locomotor  ataxia. 
In  neuritis  the  relatively  rapid  onset  of  the  ataxia,  which  follows 
closely  upon  tlie  sensory  symptoms ;  the  prominence  of  numbness  and 
anaesthesia,  rather  than  of  lightning  pains ;  the  extreme  degree  of  the 
anaesthesia  and  analgesia,  the  tenderness  of  muscles  and  nerves,  neven 
found  in  tabes,  the  usual  occurrence  of  some  degree  of  actual  paresis, 
with  atrophy  and  reaction  of  degeneration,  and  the  absence  of  bladder 
and  sexual  symptoms,  will  point  inevitably  to  the  correct  diagnosis. 
Furthermore,  the  ataxic  form  of  neuritis  only  occurs  after  poisoning 
with  alcohol  or  arsenic,  or  as  a  sequel  of  diphtheria,  and  the  establish- 
ment of  the  causation  will  aid  the  diagnosis.  Here  again,  the  course  of 
the  case  toward  recovery  and  the  return  of  the  knee-jerk  will  decide  in 
favor  of  neuritis  if  the  diagnosis  has  not  been  reached  in  an  early  stage. 
There  are  very  few  symptoms  of  diffuse  myelitis  which  are  not 
found  in  cases  of  neuritis,  but  cases  of  diffuse  myelitis  of  the  type  de- 
scribed by  Duchenne  (paralysie  generate  spinale  subdigue  ascendante) 
are  very  rare,  and,  indeed,  it  has  been  affirmed  -by  Leyden  that  the 
cases  described  by  Duchenne  under  this  name  were  really  cases  of 
multiple  neuritis.  A  differential  diagnosis  between  general  myelitis 
and  neuritis  is  made  by  a  consideration  of  the  following  points  :  In 
neuritis  affections  of  the  functions  of  micturition  and  defecation  do  not 
occur.  Girdle  sensation  is  very  rarely  mentioned  as  a  symptom. 
Bed-sores  and  cystitis  have  not  been  observed.  In  neuritis  the  advance 
of  the  paralysis  is  not  like  that  in  myelitis,  namely,  a  gradual  advance 
from  legs  to  thighs,  and  thighs  to  trunk,  and  trunk  to  arms  and  neck  ; 
but,  as  already  stated,  the  paralysis  begins  in  the  legs  and  forearms 
simultaneously,  and  does  not  usually  extend  to  the  thighs  and  arms 
and  very  seldom  invades  the  trunk.  If  the  muscles  of  the  abdomen 
and  the  muscles  of  respiration  are  involved  in  neuritis  it  is  only  in  the 
rapidly  fatal  toxic  cases,  and  even  in  these  cases  motions  of  the  shoul- 
ders and  hips  are  preserved  until  the  end.  In  the  type  of  myelitis 
described  by  Duchenne  there  are  few  sensory  symptoms,  whereas  these 
are  prominent  in  neuritis.  If  in  myelitis  there  are  sensory  distur- 
bances and  anaesthesia  the  areas  are  not  glove-shaped  and  stocking- 
shaped  and  there  is  a  well-marked  line  of  demarcation  around  the 
trunk.  This  is  particularly  evident  in  transverse  myelitis.  In  neu- 
ritis, however,  the  anaesthesia  is  chiefly  observed  in  the  hands  and  feet, 
in  the  forearms  and  legs,  but  rarely  reaches  as  high  as  the  arms  or 
thighs.  In  neuritis  there  is  usually  tenderness  in  the  nerves  and  in 
the  muscles  of  the  extremities  which  is  not  present  in  myelitis.  In 
myelitis  there  is  usually  tenderness  to  pressure  and  sensitiveness  to 
heat  along  the  spine,  a  symptom  not  present  in  neuritis.  In  the  older 
text-books  multiple  neuritis  is  not  mentioned,  and  cases  of  it  were  de- 
scribed as  cases  of  spinal-cord  disease ;  but  since  the  knowledge  of 

^  Arch.  phys.  norm,  et  path.,  1884,  p.  231. 


TREATMENT.  207 

neuritis  lias  become  general  the  diagnosis  of  myelitis  is  more  and  more 
rarely  made/ and  now  that  disease  is  regarded  as  an  unusual  one. 

Prognosis.  —  The  prognosis  in  multiple  neuritis  is  good,  provided 
the  exciting  cause  can  be  removed.  The  only  patients  who  form  an 
exception  to  the  rule  are  those  whose  constitutions  are  much  impaired 
by  excesses  or  by  other  diseases ;  those  who  have  so  far  indulged  in 
alcohol  or  are  so  completely  soaked  with  arsenic  or  lead  as  to  be  unable 
to  throw  off  the  poison,  and  those  in  whom  the  disease  begins  with 
great  suddenness,  advances  rapidly,  and  involves  the  phrenic  and  pneu- 
mogastric  nerves.  These  cases  die  either  of  respiratory  paralysis  or 
of  some  complication.  When  a  case  has  reached  the  stationary  period 
the  prognosis  is  generally  favorable,  and  if  the  encouraging  signs  of 
recovery  already  mentioned  begin  to  appear  a  cure  may  be  promised. 
The  possibility  of  the  complication  of  myelitis  must  not,  however,  be 
overlooked,  and  if  it  occurs  the  prognosis  becomes  at  once  unfavorable. 
Even  in  serious  cases  of  alcoholism,  with  gastro-intestinal  and  cerebral 
symptoms,  if  the  acute  stage  be  safely  passed  and  all  alcohol  be  re- 
moved from  the  patient's  diet,  recovery  from  very  extensive  paralysis 
will  occur.  The  cases  of  diphtheritic  paralysis  and  ataxia  usually  re- 
cover without  treatment. 

Treatment.  —  The  treatment  of  multiple  neuritis  requires  patience. 
As  we  have  already  seen,  the  majority  of  the  patients  recover,  and  it 
is  probable  that,  if  the  cause  of  the  affection  were  removed  and  the 
patients  placed  in  favorable  circumstances,  expectant  treatment  would 
alone  be  sufficient.  It  is,  however,  not  advisable  to  let  therapeutics 
play  a  passive  part.  The  course  of  the  disease  can  be  altered  and  its 
duration  much  shortened  by  active  interference.  In  the  stage  of  in- 
vasion the  free  use  of  aspirin,  salophen,  salicin,  salicylic  acid,  or  the 
salicylate  of  soda  seems  to  have  important  results.  These  remedies 
cannot  be  said  to  act  as  promptly  as  in  cases  of  acute  articular  rheu- 
matism, but  the  consensus  of  opinion  is  that  their  effect  in  multiple 
neuritis  is  very  marked.  They  should  be  given,  as  in  acute  rheumatic 
fever,  in  large  doses  until  noticeable  effects  are  obtained.  They  should 
be  combined  with  the  bromide  of  potassium  or  sodium,  partly  because 
these  drugs  counteract  unfavorable  symptoms  produced  by  the  salicin 
compounds  and  partly  because  in  the  hypersesthetic  irritable  condition 
attendant  upon  the  invasion  of  the  disease  they  are  indicated.  This 
condition  may  require  stronger  sedatives  and  not  infrequently  morphine 
must  be  employed  to  give  relief  from  the  excruciating  pains.  The 
pains  are  often  relieved  by  hot  or  cold  applications  to  the  limbs  ;  but 
as  the  muscles  are  often  exceedingly  tender,  ordinary  applications  can- 
not be  made.  It  is  then  advisable  to  use  evaporating  lotions,  prefer- 
ably those  containing  chloroform,  which  may  be  soaked  into  light 
cambric  or  gauze  and  gently  placed  upon  the  limbs,  which  lie  upon  the 
softest  pillows  or  which  may  be  more  comfortable  if  the  patient  is  put 
upon  a  water-bed.  Applications  of  a  5  per  cent,  solution  of  carbolic 
acid  and  of  extract  of  witch-hazel  have  also  been  of  use.  If  cool 
applications  prove  intolerable  heat  may  be  employed.     The  limbs  may 


208  MULTIPLE   NEUBITIS. 

be  enveloped  with  cotton  and  covered  with  oiled  silk,  a  light  bandage 
keeping  these  in  place,  or  they  may  be  frequently  bathed  in  hot  water 
and  hot  bottles  placed  against  them,  some  soft  substance  intervening. 
One  of  niy  patients  found  great  relief  from  the  parsesthesia  by  cold 
douches,  while  another  preferred  the  use  of  hot  water.  It  is  best  to 
let  the  patient  decide,  as  long  as  the  application  has  to  be  made  for  the 
relief  of  pain.  Gentle  friction  with  oil  of  cocoanut  or  cocoa-butter 
often  affords  comfort.  In  the  chronic  stage,  as  we  shall  see  presently, 
heat  is  to  be  preferred  to  cold.  Cases  which  are  distinctly  syphilitic, 
if  such  occur,  should  be  treated  from  the  outset  with  inunctions  of 
mercury  and  large  doses  of  iodide  of  potash.  I  believe  that  both  these 
drugs  should  be  employed  together,  even  in  the  tertiary  stage  of 
syphilis,  and  it  is  my  experience  that  all  syphilitic  nervous  lesions, 
whether  central  or  peripheral,  yield  more  promptly  to  their  combined 
use  than  to  the  employment  of  either  alone.  Malarial  cases  must  be 
treated  with  quinine  or  Warburg's  tincture.  In  non-malarial  cases 
quinine  has  proved  of  no  avail. 

In  cases  which  are  due  to  poisoning  of  any  kind  the  first  indica- 
tion is  to  eliminate  the  toxic  agent  from  the  system  and  the  second  to 
prevent  any  further  ingestion  of  the  poison.  Iodide  of  potash  in  small 
doses,  largely  diluted,  aids  in  the  elimination.  The  second  indication 
is  easily  fulfilled  when  arsenic  or  lead  are  the  toxic  agents,  but  when 
the  case  is  due  to  chronic  alcoholism  special  precautions  are  needed. 
Alcoholic  cases  require  from  the  outset  special  treatment.  The  condi- 
tion at  the  time  of  the  onset  of  the  paralysis  may  be  one  verging  upon 
delirium  tremens.  If  all  alcohol  is  suddenly  removed,  without  due 
care  to  supply  some  other  heart  stimulant  and  to  secure  the  perfect 
nutrition  of  the  patient,  serious  collapse  may  ensue.  The  first  neces- 
sity is  therefore  to  take  care  of  the  general  condition  of  the  patient. 
If  this  will  admit  of  the  immediate  withdrawal  of  all  alcoholic  stimu- 
lation it  should  be  done ;  if  not,  the  alcoholic  beverage  must  be  imme- 
diately reduced  in  quantity,  and  as  soon  as  possible  wholly  cut  off. 
The  use  of  milk  diet,  or  kumyss,  or  peptonized  milk,  or,  if  necessary, 
rectal  alimentation,  will  be  followed  by  a  gradual  recovery  of  the 
power  of  assimilation,  and  as  soon  as  the  patient  ceases  to  lose  weight 
all  alcohol  may  in  any  case  be  safely  stopped ;  its  elimination  by  the 
intestines  and  kidneys  may  be  hastened  by  appropriate  means,  and 
cerebral  symptoms,  if  they  arise,  may  be  treated  as  in  other  cases  of 
alcoholic  intoxication.  But  it  is  in  the  chronic  stage,  when  the  patient 
is  gradually  recovering,  that  the  vigilance  of  the  physician  is  called 
into  play  to  prevent  a  renewal  of  the  poisoning.  It  is  amazing  that 
patients  who  know  perfectly  the  injurious  effect  of  alcohol  upon  them 
should  insist  upon  getting  it ;  but  it  is  done.  And  when  these  patients 
are  surrounded,  as  is  often  the  case,  by  sympathizing  friends  or  servile 
domestics  or  unscrupulous  nurses  who  ,do  not  appreciate  the  impor- 
tance of  total  abstinence  for  the  patient,  they  often  succeed  in  baffling 
all  attempts  to  deprive  them  of  the  favorite  drink.  It  is  only  when 
they  are  watched  constantly  by  persons  who  can  be  implicitly  trusted 


TREATMENT.  209 

and  who  have  sufficient  authority  to  cut  off  all  surreptitious  supplies, 
that  the  physician  can  feel  sure  that  his  conunands  are  obeyed;  and 
this  precaution  is  by  no  means  needless,  even  when  it  is  probable  that 
family  servants  are  trustworthy,  for  the  continued  pleading  and  remon- 
strance of  the  patients  may  corrupt  the  best  of  attendants,  especially  if 
accompanied  by  threats  of  discharge  at  a  future  day.  It  is,  therefore, 
necessary  to  place  these  patients  under  the  surveillance  of  trained 
nurses  from  the  start  or  to  remove  them  to  an  institution  where  they 
will  be  under  control. 

In  the  chronic  stage  the  drugs  which  are  of  greatest  service  are 
strychnine  and  arsenic.  Strychnine  may  be  given  in  doses  of  ^\  to 
^  gr.  three  times  daily,  and  it  is  well  to  combine  it  with  phosphoric 
acid  and  the  syrup  of  the  hypophosphites,  or  with  the  glycerophos- 
phates of  lime  and  soda.  Arsenious  acid  may  be  used  in  tablets  or 
pills  containing  gV  ^*^  w  S^'  three  times  daily,  or  in  Fowler's  solution, 
three  to  five  drops,  three  times  daily.  Hypodermic  injections  of  the 
cacodylate  of  soda  are  of  use.  The  use  of  iron  will  be  indicated  in  the 
majority  of  cases  where  there  is  attendant  anaemia.  In  alcoholic  cases 
both  arsenic  and  strychnine  may  increase  the  mental  irritability,  but 
should  be  continued  unless  this  becomes  too  great.  I  have  seen  benefit 
from  both  of  these  drugs,  and  think  it  well  to  employ  then  alternately, 
using  each  for  about  two  weeks  at  a  time.  The  glycero-phosphate  of 
soda  dissolved  in  water  is  of  much  service  in  the  chronic  stage.  The 
dose  is  15  grains  three  times  daily. 

The  remedies  used  in  the  chronic  stage  have  two  objects :  one  is  to 
increase  the  rate  of  repair  in  the  nerves,  the  other  is  to  keep  the  nutri- 
tion of  the  muscles  as  good  as  possible.  While  the  drugs  mentioned 
probably  meet  the  first  indication,  there  are  other  remedies  which  meet 
both.  These  are  massage,  warm  baths,  and  electricity.  The  proper 
manipulation  of  the  limb  increases  the  circulation  in  it.  The  increase 
of  circulation  brings  fresh  supplies  of  material  to  the  nerve  which  is 
undergoing  repair ;  it  also  aids  the  nutrition  of  the  muscle,  which 
would  otherwise  be  decidedly  affected  by  the  sluggish  flow  of  venous 
blood,  due  to  the  lack  of  functional  activity.  As  soon,  therefore,  as 
the  active  progress  of  the  disease  is  checked  and  the  muscular  tender- 
ness has  sufficiently  subsided  to  allow  the  limbs  to  be  rubbed,  this 
remedy  should  be  employed  daily. 

Allusion  has  already  been  made  to  baths  and  douches  in  the  early 
stage,  for  the  purpose  of  quieting  sensory  symptoms.  In  the  chronic 
stage  the  object  is  a  different  one.  Like  massage,  warm  baths,  packs, 
and  douches  stimulate  the  circulation  and  aid  the  nutrition  and  repara- 
tive processes  in  progress.  Hence  they  are  to  be  used  daily  ;  and  if 
the  warm  bath  or  pack  be  given  at  night  it  will  secure  not  only  a  local 
action,  but  produce  a  general  sedative  effect,  insuring  quiet  rest.  In 
multiple  neuritis,  as  well  as  in  many  other  nervous  affections  not  due 
to  anaemia,  a  warm  bath  or  pack  at  night  or  a  warm  douche  to  the  neck 
and  spine,  is  far  preferable  to  and  more  efficacious  than  the  majority 
of  hypnotic  drugs  in  causing  a  good  night's  sleep. 
14 


210  MULTIPLE   NEUlilTIS. 

The  last  agent  to  be  mentioned  is  electricity.  It  is,  however,  of 
some  importance,  but  it  is  necessary  to  know  the  object  sought  in  its 
application.  There  is  first  the  object  of  increasing  'the  progress  of 
nerve  regeneration.  This  is  said  to  be  attained  by  the  application  of  a 
constant  galvanic  current  to  the  degenerated  nerve,  passing  the  cur- 
rent through  the  nerve  in  either  direction,  or  in  both  alternately.  A 
mild  current  should  be  employed,  its  strength  being  measured  by  a 
galvanometer.  The  strength  of  the  current  will  depend  (a)  on  the 
size  of  the  sponges  placed  upon  the  skin,  (6)  on  the  pressure  upon  the 
sponges,  (c)  on  the  resistance  of  the  skin,  (d^  on  the  number  of  cells 
of  the  battery  used. 

If  the  sponges  are  two  inches  in  diameter,  six  milliamperes  are 
enough,  if  they  are  three  by  five  inches  in  measurement,  twenty  mil- 
liamperes should  not  be  exceeded.  If  no  galvanometer  is  used  the 
strength  of  the  current  employed  is  uncertain ;  but  it  is  to  be  remem- 
bered that  the  current  grows  stronger  the  longer  it  passes,  since  the  skin 
resistance  is  gradually  overcome ;  and,  therefore,  if  the  strength  is 
measured  in  cells,  the  number  of  cells  used  should  be  decreased  gradu- 
ally during  the  application.  As  so  few  practitioners  use  a  galvanome- 
ter, it  may  be  well  to  state  that  with  large  sponges — i.  e.,  three  by 
five  inches — wet  with  warm  water,  a  freshly  filled  bichromate  of  pot- 
ash battery  will  give  nearly  one  milliampere  of  strength  for  every  cell 
used  during  the  first  three  minutes,  provided  the  sponges  be  put  on 
any  part  of  the  body  except  the  soles  of  the  feet  or  the  palms  of  the 
hands,  and  pressed  firmly  upon  the  skin.  After  the  first  three  min- 
utes the  body  resistance  decreases,  so  that  when  twenty  cells  are  used 
to  start  with,  one  should  be  cut  oif  every  half-minute  until  the  num- 
ber is  reduced  one-third.  The  duration  of  the  application  should  be 
about  ten  minutes  to  each  limb.  During  this  time  the  distal  sponge 
should  be  passed  over  various  parts,  so  as  to  include  all  the  nerve 
branches  in  the  current ;  the  central  sponge  should  be  put  over  the 
nerve  trunk  high  up  on  the  limb.  The  current  should  be  begun  and 
stopped  gradually,  and  never  suddenly  broken.  Applications  may  be 
made  daily. 

The  second  object  to  be  obtained  is  to  reestablish  the  conduction  of 
impulses  in  the  regenerated  nerve.  This  is  secured  by  the  method  just 
described.  It  may  be  attained  by  the  use  of  faradism,  the  interrupted 
secondary  current  being  sent  along  the  nerves  by  placing  one  pole  over 
the  nerve  trunk  and  passing  the  other  over  the  skin  of  the  limb.  The 
strength  used  should  be  just  sufficient  to  be  felt  distinctly  through  the 
palms  of  the  operator's  hands.  The  third  object  sought  is  to  maintain 
the  nutrition  and  function  of  the  muscles  by  exercising  them,  and  thus 
to  preserve  their  normal  irritability.  This  cannot  be  done  by  a  far- 
adic  current  as  long  as  they  do  not  contract  to  it.  The  galvanic 
current  must,  therefore,  be  employed.  But  now  it  is  not  a  steady  cur- 
rent which  is  needed,  for  this  does  not  cause  any  motion.  It  is  only 
when  the  steady  current  is  suddenly  broken  and  renewed  that  the  con- 
traction occurs..     Hence,  place  one  pole  over  the  trunk  of  the  nerve 


TREATMENT.  211 

and  the  other  upon  the  muscle,  and  with  an  interrupting  electrode 
make  and  break  the  current  at  the  pole  which  is  on  the  muscle.  The 
pole  which  ■^'produces  a  'contraction  with  the  least  current  possible  is 
the  one  to  be  applied  to  the  muscle.  This  is  in  the  reaction  of  degen- 
eration, the  positive ;  in  normal  conditions,  the  negative  pole.  Each 
muscle  should  be  exercised  for  three  or  four  minutes  every  other  day. 
When  electrical  treatment  is  thus  employed  very  marked  improvement 
is  observed,  which  can  be  measured  accurately  if  a  galvanometer  is 
used,  since  every  week  will  show  a  change  of  the  strength  of  current 
needed  to  produce  muscular  contractions  toward  the  normal. 

The  treatment  must  be  kept  up,  in  the  chronic  stage,  until  recovery 
is  complete. 

If  contractures  have  occurred  in  the  paralyzed  limbs,  persistent 
massage  may  overcome  them.  If  it  does  not,  they  are  to  be  treated  on 
general  surgical  principles.  When  the  posterior  tibial  muscles  are 
contractured,  the  patients  cannot  stand  because  the  heels  cannot  be 
placed  on  the  floor.  In  this  condition  shoes  with  very  high  rubber 
heels,  and  either  corset-like  ankles  or  braces  to  support  the  ankle,  may 
be  fitted,  and  thus  the  patient  may  be  enabled  to  walk  with  a  little 
help  long  before  he  can  stand  in  his  bare  feet.  Sometimes  division  of 
the  Achilles  tendon  is  necessary  to  restore  the  power  of  walking. 


CHAPTER   IX. 

THE   TOXIC   FORMS   OF   MULTIPLE   NEURITIS. 

Alcoholic  Neuritis.  Multiple  Neuritis  from  Poisoning  by  Illuminating  Gas.  Multiple 
Neuritis  from  Poisoning  by  Sulphide  of  Copper.  Multiple  Neuritis  from  Poisoning 
by  the  Coal-tar  Products.  Arsenical  Neuritis.  Multiple  Neuritis  Due  to  Poisoning 
by  Lead.  Multiple  Neuritis  Due  to  Poisoning  by  Phosphorus,  Mercury,  Silver, 
and  Copper. 

ALCOHOLIC  NEURITIS. 

Historical.  —  Alcoholic  neuritis  is  the  most  common  of  all  the  toxic 
forms  of  multiple  neuritis.  The  credit  of  the  discovery  of  alcoholic 
paralysis  must  be  awarded  to  James  Jackson,  of  Boston,  who,  in  1822, 
gave  a  most  accurate  description  of  the  disease  and  ascribed  it  to  the 
use  of  ardent  spirits.^  Magnus  Huss,  in  1852,  published  an  extensive 
work  on  chronic  alcoholism,  in  which  he  gave  a  very  complete  account 
of  the  nervous  symptoms  produced  by  the  abuse  of  liquor,  dividing  the 
cases  into  paralytic,  ansesthetic,  convulsive,  epileptic,  and  hypersesthetic 
forms.  But  he  ascribed  all  the  symptoms  to  lesions  of  the  central 
nervous  system.  In  1855  Duchenne  de  Boulogne,  in  his  epoch-mak- 
ing book,  Electrisation  Localisee,  recorded  many  cases  which  we  now 
recognize  as  cases  of  neuritis;  but  he,  too,  supposed  them  to  be  of 
spinal  origin.  Lancereaux  added  further  to  the  knowledge  of  alco- 
holic paralysis  in  an  article  on  alcoholism  in  the  Dictionnaire  Ency- 
dopcedique  des  Sciences  Medicates  in  1864,  and  in  the  same  year 
DumesniP  published  the  first  case  in  which  a  lesion  was  found  in  the 
peripheral  nerves.  But  ten  years  then  elapsed  before  his  observations 
were  confirmed  by  Eichhorst,^  and  it  required  further  evidence  fur- 
nished by  JoflFroy,*  by  Leyden,^  and  by  others  to  establish  the  relation 
between  alcoholic  paralysis  and  neuritis.  It  is  to  Lancereaux,^  of 
Paris,  to  Moeli/  of  Berlin,  to  Dreschfeld,^  of  Manchester,  England,  to 
Henry  Hun,^  of  Albany,  N.  Y.,  and  to  Bernhardt,^''  of  Berlin,  that  we 
owe  our  knowledge  of  the  pathology  and  symptomatology  of  alcoholic 
multiple  neuritis,  though    other  and   subsequent  writers  have  added 

^New  England  Journal  of  Medicine  and  Surgery,  1822,  vol.  xi.,  p.  351,  "On  a 
Peculiar  Disease  Resulting  from  the  Use  of  Ardent  Spirits." 
''Gaz.  Heb.  de  Paris,  1864,  p.  203,  and  1866,  No.  4. 
»Virchow's  Archiv,  1876,  Bd.  69,  p.  205. 
*Arch.  de  phys.  norm,  et  path.,  1879,  p.  172. 
^Charite  Annalen,  1880,  Zeitschr.  f.  klin.  Med.,  1880. 
*De  la  Paralysie  Alcoolique,  Gaz.  Heb.  de  Paris,  1881,  p.  120. 
'  Brain,  Nos.  26  and  32. 
8Moeli.     Charitd  Annalen,  1884. 

®  American  Journal  of  the  Medical  Sciences,  April,  1885. 
"Zeitschr.  f.  klin.  Med.,  1886. 

212 


PLATE  XVI 


m  te 


liMM 


Longitudinal  Section  of  Tibial  Nerve  in  a  Case  of  Alcoholic  Neuritis. 
(Osmic  acid  and  carmine  stain.) 

Tlie  degenerated  nerve  fibres  are  stained  black ;  the  thickened  interstitial 
connective  tissue  is  stained  red. 

(Kla.tau,  Spec:.  Pathol,  u.  Therap.,  Nothnagel,  Bd.  xi.,  Taf.  vii.') 


ALCOHOLIC    NEURITI8.  213 

valuable  facts  and  have  made  the  picture  of  the  condition  complete. 
James  Rossf  Brissaud,^' Pal/  and  Growers*  may  be  mentioned  as  hav- 
ing made  interesting  additions  to  the  general  knowledge  of  the  affec- 
tion, and  as  having  clearly  presented  its  differential  diagnosis  from 
other  diseases.  The  most  complete  monograph  on  the  subject  is  by 
Remak  and  Flatau  in  Nothnagel's  System  of  Medieiiie,  1900. 

Pathology.  —  All  forms  of  neuritis  have  been  found  in  alcoholic 
cases.  In  the  majority  of  cases  a  parenchymatous  neuritis  with  simple 
degeneration  and  atrophy  is  the  only  lesion  in  the  nerves.  (Plate 
XII.)  In  other  cases  a  diffuse  inflammation  is  present,  with  an  in- 
crease of  connective  tissue  in  the  endoneurium  and  perineurium,  and  a 
marked  congestion  of  the  bloodvessels,  as  well  as  a  degeneration  of  the 
nerve  fibres.  All  the  characteristic  changes  already  described  as  oc- 
curring in  local  neuritis  are  present  in  alcoholic  cases.  But  it  is  not 
possible  to  distinguish  clinically  between  cases  in  which  the  lesion  is  a 
simple  degeneration,  and  cases  in  which  there  is  a  diffuse  inflammation. 
The  lesion  is  more  marked  in  the  finer  branches  of  the  nerves,  in  the 
skin  and  muscles,  and  becomes  less  intense  the  higher  up  the  nerve  is 
examined.  In  the  plexuses  no  change  may  be  found,  when  at  the 
same  time  few  normal  fibres  can  be  discovered  below  the  wrists  and 
ankles.  Certain  nerves  seem  particularly  susceptible  to  the  influence 
of  alcohol.  The  degeneration  begins  in  the  peronei,  the  tibials,  then 
in  the  radial,  median,  and  ulnar  nerves,  and  is  more  intense  in  these 
than  elsewhere.  (See  Plate  XVI.)  The  sciatic  and  crural  and  the 
musculocutaneous  and  circumflex  are  but  slightly  affected.  Lesions 
have  been  found  in  the  phrenic,  in  the  pneumogastric,  and  in  the  optic 
nerves.  Normal  fibres  are  found  here  and  there  in  the  nerves  which 
are  severely  affected.  A  thickening  of  the  bloodvessels  in  the  nerves 
has  been  discovered  in  some  cases,  with  a  small-cell  infiltration  of  the 
walls,  and  occasional  rupture,  with  capillary  hemorrhages. 

Recent  observation  establishes  the  fact  that  while  the  principal 
changes  in  alcoholic  paralysis  are  found  in  the  peripheral  branches  of 
the  nerves  in  all  cases,  yet  in  some  cases  changes  in  the  cells  of  the 
central  nervous  system  are  present.  These  have  been  discovered  in 
the  cells  of  the  anterior  horns  of  the  spinal  cord  and  in  the  posterior 
spinal  ganglia.^  It  is  evident,  therefore,  that  while  the  poison  circulat- 
ing in  the  blood  produces  a  destructive  process  in  the  delicate  fila- 
ments of  nerve  fibres  in  the  periphery,  yet  the  central  nervous  system 
is  by  no  means  free  from  its  action.  The  cells  of  the  spinal  cord  are 
less  affected  by  the  poisoning  than  the  more  highly  organized  and  de- 
veloped cells  of  the  brain.  The  changes  in  the  cord  cells  consist  of  a 
change  in  the  Nissl  granules,  which  become  finely  granular,  and  lose, 
finally,  their  power  of  absorbing  anilin  dye.  This  change  is  more 
marked  about  the  nucleus  and  in  the  centre  of  the  cell  body  which  has 

'.James  Eoss  and  Judson  Bury.     "On  Peripheral  Neuritis,"  1889. 

^  Briasaud.     Les  Paralysies  Toxiques,  Paris,  1890. 

8Pal.     "Ucber  Neuritis,"  Berlin,  1892. 

*Gower8.     Text-hook  of  Nervous  Diseases,  1892,  2d  ed. 

^Larkin  and  .Jell i fie.     N.  Y.  Med.  Record,  .July  8,  1899. 


214  TEE    TOXIC   FORMS    OF   MULTIPLE    NEURITIS. 

a  pale  appearance.  (See  Plate  II,  g.)  There  is  often  a  deposit  of  pig- 
ment in  the  cells.  As  these  changes,  however,  have  been  found  in  all 
the  cells  of  the  cord,  even  in  those  connected  with  muscles  which  were 
not  paralyzed,  and  as  they  resemble  closely  the  changes  known  to  occur 
after  division  of  a  nerve,  they  are  not  to  be  regarded  as  the  cause  of 
alcoholic  paralysis.  Heilbronner  ^  has  found  an  ascending  degeneration 
in  the  posterior  columns  of  the  spinal  cord  in  an  advanced  case  of 
alcoholic  neuritis,  and  is  inclined  to  trace  it  to  extensive  degeneration 
in  the  posterior  spinal  neurones,  but  this  is  certainly  an  exceptional 
case. 

Berkeley,  Andriesen  and  Van  Gieson  have  demonstrated  a  uniform 
pathological  condition  produced  in  the  large  cortical  cells  of  the  brain 
in  chronic  alcoholism,  and  these  changes  have  been  frequently  found 
in  cases  of  alcoholic  neuritis.  The  dendrites  of  these  cells  lose  the 
fine  granules  which  cover  them,  and  become  smooth  and  bare.  (See 
Fig.  6.)  Then  the  dendrites  become  broken  and  disintegrated.  The 
cells  become  swollen,  vacuolated,  degenerated,  and  finally  atrophied, 
and  their  axones  share  in  the  destruction.  So  long  as  the  lesion  is 
limited  to  the  shedding  of  the  granules  it  is  not  irreparable,  and  re- 
generation may  occur.  In  the  cases  of  alcoholic  neuritis  which  present 
psychical  symptoms,  there  is  every  reason  to  suppose  that  these  patho- 
logical changes  are  present  in  the  brain,  and  the  degree  of  impairment 
of  mental  capacity  is  directly  proportionate  to  their  intensity.  A  gen- 
eral atrophy  of  the  muscular  fibres  is  always  present  in  the  paralyzed 
muscle,  with  a  loss  of  their  striation,  a  fatty  degeneration  and  a  dis- 
integration of  the  muscular  substance,  and  an  increase  of  the  connec- 
tive tissue.  The  muscular  fibres  show  great  irregularity  of  size,  and 
present  both  parenchymatous  and  interstitial  changes  with  the  deposit 
of  fat. 

Etiology. — Males  are  more  commonly  affected  than  females  by  al- 
coholic paralysis.  Thus  in  250  cases  which  I  have  collected  or  ob- 
served but  89  were  in  women.  When,  however,  the  much  greater 
prevalence  of  drinking  among  men  is  considered,  it  becomes  evident 
that  the  disease  is  relatively  far  more  frequent  among  women  than 
among  men.  And  this  fact  has  been  emphasized  by  all  writers.  In 
men  the  poison  seems  more  liable  to  manifest  itself  by  acute  cerebral 
symptoms  than  by  those  of  disease  of  the  peripheral  nerves.  Neuritis 
is  especially  frequent  among  those  persons  in  the  higher  classes  whose 
nervous  organism  is  highly  developed,  and  who  lead  a  comparatively 
inactive  life.  It  seems  not  at  all  improbable  that  sedentary  habits 
predispose  an  alcoholic  drinker  to  this  disease,  and  hence  active  work- 
ers, male  or  female,  though  taking  an  equally  large  amount  of  liquor 
as  the  luxurious  drinker,  escape. 

All  alcoholic  drinks  are  not  equally  prone  to  produce  paralysis.  It 
is  the  spirituous  liquors — brandy,  whiskey,  gin,  and  rum  and  the 
liqueurs,  absinthe,  vermouthe,  etc.  — which  are  particularly  dangerous. 
But  many  cases  are  known  to  be  due  to  excessive  beer  drinking.     I 

*  Heilbronner.     Monatsschrift  f.  Psych,  u.  Neurologie,  1898,  p.  246. 


AlCOHOLIG    NEURtTiS.  .       ^l5 

see  every  year  at  the  clinic  cases  in  drivers  of  beer  wagons  who  con- 
sume forty  gr  fifty  glasses  of  beer  daily.  I  have  even  seen  a  case  in 
a  child  of  three  years  who  had  been  given  beer  several  times  a  day  for 
some  weeks.  I  have  known  cases  in  women  to  be  due  to  taking  col- 
ogne ;  and  in  Ireland,  where  ether  is  used  as  a  drink,  many  cases  are 
due  to  this  cause.  The  fumes  of  alcohol  are  capable  of  causing  the 
disease  in  a  mild  form.  It  is  steady  drinking  of  small  amounts 
throughout  the  day,  rather  than  excessive  occasional  indulgence,  which 
is  more  likely  to  cause  neuritis.  The  liability  to  neuritis  from  medici- 
nal uses  of  alcohol  should  never  be  forgotten  in  those  cases  where  it  is 
used  as  a  heart  stimulant.  I  have  seen  many  cases  developing  after 
typhoid  fever  and  pneumonia  where  it  seems  very  probable  that  the 
actual  cause  was  the  free  use  of  alcoholic  stimulants  given  to  support 
the  heart,  little  food  being  given.  All  ages  are  liable  to  be  affected, 
but  the  majority  of  cases  are  between  thirty-five  and  fifty  years  of  age. 

Symptoms. — It  is  only  after  alcohol  has  been  consumed  in  large 
amounts  and  for  a  considerable  length  of  time  that  neuritis  develops. 
Its  onset,  though  often  apparently  gradual,  is  usually  sudden.  For 
months  the  patient  has  suffered  from  chronic  gastritis,  insomnia,  general 
neuralgic  pains,  or  severe  pains  in  the  joints  or  limbs,  and  from  alco- 
holic tremor  and  a  certain  feebleness  in  movement,  when  all  at  once 
his  legs  give  way  beneath  him  and  after  the  sudden  fall  he  finds  him- 
self unable  to  rise.  Thus  a  patient  of  mine,  after  a  year  of  such  pre- 
monitory symptoms,  was  seized  with  paralysis  quite  unexpectedly  when 
getting  out  of  bed  in  the  night.  The  onset  in  other  cases  is  preceded 
by  symptoms  of  disturbance  in  the  heart  and  lungs.  The  patient  has 
had  palpitation  and  dyspnoea ;  has  had  swelling  of  the  feet,  undue  ex- 
haustion on  exertion  and  possibly  a  bronchial  cough  ;  and  when  ex- 
amined is  found  to  have  a  feeble  heart,  dilated  and  flabby,  with  faint 
sounds  or  distinct  mitral  regurgitant  murmur,  and  the  signs  of  chronic 
pulmonary  congestion.  Then,  after  a  period  of  unusual  indulgence  in 
stimulants,  paralysis  suddenly  develops  with  all  the  sensory  distur- 
bances of  a  neuritis. 

When  the  paralysis  begins  it  soon  becomes  complete  in  the  feet  and 
legs  to  the  knee,  and  may  advance  up  to  the  thigh,  involving  the  ex- 
tensors of  the  legs.  It  next  attacks  the  hands  and  forearms,  and 
while  in  all  extremities  it  is  greater  in  the  extensors  than  in  the  flexors, 
in  some  cases  both  groups  of  muscles  become  entirely  helpless.  The 
paralyzed  muscles  are  flabby,  and  soon  become  atrophied,  they  have 
no  excitability  to  mechanical  irritation,  and  the  tendon  reflexes  are 
lost.  They  fail  to  react  to  a  faradic  current  in  the  majority  of  cases, 
though  occasionally  a  very  strong  current  may  produce  a  response. 
When  galvanism  is  applied  the  reaction  of  degeneration  is  found  to  be 
present.  It  is  often  found  that  strong  galvanic  currents  have  to  be 
used  to  produce  any  contraction  at  all.  The  paralysis  of  the  muscles 
may  advance  rapidly  in  severe  cases,  involving  the  motor  cranial 
nerves,  the  muscles  of  the  trunk,  and,  lastly,  the  diaphragm,  thus  caus- 
ing death.     More  frequently,  however,  it  is  arrested  when  only  the 


^16  TEE   TOXIC  FORMS   OF  MVLTtPLiJ   NBVRlTt^. 

distal  parts  of  the  extremities  are  involved,  then,  after  a  stationary- 
period,  which  varies  from  a  few  weeks  to  several  months,  it  gradually 
subsides  until  recovery  is  complete. 

The  position  assumed  by  the  paralyzed  limbs  has  been  thought  to 
be  almost  characteristic.  Dropped-wrist  and  dropped-foot  appear 
early,  due  to  the  paralysis  of  the  extensors,  and  further  deformities  of 
hands  and  feet  follow.  When  the  feet  are  dropped  the  first  joint  of 
the  toes  may  be  hyperextended  though  the  second  is  flexed,  but  as 
time  goes  on  this  hyperextension  gives  place  to  flexion  and  then  the 
entire  foot  is  paralyzed  and  hangs  down,  the  natural  concavity  of  the 
sole  being  increased.  The  weight  of  the  bedclothes  may  increase  the 
dropping  of  the  feet  and  flexion  of  the  toes.  Later  the  muscles  of 
the  sole  and  of  the  calf  become  permanently  shortened  and  the  sole  can 
no  longer  be  placed  on  the  floor.  When  the  patient  is  able  to  begin 
to  stand,  all  the  weight  comes  on  the  toes,  which  often  turn  under. 
Then  the  heel  of  the  shoe  has  to  be  built  up  in  order  to  form  any  sup- 
port. The  deformity  of  the  hands  is  also  different  in  different  stages 
of  the  disease.  At  first  there  is  dropped-wrist,  the  fingers  hanging 
limp.  Later,  a  claw-hand  appears,  the  first  phalanges  being  hyperex- 
tended, the  second  and  third  flexed.  The  thumb  is  also  hyperex- 
tended, and,  the  metacarpal  bone  being  drawn  backward  but  adducted, 
the  thumb  can  no  longer  be  apposed  to  the  fingers.  (See  Fig.  104.) 
After  a  time  the  hyperextension  gives  way,  the  intrinsic  muscles  of  the 
hand  being  paralyzed  and  the  fingers  are  straight,  adducted,  and  im- 
movable, being  fixed  by  contractured,  atrophied  muscles.  In  this 
state  ankylosis  of  the  smaller  joints  often  develops,  and  the  use  of  the 
hands  is  very  slowly  regained  after  painful  massage  and  passive 
motions.  I  have  seen  bilateral  facial  paralysis  of  the  peripheral  type 
in  a  case  of  alcoholic  paralysis ;  and  several  cases  in  which  the  ocular 
muscles  were  paralyzed.  Irregular,  rapid  pulse,  due  to  an  affection 
of  the  pneumogastric,  is  a  not  uncommon  occurrence  in  alcoholic  cases, 
and  is  a  dangerous  symptom. 

The  patient  suffers  far  more  from  the  disturbance  of  sensation  than 
from  the  paralysis.  In  the  description  of  Jackson,  the  pains  were 
graphically  portrayed.  They  are  the  cause  of  terrible  agony"  are  suf- 
ficient to  produce  insomnia,  and  wear  seriously  upon  the  endurance  of 
the  sufferer. 

In  addition  to  pain,  hyperaesthesia,  both  of  the  skin  and  muscles,  is 
always  observed.  It  is  usually  quite  extensive  in  the  legs,  though  in 
cases  of  poisoning  by  absinthe  it  has  been  limited  to  the  soles  of  the 
feet.  The  muscles,  as  well  as  the  skin,  are  sensitive  to  handling  and 
to  pressure,  and  marked  tenderness  in  the  course  of  the  nerves  is  con- 
stantly elicited  by  examination.  In  many  cases  soon  after  the  onset, 
the  patients  cannot  bear  to  be  touched  or  moved,  though  perfectly  un- 
able to  help  themselves.  Cramps  in  the  calves  or  in  the  extensor  sur- 
face of  the  thighs  are  often  complained  of  and  give  rise  to  much 
distress. 

Parsesthesise  are  always  complained  of.     Numbness,  tingling,  and 


ALCOHOLIC    NEURITIS.  217 

formication  are  frequent.  In  one  of  my  patients  the  sensation  was  as 
if  heavy  brsteelets  were  'around  the  wrists,  and  as  if  very  tight  drawers 
were  on  the  legs.  At  other  times  she  felt  as  if  the  limbs  were  swollen, 
and  as  if  the  skin  was  about  to  burst.  One  patient  described  the  sen- 
sation as  being  like  iron  stockings  on  the  legs.  Another  insisted  that 
there  were  needles  under  the  skin  of  the  hands.  Such  sensations  may 
cease  as  the  case  increases  in  severity,  and  give  place  to  a  total  lack  of 
sensation  in  the  parts.  They  return,  however,  with  advancing  recov- 
ery, and  are  among  the  last  symptoms  to  disappear. 

Abolition  of  tactile  sense,  and  to  some  degree  of  muscular  sense,  is 
the  rule  after  the  paralysis  has  developed.  Temperature  sense  and  the 
perception  of  pain  are  never  wholly  lost,  but  may  be  delayed  in  trans- 
mission. Thus  in  multiple  neuritis  the  phenomena  of  dissociated  sen- 
sation may  be  found,  but  in  the  reverse  sense  to  that  observed  in 
syringomyelia.  The  anaesthesia  may  be  limited  to  irregular  areas,  and 
may  be  found  in  the  cutaneous  distribution  of  one  nerve  only,  but 
is  usually  found  over  the  entire  distal  part  of  the  paralyzed  limb. 
Usually  the  cutaneous  reflexes  are  preserved. 

The  loss  of  muscular  sense  is,  in  some  cases,  so  marked  a  symptom, 
and  one  of  such  early  occurrence,  that  Dreschfeld  distinguished  a  class 
of  cases  which  he  terms  ataxic  rather  than  paralytic.  And  this  dis- 
tinction is  perfectly  justifiable,  for  in  many  cases  it  is  the  incoordina- 
tion which  first  attracts  the  attention  of  both  the  patient  and  the 
physician.  It  is  this  class  of  alcoholic  cases  which  may  be  mistaken 
for  locomotor  ataxia,  and  which  have  been  named  by  French  writers 
pseudo-tabes  alcoolique,^  or  neurotabes  peripherica  by  the  Germans. 
But  ataxia  is  not  exclusively  limited  to  this  class  of  cases.  It  may  be 
present  in  some  degree  in  cases  of  paralysis,  and  during  recovery  from 
paralysis  the  deficiency  in  coordinating  power  may  become  evident, 
and  appear  to  retard  the  progress  of  the  case.  Nor  are  the  cases  of 
ataxia,  on  the  other  hand,  free  from  paralysis. 

The  gait  in  neuritis  is  quite  characteristic.  By  contrasting  the 
walk  of  a  true  ataxic  patient  with  that  of  an  ataxic  alcoholic  patient, 
WestphaP  and  Charcot^  have  each  pointed  out  several  points  of 
difference.  The  tabetic  patient  throws  the  foot  forward  with  undue 
violence,  the  toe  lifted  high  in  the  air,  and  brings  first  the  heel  down 
forcibly  and  then  the  entire  foot.  The  alcoholic,  however,  has  some 
weakness  in  the  muscles  of  extension  and  cannot  raise  the  toe.  He, 
therefore,  lifts  the  foot  high  in  order  to  step  over  the  hanging  toe  and 
not  to  trip  on  it,  but  the  motion  is  made  without  undue  force.  He 
then  throws  the  foot  forward  in  order  to  throw  the  toes  up  and  get 
them  out  of  the  way  as  he  brings  the  foot  down  to  the  floor.  The 
motion  is  awkward,  and  has  an  appearance  of  one  stepping  over  high 
obstacles,  but  it  is  a  voluntary  attempt  to  remedy  a  deficient  power — 
not  the  involuntary  awkwardness  of  a  man  unable  to  manage  strong 

^Dejerine.     Arch,  de  phys.,  1884.     See  also  Dreschfeld,  Brain,  Nos.  26  and  32. 
^  Westphal.     Ueber  eino  bei  chronischen  Alcoholisten  beobachtete  Form  von  Geh- 
storungen,  Charitd  Annalen,  1879. 

*  Charcot.     Lejons,  Le  Progress  M^dicale,  1886. 


218  TEE    TOXIC   FOliMS   OF   MULTIPLE   NEVRItIS. 

muscles.  There  may  be  in  both  patients  some  tottering  and  swaying 
when  standing  witli  '^e  eyes  closed;  this  so-called  Romberg  symptom 
is  common  to  both  locomotor  ataxia  and  alcoholic  neuritis. 

The  vasomotor  symptoms  in  alcoholic  neuritis  are  very  variable. 
Sometimes  they  precede  the  attack  of  paralysis  for  some  months. 
There  are  irregularities  of  circulation  in  the  extremities  causing  cold 
hands  and  feet,  or  burning  red  hands  and  feet,  or  undue  sweating. 
When  the  paralysis  is  developed  the  color  of  the  extremities  is  pale ; 
they  are  cool  and  often  wet  with  perspiration  or  occasionally  livid  and 
hot.  If  allowed  to  hang  down  both  hands  and  feet  get  purple  and 
swell.  Ross  saw  one  case  in  which  Raynaud's  disease  developed.  As 
the  disease  goes  on,  glossy  skin  appears  and  then  the  dermal  tissues 
become  atrophied  and  the  skin  seems  too  tight  for  the  fingers  and  toes. 
It  is  often  pigmented  and  scaly,  and,  when  the  tenderness  is  so  great 
as  to  prevent  washing,  dark  brown,  scaly  masses  may  collect  on  the 
fingers  and  hands  and  feet.  The  growth  of  the  nails  is  always 
affected.  They  become  ridged  transversely,  are  thick  and  brittle,  and 
too  tender  to  be  cut.  As  recovery  begins  new,  thin,  healthy  nail  ap- 
pears above  the  thick  ridge.     (See  Fig.  104.) 

The  special  senses  are  occasionally  affected  in  cases  of  alcoholic 
paralysis.  Amblyopia  has  been  observed,  and  also  defective  vision 
from  central  scotoma.  The  field  of  color  vision  is  often  contracted 
even  when  sight  is  preserved.  There  may  develop  a  true  optic 
neuritis,  evident  to  the  ophthalmoscope,  and  this  may  go  on  to  optic 
nerve  atrophy.^  Inequality  of  the  pupils  is  frequently  seen,  as  is  also 
a  moderate  contraction  of  the  pupil.  All  these  eye  symptoms,  occurring 
as  they  may  in  a  case  of  the  ataxic  variety,  make  a  differential  diag- 
nosis from  locomotor  ataxia  difficult.  The  Argyll-Robertson  pupil 
(which  contracts  in  accommodation,  but  not  to  light)  has  rarely  been 
seen  in  alcoholic  cases,  while  it  is  an  early  symptom  of  tabes.  One 
writer  affirms  that  a  condition  of  the  pupil  just  the  reverse  of  the  Argyll- 
Robertson  pupil  is  present  in  alcoholic  neuritis,  a  pupil  which  reacts 
to  light  but  fails  to  react  in  accommodation.  I  have  been  unable  to 
confirm  this  statement. 

One  important  feature  of  alcoholic  paralysis  remains  to  be  noticed, 
viz.,  the  mental  symptoms.  These  are  hardly  ever  wanting  in  severe 
cases.  I  called  attention  to  these  symptoms  in  1887.^  They  have 
been  most  fully  described  by  Korsakow,^  Soukhanoff  *  and  lately  by 
Jelliffe.^  The  name  Korsakow's  psychosis  has  been  given  to  these 
symptoms,  but  I  prefer  the  term  pol}Tieuritic  psychosis,  or  chronic 
alcoholic  delirium.  There  is  at  first  excitement  rising  to  the  degree  of 
active  delirium,  with  illusions  and  hallucinations  of  the  various  senses ; 
there  is  insomnia,  which  soon  exhausts  the  patient  if  it  is  not  remedied  ; 
there  is  a  loss  of  memory,  especially  of  recent  occurrences  ;  and  a  lack 
of  power  of  attention  or  concentration  which  prevents  intelligent 
conversation,  and  a  mild  dementia. 

^Brissaud.     Les  Paralysies  Toxiques,  p.  31,  Paris,  1886. 

'Middleton  Goldsmith  Lectures,  1887.     Medical  News,  Mrch,  1887. 

3  Arch.  f.  Psych.,  1892,  Bd.  xxi.,  669.  "Eaev.  de  Med.,  Mav,  1897. 

^N.  Y.  Med."  Jour.,  Oct.  24,  1908. 


ALCOHOLIC  NEURITIS.  .  219 

The  patients  are  usually  greatly  depressed  aud  alarmed  at  their  con- 
dition, and  y^quire  hourly  reassurance  of  the  probability  of  recovery, 
forgetting  what  has  been  told  them  almost  as  soon  as  it  has  been 
uttered.  The  depression  may  lead  to  crying  for  hours  at  a  time,  but 
the  self-reproaches  common  in  true  melancholia  are  never  heard  in  this 
state.  A  marked  confusion  of  ideas  is  often  noticed,  the  patient  being 
indiiFerent  and  incapable  of  connected  conversation.  The  indifference 
to  bodily  wants  may  be  so  great  as  to  lead  to  uncleanliness,  and  since 
paralysis  of  the  sphinctei-  is  the  rare  exception,  incontinence,  if  it  occurs, 
is  to  be  ascribed  to  the  mental  state. 

It  is  useless  to  attempt  to  get  any  reliable  history  of  their  illness 
from  these  patients.  Their  statements  are  unintelligible  or  unreliable. 
They  will  relate  occurrences  as  having  happened  recently,  with  much 
elaboration  of  detail,  when  as  a  fact  the  story  is  entirely  a  product  of 
their  imagination.  Thus  one  patient  of  my  own  who  had  been  confined 
to  bed  for  many  days,  told  me  one  afternoon  that  she  had  been  out  to 
see  an  eminent  gynecologist  during  the  morning ;  had  gone  to  his  office 
and  waited  for  him  several  hours ;  had  seen  other  patients  there,  and 
finally  had  been  told  by  the  doctor's  brother  that  he  would  not  return 
in  time  to  see  her,  so  she  had  come  home  again.  And  this  was  all 
related  in  apparent  good  faith,  so  that  I  have  no  doubt  that  she  be- 
lieved that  what  she  said  had  occurred.  Several  patients  have  de- 
clared that  they  had  been  out  to  walk  or  to  drive,  when  they  had  been 
unable  to  leave  the  bed  for  some  weeks.  One  patient  told  me  a  dif- 
ferent history  of  her  case  every  day  for  a  week,  and  it  was  only 
from  her  friends  that  the  correct  account  was  obtained.  It  is  evident 
that  the  statements  of  these  patients  cannot  be  accepted  regarding  any- 
thing, especially  as  to  their  own  history.  The  defects  of  memory  are 
permanent  and  after  recovery  patients  have  no  clear  recollection  of 
their  illness.  This  mental  state  may  last  for  two  or  three  months,  but 
after  a  time  the  mind  becomes  clearer,  the  power  of  attention  returns, 
memory  improves,  the  depression  is  less  constant,  and  mental  health 
is  regained.  Even  in  cases  lasting  four  months  I  have  seen  permanent 
recoveries  now  lasting  ten  years.  Occasionally  a  permanent  dementia 
develops.  While  Korsakow's  psychosis  is  usually  due  to  alcohol,  it 
may  appear  as  a  result  of  long-continued  malnutrition,  and  anaemia  in 
cases  of  gastric  origin,  and  after  childbirth  attended  by  severe  hem- 
rhages.^  Patients  suffering  from  this  psychosis  are  legally  insane  for 
the  time  until  they  recover. 

,  The  course  of  alcoholic  neuritis  is  quite  uniform.  After  a  sudden 
onset  the  symptoms  advance  rapidly  to  a  high  degree,  which  is  reached 
in  a  week  or  two  from  the  beginning  of  the  paralysis  or  ataxia.  Then 
they  may  increase  further,  and  cause  death  by  respiratory  paralysis. 
Usually  they  remain  stationary  for  a  time,  which  may  be  several 
months  in  severe  types,  and  then  gradually  subside,  the  entire  duration 
being  from  four  months  to  two  years.  Individual  muscles  regain  their 
power,  tone,  firmness,  and  electrical  reaction  slowly,  and  during  recov- 
ery the  tingling  and  numbness  in  the  hands  and  feet  may  be  severe. 
*  Serbsky.     Arbeit,  a.  d.  Wiener,  Neur.  Institut,  xv,  1907. 


220  THE    TOXIC   FORMS    OF   MULTIPLE   NEURITIS. 

In  a  few  cases  the  muscles  become  contractured,  and  permanent  defor- 
mities develop,  only  to  be  overcome  by  long-continued  massage  or  by 
operative  measures.  These  deformities  cause  great  difficulty  in  regain- 
ing the  power  of  walking.  The  feet  cannot  be  placed  flat  on  the 
ground  and  the  knees  are  partly  flexed,  so  that  it  is  often  months  before 
the  patients  can  stand,  and  even  when  by  the  aid  of  high  heels  on  the 
shoes,  supporting  braces  to  the  ankles,  and  crutches,  the  upright  position 
can  be  attained,  it  is  with  difficulty  that  the  balance  can  be  preserved. 
When  the  fact  is  considered  that  those  who  recover  rapidly  rarely  fail 
to  resort  again  at  once  to  the  use  of  stimulants,  and  thus  expose  them- 
selves to  the  danger  of  a  relapse,  the  ultimate  fate  of  the  chronic  cases 
is  hardly  more  serious  than  that  of  those  who  get  well  quickly. 

Treatment. — The  treatment  of  alcoholic  neuritis  differs  in  no  re- 
spect from  that  already  described  on  page  207. 

The  following  cases  illustrate  the  various  forms  of  the  disease : 

A  young  man  who  had  drank  whiskey  eight  or  ten  times  a  day  for 
some  months  without  ever  being  drunk,  began  to  feel  a  numbness  in 
the  legs  and  then  in  the  hands,  which  was  followed  in  three  weeks  by 
some  undue  fatigue  on  walking ;  occasional  sharp  pains  in  the  legs, 
and  a  week  later  by  weakness  in  the  extensors  of  the  feet  and  un- 
steadiness of  gait.  He  went  to  the  Hot  Springs  of  Virginia,  was 
treated  for  rheumatism,  as  he  suiFered  while  there  from  pains  in  the 
legs,  but  was  finally  told  after  two  weeks  that  he  had  locomotor  ataxia 
and  was  advised  to  go  home.  When  I  saw  him  on  his  return  his  sub- 
jective numbness  was  attended  by  a  slight  anaesthesia  of  the  fingers 
and  toes.  His  calves  and  forearms  were  very  tender  to  pressure  and 
the  extensors  were  decidedly  weak,  so  that  he  could  not  raise  his  toes 
from  the  floor  when  standing,  but  he  had  no  drop-foot  or  drop-wrist. 
He  was  quite  ataxic  in  his  gait,  swayed  with  eyes  closed,  but  had  no 
ataxia  of  the  hands.  His  knee-jerks  were  absent,  but  he  had  no  dis- 
turbance of  his  bladder,  and  his  pupils  reacted  to  light.  His  pulse 
was  rapid  and  feeble,  and  he  was  subject  to  faint  feelings  and  occa- 
sional sudden  fears.  He  was  put  to  bed,  deprived  of  alcoholic  drinks, 
fed  frequently  and  well,  and  given  two  warm  baths  daily  and  general 
massage.  Cod-liver  oil  and  glycero-phosphate  of  soda,  15  grains, 
three  times  a  day,  were  given  daily,  with  strychnine,  gi^p,  three  times  a 
day,  four  days  in  each  week.  Within  four  weeks  his  symptoms  had 
improved  so  much  that  he  was  allowed  to  get  up.  At  the  end  of  nine 
weeks  his  paralysis  had  entirely  disappeared  and  he  no  longer  had 
pains  or  anaesthesia  or  any  ataxia ;  and  fourteen  weeks  from  the  date 
of  his  return  from  Virginia  he  was  perfectly  well,  except  for  a  very 
slight  numbness  in  the  sole  of  one  foot.  The  knee-jerks  returned  in 
the  tenth  week.  The  entire  duration  of  his  illness  was  nineteen 
weeks.     He  has  had  no  recurrence. 

A  man,  aged  thirty-six  years,  a  hard  drinker,  after  an  attack  of 
gastritis  became  paralyzed  suddenly  in  both  legs.  A  few  days  after 
this  both  hands  became  useless,  so  that  he  was  completely  helpless. 
On  admission  to  the  hospital,  seven  weeks  after  the  onset,  he  com- 
plained of  pain  in  all   four  extremities,   and  of  tenderness  in  the 


ALCOHOLIC   NEURITIS.  221 

muscles  and  joints  when  these  were  handled.  The  upper  extremities 
were  almost  totally  paralyzed  below  the  elbows,  the  only  motion  pos- 
sible being  a  slight  flexion  of  the  fingers.  There  was  drop-wrist  on 
both  sides.  The  lower  extremities  were  totally  paralyzed  below  the 
knee,  and  the  feet  hung  down  motionless.  The  muscles  of  the  thighs 
were  also  aifected,  for  although  he  could  pull  his  legs  up  in  bed,  it  was 
with  great  difficulty  that  they  could  be  straightened  out.  All  the 
paralyzed  muscles  were  atrophied,'  and  showed  a  reaction  of  degenera- 
tion. Tactile  sense  was  considerably  impaired  in  areas  corresponding 
to  the  region  covered  by  long  stockings  and  gloves,  but  the  senses  of 
pain  and  temperature  and  muscular  sense  seemed  to  be  natural.  The 
knee-jerks  were  lost.  When  an  attempt  was  made  to  have  him  stand, 
the  feet  were  pushed  out  and  the  knees  doubled  under  him  at  once. 
Fibrillary  twitchings  were  very  noticeable  in  the  atrophied  muscles, 
and  a  marked  tremor  of  the  tongue  was  seen.  The  facial  and  ocular 
muscles  were  not  affected,  and  the  special  senses  were  normal.  There 
was  no  incontinence  of  urine,  and  no  symptoms  of  thoracic  or  visceral 
disease.  His  mind  was  much  impaired.  He  talked  in  a  rambling 
manner,  laughed  much,  and  could  not  fix  his  attention,  and  his  memory 
was  so  poor  that  no  reliance  could  be  put  in  his  statements.  Under 
treatment  by  complete  cutting  off  of  all  stimulants  and  by  iodide  of 
potash  he  began  at  once  to  improve.  In  two  months  from  the  time 
of  admission  he  was  able  to  walk,  and  two  months  later  he  was  dis- 
charged perfectly  well.     In  this  case  the  duration  was  six  months. 

A  young  woman,  whose  father  died  of  alcoholism,  had  drank 
champagne  and  brandy  very  freely  for  two  years  during  a  period 
of  mental  distress.  She  was  then  (August)  suddenly  seized  with 
paralysis  attended  by  severe  pain  in  arms  and  legs.  She  was  at  the 
time  in  Europe,  but  was  brought  home,  being  carried  to  and  from  the 
steamer.  When  I  saw  her  in  November  she  had  a  typical  alcoholic 
neuritis,  with  great  tenderness  along  the  nerves  in  the  calves  and  on 
the  front  of  the  thighs,  and  drop-feet;  cold,  clammy  hands  covered 
with  scales  and  pigmented,  and  anaesthesia  in  the  glove  and  stocking 
areas.  She  was  suffering  intensely  from  such  pains  and  hypersesthesia 
in  her  arms  and  legs  that  she  could  not  bear  the  weight  of  the  bed- 
clothes. She  lay  in  bed  with  knees  flexed  and  feet  extended;  the 
knee-jerks  were  absent.  Her  pulse  was  feeble  and  rapid  and  she  was 
in  a  state  of  great  mental  distress  at  her  condition.  She  required 
anodynes  to  relieve  the  pain.  Under  food,  cessation  of  alcohol,  which 
was  extremely  difficult  to  secure,  as  it  was  the  only  thing  which 
relieved  her  pains,  and  general  tonics,  especially  strychnine,  she 
improved  rapidly.  In  February  she  was  able  to  walk  with  the  help 
of  high  heels  and  corset  shoes,  and  in  April  all  her  symptoms  had  dis- 
appeared excepting  numb  sensations  and  occasional  pains  in  her  feet. 
Her  knee-jerks  had  returned.  She  soon  resumed  drinking  in  spite  of 
warning,  and  six  months  after  her  recovery  she  was  again  paralyzed ; 
this  time  the  hands  as  well  as  the  feet  being  helpless,  and  drop- wrist 
developing  at  the  same  time  as  drop-foot.     I  saw  her  in  December  in 


222  THE    TOXIC  FORMS   OF   MULTIPLE    NEURITIS. 

the  second  month  of  this  attack.  She  had  a  pulse  of  150,  irregular 
and  intermittent,  and  was  cyanotic.  Her  suffering  from  pain  was 
extreme,  and  contractures  existed  in  all  the  extremities.  Glossy 
skin  was  present  on  legs  and  arms,  and  the  extremities  were  hot  and 
perspiring.  Her  mind  was  much  affected.  She  had  hallucinations  of 
touch  and  sight,  affirmed  that  her  hands  and  feet  were  filled  with 
needles,  which  she  spent  hours  in  drawing  out  of  the  skin,  filling  an 
empty  box  which  she  showed  me  as  full  of  them;  declared  that 
scissors  and  knives  were  still  buried  in  her  flesh  and  cutting  her. 
She  had  no  memory  of  the  events  of  the  past  two  months.  This  con- 
dition continued  for  three  weeks,  her  life  being  despaired  of  on  account 
of  her  weak  heart.  During  this  time  she  told  me  daily  some  new 
story  of  having  been  to  balls  and  receptions,  and  to  drive  in  the  park. 
Then  (January)  her  circulation  improved,  her  pains  became  less 
severe,  she  became  less  restless  and  was  able  to  sleep,  and  her  mind 
gradually  became  clearer.  At  the  end  of  April  she  could  use  her 
hands  a  little  and  had  lost  the  glossy  skin  and  her  mind  was  clear. 
But  it  was  not  until  October  that  she  was  able  to  walk,  and  then  she 
had  to  wear  braces.     In  January  she  had  recovered. 

MULTIPLE  NEURITIS  DUE  TO  POISONING  BY  CARBON-MONOXIDE 
OR  ILLUMINATING  GAS. 

A  certain  number  of  persons  are  brought  into  the  hospitals  every 
year  suffering  from  acute  poisoning  by  illuminating  gas.  Either  acci- 
dentally or  with  suicidal  intention  they  have  turned  on  the  gas  in  their 
sleeping  rooms,  and  have  been  found  after  several  hours  in  a  state  of 
coma.  If  they  die  softening  of  both  corpora  striata  in  the  brain  are 
found.  (See  Fig.  223,  page  482.)  If  they  survive  this  condition  of 
acute  poisoning  they  sometimes  develop  symptoms  of  multiple  neuritis 
within  a  week.  These  symptons  consist  of  tingling  and  numbness  of 
the  extremities,  more  acutely  felt  in  the  distal  parts  and  attended  by 
slight  anaesthesia,  also  a  condition  of  weakness  in  the  muscles  which 
does  not  go  on  to  a  total  paralysis  but  makes  them  feeble.  There  are 
rarely  any  atrophies  or  changes  in  the  electrical  contractility  in  the 
affected  muscles,  and  the  sensory  symptoms  are,  as  a  rule,  much  more 
marked  than  the  motor  symptoms.  For  many  weeks,  or  even  months, 
these  patients  suffer  from  parsesthesise  which  are  excessively  disagree- 
able. In  a  few  cases  I  have  seen  a  slight  ataxia  of  movement,  both  in 
walking  and  in  the  use  of  the  hands.  The  symptoms  gradually  subside 
under  general  tonic  treatment,  and  the  patient  finally  recovers. 

The  following  case  is  of  interest,  as  it  appears  to  be  a  case  of  multiple 
neuritis  following  poisoning  by  natural  gas  : 

The  patient  was  a  healthy  man  of  good  habits  who  had  been  very 
much  exposed  to  the  fumes  of  natural  gas  in  his  home  in  Peru,  Ind.  He 
gradually  developed  symptoms  of  tingling  and  numbness  in  the  legs, 
which  soon  after  extended  to  the  hands  and  increased  in  extent  until 
the  legs  and  thighs  and  the  hands  and  forearms  were  the  constant  s^at 


NEURITIS  DUE  TO  POISONING  BY  SULPHIDE  OF  CARBON.       223 

of  disagreeable  numbness.  After  suffering  in  this  manner  for  three 
months  he  began  to  notice  an  uncertainty  of  gait,  which  soon  became 
a  marked  araxia.  There  were  at  no  time  any  pains,  paralysis,  atrophy, 
fibrillary  twitchings,  or  disturbances  of  the  bladder  and  rectum.  His 
knee-jerks  were  normal  and  his  pupils  reacted  perfectly  to  light,  but 
his  gait  was  distinctly  ataxic  ;  he  swayed  with  his  eyes  closed  and  had 
a  marked  condition  of  anaesthesia  to  touch,  temperature,  and  pain  as 
high  as  the  middle  of  the  thighs  and  elbows,  this  anaesthesia  being 
more  intense  in  the  hands  and  feet  than  in  the  upper  parts.  He  had 
had  during  the  entire  illness  some  gastro-intestinal  catarrh  with  at- 
tacks of  diarrhoea,  but  careful  investigation  failed  to  reveal  any  pos- 
sible source  of  poisoning  by  lead,  arsenic,  mercury,  or  drugs.  It  was 
discovered,  however,  that  in  both  his  house  and  office  natural  gas  was 
burned  for  heating  and  lighting  purposes,  and  that  the  fixtures  were 
imperfect,  resulting  in  incomplete  combustion  and  leakage  so  that  it 
was  necessary  to  make  special  provision  for  ventilation,  and  both  house 
and  office  were  often  filled  with  foul  and  nauseating  gases  when  the 
draughts  through  the  ventilators  were  imperfect.  On  removal  from 
his  home  to  the  Clifton  Springs  Sanitarium,  he  rapidly  improved  under 
general  treatment  and  was  able  to  return  home  after  three  months  quite 
recovered.  A  year  later,  however,  also  in  the  spring,  after  the  con- 
finement of  the  winter,  the  symptoms  recurred  with  equal  intensity 
and  remained  for  eight  weeks  until  he  again  left  home,  when,  after 
four  weeks,  they  subsided.  In  the  absence  of  any  other  cause  (and 
great  care  was  taken  to  ascertain  some  cause  for  the  condition)  the 
neuritis  was  ascribed  to  the  inhalation  of  natural  gas. 

A  number  of  cases  of  neuritis  in  single  nerves  have  been  reported  by 
foreign  observers  occurring  after  poisoning  by  illuminating  gas.  It 
is  to  be  remembered  that  in  a  state  of  long-continued  coma,  pressure 
upon  nerve  trunks  may  occur  of  sufficient  duration  to  cause  traumatic 
neuritis  which  has  not,  under  such  circumstances,  any  relation  to  the 
cause  of  the  coma. 

MULTIPLE  NEURITIS  DUE  TO  POISONING  BY  SULPHIDE 
OF  CARBON. 

Multiple  neuritis  may  develop  in  workmen  who  are  exposed  to 
poisoning  from  sulphide  of  carbon.  This  substance  is  present  in  rubber 
and  the  disease  has  been  observed  in  those  who  work  in  rubber  fac- 
tories where  ventilation  is  neglected.  The  fumes  or  the  powdered 
dust  containing  sulphide  of  carbon  are  inhaled  or  get  into  the  mouth 
and  are  taken  into  the  stomach.  Guillain  ^  has  reported  a  case  in  a 
man  who  was  employed  in  manufacturing  toy  balloons.  These  balloons 
are  plunged  in  a  bath  containing  bisulphide  of  carbon.  This  patient 
developed  total  paralysis  without  sensory  symptoms.  The  effect  of 
the  poison  is  slowly  developed  and  seems  to  be  widespread.^     In  addi- 

^ Revue  de  Neurol.,  February,  1904. 

^  R.  Landenhoimer.  Die  SchwefelkohlenstofT  Vergiftungen  dcr  Gummiarbeiter, 
Leipzig,  1899,  where  full  references  to  all  published  esuies,  about  forty  in  number,  can 
be  found. 


224  TEE   TOXIC  F0R3IS   OF  MULTIPLE   NEURITIS. 

tion  to  a  state  of  general  ansemia,  with  disturbances  of  digestion,  the 
patients  suffer  from  headache,  inability  to  fix  the  attention  and  to  re- 
member, and  from  many  symptoms  of  functional  nervous  disorder, 
which  may  awaken  a  suspicion  of  hysteria ;  but  as  time  goes  on  the 
symptoms  of  multiple  neuritis  appear,  and  these  may  be  of  the  paralytic 
or  of  the  ataxic  type,  and  pursue  a  course  not  unlike  that  observed  in 
alcoholic  or  arsenical  cases.  The  development  of  cerebral  and  of 
spinal  symptoms  in  some  of  the  recorded  cases  makes  it  probable  that 
the  poison  has  an  effect  upon  the  central  nervous  system,  and  that  the 
lesion  is  not  in  all  cases  limited  to  the  peripheral  nerves.  Autopsies 
are  wanting  to  establish  an  exact  pathology. 

MULTIPLE  NEURITIS  DUE  TO  POISONING  BY  THE  COAL-TAR 
PRODUCTS,  ESPECIALLY  SULPHONAL  AND   TRIONAL. 

Since  the  general  use  of  the  coal-tar  products  in  the  treatment  of 
disease,  especially  of  painful  affections,  and  the  free  use  of  sulphoual 
and  trional  in  the  treatment  of  insomnia,  a  few  cases  of  multiple  neu- 
ritis have  been  reported,  occurring  in  patients  who  had  abused  these 
drugs.  And  their  chemical  resemblance  to  alcohol  lends  a  certain 
amount  of  support  to  the  hypothesis  that  they  are  capable  of  producing 
multiple  neuritis.  Remak  has  reported  a  case  as  having  occurred 
subsequent  to  the  use  of  antipyrine,  and  Stewart  Hart  ^  has  recorded  a 
case,  which  I  saw  with  him,  probably  produced  by  the  constant  use  of 
trional.  A  patient  of  my  own,  who  took  120  grains  of  sulphoual  in 
four  hours  by  mistake,  slept  two  days,  was  markedly  cyanotic,  with 
pulse  of  120  and  shallow  respiration,  and,  for  three  weeks  afterward, 
suffered  from  parsesthesise,  weakness,  and  ataxia  in  the  arms  below  the 
elbows  and  in  the  legs  below  the  knees.  The  symptoms  gradually  sub- 
sided. They  resembled  so  closely  those  which  I  had  seen  in  hospital 
cases  of  illuminating-gas  poisoning,  that  it  seemed  justifiable  to  ascribe 
them  to  a  mild  condition  of  neuritis. 

Multiple  neuritis  has  been  said  by  Ross  to  have  been  caused  by 
poisoning  with  roburite  (dinitrobenzol)  used  by  miners,  and  by  poison- 
ing with  anilin  oil,  used  by  dye  manufacturers.  In  the  cases  recorded 
the  symptoms  in  both  forms  of  poisoning  were  those  of  multiple  periph- 
eral neuritis,  together  with  general  constitutional  effects  —  anaemia, 
cyanosis,  and  a  blue  discoloration  of  the  mucous  membranes,  due  to 
the  action  of  the  poison  on  the  haemoglobin. 

ARSENICAL  NEURITIS. 

Historical. — Up  to  the  year  1883  the  various  nervous  affections 

produced  by  acute  or  chronic  poisoning  by  arsenic  had  been  ascribed 

to  spinal-cord  lesions.     Vulpian,^  Virchow  and  Scolozouboff  ^  insisted 

upon  the  central  origin  of  arsenical  paralysis ;  the  latter,  with  Popow,^ 

^  American  Journal  of  the  Medical  Sciences,  1901. 
^Lepons  sur  les  Mai.  du  Syst.  Nerv.,  1879,  p.  157, 
»  Arch,  de  Phys.  Norm,  et  Path.,  1884,  p.  325. 
*  yirchow's  Arch.,  1883,  Bd.  93,  p.  351- 


ARSENICAL    NEURITIS.  225 

basing  his  assertions  upon  physiological  experiments.  Dejerine/  how- 
ever, in  188^,  after  his,  studies  of  alcoholic  paralysis,  already  cited, 
concluded  from  the  close  resemblance  between  alcoholic  and  arsenical 
paralysis  that  the  lesion  was  a  multiple  neuritis.  And  subsequent 
observations  by  Philadelphia^  and  Boston  physicians,  by  Jaesche,^ 
Naunyn,*  Dana,^  Goldflam,''  Jolly,^  and  Henschen,^  have  established 
beyond  doubt  that  the  lesion  in  arsenical  poisoning  lies  exclusively  in 
the  nerves.  This  fact  has  received  complete  confirmation  during  the 
prevalence  of  an  epidemic  of  arsenical  poisoning  from  beer  which 
occurred  in  England  in  1899.^ 

Etiology. — Arsenical  poisoning  may  occur  from  the  accidental  or 
suicidal  ingestion  of  any  one  of  the  arsenical  salts  —  Paris  green  being 
the  one  usually  selected  by  those  attempting  suicide.  The  most 
common  cause,  however,  is  the  accidental  chronic  poisoning  which 
occurs  in  certain  manufacturers,  notably  in  tin  mines,  and  in  tin 
works;  in  dyeing  establishments  and  in  sulphuric  acid  factories,  and 
in  those  industries  in  which  sulphuric  acid  is  largely  used.  This  acid 
is  now  made  chiefly  from  arsenical  pyrites  and  thus  contains  consider- 
able quantities  of  arsenic. 

Chronic  arsenical  poisoning  may  occur  from  drinking  beer  in  the 
brewing  of  which  glucose,  or  "invert  sugar,"  prepared  by  the  aid  of 
sulphuric  acid,  enters.  Thus  in  the  English  epidemic  it  was  ascer- 
tained that  many  beers  brewed  in  Salford  and  Manchester  contained 
from  0.14  to  0.28  grain  of  arsenious  acid  to  the  gallon,  and  this 
was  shown  conclusively  to  be  present  only  in  the  glucose  or  invert 
sugar  used  in  the  brewing,  all  other  constituents  of  the  beer  being  free 
from  arsenic.  The  glucose  was  found  to  contain  arsenic  in  the  pro- 
portion of  4  parts  to  1 0,000. 

Chronic  arsenical  poisoning  may  also  arise  from  the  inhalation  of 
particles  of  arsenic  in  the  air,  which  are  given  off  from  wall-papers 
and  from  carpets,  hangings  and  furniture  coverings,  and  from  arti- 
ficial flowers  containing  arsenical  dyestuffs.  ^"^  This  was  the  cause  in  a 
case  of  my  own.  The  patient  was  a  woman,  who,  to  avoid  mis- 
carriage, was  kept  for  four  months  in  a  room  which  had  been  newly 
papered  and  furnished  with  green  cretonne  subsequently  found  to  con- 
tain large  quantities  of  arsenic.  The  frequency  of  such  poisoning  has 
led  to  the  passage  of  laws  in  the  State  of  Massachusetts  restricting  the 
use  of  dyes  containing  arsenic.  Many  toilet  powders  contain  arsenic, 
and  the  inhalation  of  particles  given  off  from  these,  or  the  application 

^  Cbmptes-rendus,  Tome  xcviii.,  Nr.  17,  October,  1883. 

2  Proceedings  of  the  College  of  Physicians  of  Philadelphia,  1883, 

^Inaug.  Dis.,  Breslau,  1883. 

*  Berliner  klin.  Woch.,  1886,  p.  555. 

*  Brain,  1887,  vol.  ix.,  p.  456. 
«Zeitsch.  f.  klin.  Med.,  1888,  p.  399. 

'Deutsche  med.  Wochen.,  1893,  Nr.  5,  and  Charity  Annalen,  1893. 

*  On  Arsenical  Paralysis,  Transactions  of  the  Eoyal  Society  of  Sciences  in  Upsala, 

9  Kelynack  and  Kirkby,  Arsenical  Poisoning  in  Beer  Drinkers,  Balliere,  Tindall  and 
Cox,  London,  1901.     Also  Lancet,  1900,  vol.  i.,  p.  1610. 

i»  J.  J.  Putnam.     Boston  Medical  and  Surgical  Journal,  1889,  p.  235. 
15 


226  THE    TOXIC   FORMS   OF   MULTIPLE    NEURITIS. 

of  arsenic  in  them  to  the  skin  may  produce  poisoning.  The  medi- 
cinal use  of  arsenic  in  large  doses  or  in  small  doses  long  continued 
may  give  rise  to  arsenical  poisoning.  This  is  particularly  liable  to 
occur  in  the  treatment  of  chorea,  of  pernicious  anaemia,  and  of  car- 
cinoma, in  all  of  which  diseases  the  drug  is  employed  for  months  at 
a  time.  I  have  seen  two  cases  of  arsenical  paralysis  among  1,400 
children  treated  for  chorea  in  my  clinic.  The  more  the  danger  of 
chronic  arsenical  poisoning  is  appreciated  and  the  greater  the  use 
of  articles  into  the  manufacture  of  which  it  enters,  the  more  frequent 
are  the  reports  of  cases  arising  in  an  unexpected  manner.  It  is  known 
that  in  Steiermark,  in  Austria,  large  numbers  of  the  peasants  eat 
arsenic.  F.  Miiller  ^  has  found  arsenical  paralysis  very  common  among 
this  class,  in  fact,  even  more  common  than  diphtheritic  paralysis. 

Pathology.  —  A  simple  parenchymatous  neuritis,  without  any  inter- 
stitial inflammation,  has  been  found  in  many  cases  of  arsenical  multiple 
neuritis.  In  these  cases  the  chief  change  is  limited  to  a  destruction 
and  degeneration  of  the  myelin  sheath,  the  axis  cylinder  remaining 
intact.  The  myelin  is  broken  into  fragments  or  little  balls,  which  are 
found  in  all  stages  of  fatty  and  granular  degeneration  within  the 
sheath  of  Schwann.  The  nuclei  within  the  sheath  are  increased  in 
number.  This  lesion  has  been  termed  segmental  periaxillary  neuritis, 
as  the  process  may  be  scattered  along  the  nerve  and  may  not  be  con- 
tinuous from  one  segment  to  the  next.  In  severer  cases  the  axis  cyl- 
inder is  destroyed,  being  found  in  all  stages  of  degeneration.  In  these 
cases  many  empty  sheaths  of  Schwann  are  seen. 

Changes  have  also  been  found  in  arsenical  paralysis  in  the  spinal 
cord.  The  same  chromatolysis  in  the  cells  of  the  anterior  horns,  which 
has  been  described  in  alcoholic  paralysis,  has  been  observed,  but  this, 
as  already  stated,  is  of  little  importance.  Henschen  has  found  a  true 
degeneration  in  these  cells,  and  has  also  noticed  an  ascending  degen- 
eration in  the  columns  of  Goll,  which  may  be  due  to  a  primary 
degeneration  in  the  cells  of  the  posterior  spinal  ganglia. 

Symptoms.  —  The  symptoms  of  arsenical  neuritis  do  not  differ 
very  markedly  from  those  of  alcoholic  neuritis.  In  both  there  are 
two  types  of  case  —  one  characterized  by  paralysis,  and  the  other  by 
ataxia.  There  is  little  or  no  difference  in  the  mode  of  onset  of  the 
symptoms  between  cases  of  acute  and  chronic  poisoning. 

There  are  certain  general  constitutional  symptoms  which  first  appear 
in  arsenical  poisoning  and  attract  attention.  If  the  poisoning  is  acute, 
violent  vomiting  and  diarrhoea  occur  at  once,  and  sometimes  save  the 
life  of  the  patient,  the  poison  being  rejected  and  not  absorbed.  If  the 
poisoning  is  chronic,  a  puffiiness  of  the  eyelids  appears,  at  first  most 
noticeable  in  the  morning ;  a  tearful  appearance  of  the  face,  and  some 
catarrh  of  the  nose  is  observed,  and  not  infrequently  gastric  and  en- 
teric symptoms  develop.  There  may  be  pain  in  the  stomach,  nausea 
and  inability  to  retain  food  ;  and  fluid,  green  or  slimy  movements  with 

'Wienermed.  Presse,  1894,  Nr.  15. 


ARSENICAL    NEURITIS.  227 

some  tenesmus.  In  some  cases  a  mild  fever  attends  the  attack,  a  rise 
of  1°  ov  2°  ^.  occurring  and  persisting  for  some  weeks. 

In  arsenical  neuritis  the  first  symptoms  complained  of  are  sensory 
in  type.  Tingling  and  numbness  in  the  feet  and  hands,  burning  of  the 
surface,  tearing  and  shooting  pains,  and  great  soreness  in  the  skin  and 
in  the  calves  of  the  legs  and  feet,  and  soon  a  feeling  of  weakness  in 
the  legs,  great  fatigue  on  walking,  difficulty  in  rising  from  a  seat  or  in 
going  up  stairs,  and  a  staggering  gait  are  noticed.  As  the  general 
feebleness  increases,  paralysis  develops  rapidly  in  some  cases,  in  others 
ataxia  is  more  marked.  The  paralysis  appears  in  the  feet  and  hands, 
the  lower  extremities  being  first  affected.  There  is  the  same  limitation 
of  this  paralysis  to  the  small  muscles  and  to  the  extensor  groups  of 
muscles  of  the  extremities  that  is  seen  in  alcoholic  cases.  The  paralysis 
is  flaccid  in  type,  is  soon  followed  by  atrophy,  which  is  first  noticed  in 
the  interossei  of  the  hands,  and  then  appears  in  the  peronei  of  the  legs 
and  in  the  extensors  of  the  wrists,  and  is  attended  by  loss  of  muscular 
tone  and  mechanical  excitability.  The  reaction  of  degeneration  is 
usually  present.  The  tendon  reflexes  are  diminished  or  lost.  The 
superficial  reflexes  are  normal  or  are  increased.  Drop-wrist  and  drop- 
foot  develop  early  and  may  become  extreme.  The  distribution  of  the 
paralysis  is  always  bilateral  and  symmetrical.  In  severe  cases  the 
patients  are  confined  to  bed,  and  contractures  in  a  flexed  position 
gradually  develop  and  may  become  permanent.  Thus  in  a  case  of 
mine  which  followed  acute  poisoning,  the  hands  were  fixed  in  a  claw 
position  for  many  months  after  all  other  symptoms  had  passed  away. 

When  the  ataxia  precedes  paralysis  the  gait  becomes  unsteady  and 
irregular,  the  patient  sways  in  standing  with  eyes  closed,  he  drags  his 
feet  and  shuffles  along  and  finally  shows  the  "  stepping  gait "  described 
in  alcoholic  cases.  The  dropping  of  the  feet  serves  to  distinguish  this 
gait  from  that  of  tabes,  though  French  writers  have  described  these 
cases  as  pseudo-tabes  arsenicale.  Such  a  gait  was  well  marked  in  both 
patients  with  chorea,  who  developed  arsenical  paralysis  under  my  ob- 
servation. The  ataxia  is  also  present  in  the  hands  and  is  accompanied 
by  an  impairment  or  loss  of  muscular  sense.  It  is  always  attended  by 
a  considerable  degree  of  weakness,  and  this  commonly  goes  on  to 
paralysis  and  the  patients  have  to  go  to  bed. 

In  both  types  of  the  disease,  when  the  symptoms  are  fully  developed, 
the  pain  and  tenderness  are  extreme.  Many  patients  cannot  endure 
the  slightest  touch,  even  the  bedclothes  causing  agony ;  and  the  ex- 
treme tenderness  in  the  muscles  below  the  knees  and  elbows  prevents 
any  manipulation  of  the  extremities.  The  hypersesthesia  is  felt  more 
acutely  in  the  distal  parts  of  the  extremities  aud  rarely  above  the 
elbows  and  knees,  but  when  the  ataxia  and  paralysis  are  fully  devel- 
oped it  renders  the  patient  particularly  helpless.  A  marked  tremor  in 
the  hands  is  usually  an  early  symptom  in  both  types  of  case.  The 
ocular  muscles  and  the  facial  muscles  are  not  affected  and  the  trunk 
muscles,  the  intercostals,  and  the  diaphragm  also  escape.  Occasionally 
erythema,  redness  of  the  skin,  oedema  of  the  extremities,  cyanosis  and 


228  THE    TOXIC   FORMS   OF   MULTIPLE    XEVIilTIS. 

unusual  sweating,  indicate  an  implication  of  vasomotor  and  trophic 
nerve  fibres.  Some  observers  have  reported  a  condition  resembling 
erythromelalgia. 

The  diagnostic  symptom  of  greatest  importance  in  arsenical  neuritis 
is  the  appearance  presented  by  the  skin.  This  becomes  gradually 
darker  from  the  deposit  of  pigment,  so  that  the  complexion  resembles 
that  of  a  gypsy,  and  if  extreme  the  skin  may  finally  turn  almost  black. 
The  pigment  is  first  deposited  about  the  normally  pigmented  regions 
of  the  body,  in  the  axillae  and  groins  and  around  the  nipples.  It  soon 
appears,  however,  on  the  eyelids,  on  the  abdomen  and  thorax,  and 
finally  on  the  surfaces  of  the  arms  and  legs,  the  flexor  surfaces  being 
last  aifected.  The  pigmentation  is  diffuse,  but  here  and  there  little 
islets  of  normal  skin  give  a  mottled  appearance  to  the  surface.  The 
color  is  rather  darker  than  that  seen  in  Addison's  disease,  and  the 
mucous  membranes  are  never  discolored  as  in  that  affection.  The  pig- 
mentation may  be  preceded  by  erythema  and  may  be  followed  by 
various  eruptions,  papillary,  eczematous,  or  herpetic.  Herpes  has  been 
observed  in  many  cases,  usually  on  the  limbs,  sometimes  on  the  body. 
After  a  time  the  skin  desquamates  in  small  flakes,  but  several  desqua- 
mations may  be  necessary  before  a  normal  color  is  regained.  Some- 
times there  is  a  marked  thickening  of  the  skin,  especially  over  the 
knuckles,  on  the  palms  and  soles.  Changes  in  the  nails  are  very 
common,  a  thickening  and  brittle  state  being  evident  and  rough  ridges 
appearing  transversely.  The  appearance  of  normal  nails  growing  out 
above  the  ridges  indicates  beginning  recovery.  In  the  Manchester 
epidemic  there  were  skin  lesions  in  97  per  cent,  of  the  cases.^ 

In  one  case  of  acute  poisoning  by  Paris  green,  a  reddish  eruption 
appeared  on  the  tenth  day  upon  the  hands,  wrists,  and  forearms,  and 
upon  the  aukles  and  feet,  and  in  twenty-four  hours  had  spread  over 
the  entire  body.  Four  days  later  this  had  gradually  disappeared,  and 
then  numbness  was  first  felt  in  the  hands  and  feet.  This  extended 
rapidly,  and  in  three  days  had  reached  elbows  and  knees.  Paralysis 
began  at  the  same  time  as  the  numbness,  and  in  a  week  all  power  of 
movement  below  the  knees  and  elbows  was  lost.  This  remained  for 
two  weeks,  and  then  slowly  passed  off,  recovery  not  being  complete 
for  six  months. 

The  sphincters  are  never  involved  in  arsenical  paralysis. 

Mental  symptoms  have  been  described  by  a  few  observers,  consist- 
ing of  hallucinations,  mild  delusions,  stupor,  and  loss  of  memory.  In 
the  English  epidemic  these  were  observed  only  in  individuals  who 
showed  other  signs  of  chronic  alcoholism,  but  Jolly  has  seen  them  in 
a  non-alcoholic  case.  And  the  fact  that  arsenic  has  a  stimulating 
action  upon  the  brain  is  said  to  be  one  reason  for  its  use  as  a  food  by 
the  Steiermark  peasantry. 

The  symptoms  all  subside  slowly  and  progressively,  and  recovery  of 
power  and  of  coordination  is  accompanied  by  a  relief  from  the  hyper- 
sesthesia  and  the  pain. 

^British  Medical  Journal,  1900,  vol.  ii.,  p.  1725. 


ARSENICAL    NEVRiTlS.  229 

The  duration  of  arsenical  paralysis  is  somewhat  greater  than  that  of 
arsenical  a^xia.  Either  condition  may  last  several  months,  but  occa- 
sionally the  recovery  is  complete  in  a  few  weeks.  Frequently  after 
the  power  has  returned,  the  patient  suffers  for  months  from  numbness 
and  tingling  in  the  extremities,  which  are  sufficient,  as  in  a  case  under 
my  own  observation,  to  interfere  with  the  finer  motions,  to  disturb  the 
sleep,  and  to  cause  constant  discomfort.  According  to  Gerhardt,  97 
per  cent,  recover  wholly.  And  in  the  English  epidemic,  although  the 
number  of  cases  was  very  large,  several  thousand  having  been  ob- 
served in  the  hospitals  and  dispensaries,  the  number  of  deaths  reported 
was  comparatively  small. 

Treatment  consists  in  elimination  of  the  arsenic  by  the  free  use  of 
water  and  in  restoring  the  general  health  of  the  patient.  It  does  not 
differ  in  any  way  from  the  line  of  treatment  for  multiple  neuritis, 
already  described  in  detail  on  page  205. 

The  following  case  is  interesting  on  account  of  its  causation,  and 
offers  a  typical  history  of  the  disease  : 

Female,  aged  thirty  years,  previously  in  good  health,  became  preg- 
nant in  November,  1894,  and  until  March  10,  1895,  suffered  exceed- 
ingly from  the  vomiting  of  pregnancy,  and  became  rapidly  emaciated 
from  inability  to  retain  food,  so  that  she  lost  ninety  pounds  in  weight. 
During  this  time  she  was  confined  to  one  room,  the  furniture  and  wall- 
paper of  which  on  subsequent  examination  were  found  to  contain  about 
two  grains  of  arsenic  to  the  square  yard.  On  March  10th  she  began 
to  suffer  from  tingling  and  numbness  in  the  legs  and  noticed  some  dif- 
ficulty in  stretching  out  her  legs,  and  this  paralysis  increased  rapidly, 
so  that  by  the  15th  of  March  there  was  almost  total  paralysis  of  both 
legs  and  very  great  pain  and  tenderness  in  both  legs,  together  with  a 
sense  of  numbness  and  tingling  in  the  feet  and  legs  as  high  as  the 
knees.  There  was  great  sensitiveness  of  the  legs  to  cold  and  heat, 
and  any  movement  of  the  legs  was  extremely  painful,  the  pain  being 
felt  in  the  muscles.  The  knees  were  preferably  kept  in  a  position  of 
flexion  as  extension  was  too  painful  to  be  endured.  On  the  20th  of 
March  an  extensive  eczema  appeared  all  over  the  body,  but  more  par- 
ticularly in  the  legs  and  arms,  and  was  attended  by  a  deep  brown  pig- 
mentation of  the  skin  and  by  some  desquamation.  On  April  1st,  a 
total  paralysis  had  developed  in  the  feet  and  ankles  and  drop-foot  was 
present  on  both  sides.  The  muscles  below  the  knees  were  very  weak, 
but  the  thighs  could  be  moved  normally.  The  knee-jerks  were  lost. 
Any  attempt  to  stand  was  impossible  on  account  of  the  paralysis  of 
the  feet  and  ankles,  and  the  extreme  pain  that  any  pressure  upon  the 
soles  of  the  feet  produced.  The  muscles  were  flabby  and  the  muscu- 
lar tone  and  mechanical  excitability  were  abolished,  and  faradic  con- 
tractility was  lost  in  the  muscles  below  the  knees.  There  was  no 
affection  of  the  bladder  or  rectum  and  no  tendency  to  bed-sores,  but 
the  nails  of  the  toes  were  discolored  and  rigid,  and  the  nails  ceased 
to  grow  for  three  months.  There  was  a  constant  sensation  of  tingling 
and  numbness  in  the  fingers,  but  no  evidence  of  paralysis  or  anses- 


230  TEE   TOXIC  FORMS   OF  MULTIPLE   NEURITIS. 

thesia.  Her  room  was  changed,  thus  removing  her  from  the  source  of 
the  poison.  The  gastro-iutestinal  catarrh,  which  was  doubtless  due  to 
the  arsenic,  soon  subsided  under  treatment  and  she  began  to  retain  her 
food.  In  Auffust  she  was  able  to  walk  with  a  little  aid.  In  Decern- 
ber  she  still  required  some  help  in  walking,  as  the  foot-drop  persisted, 
and  braces  had  to  be  used  for  the  ankles,  with  high-heeled  shoes,  and 
at  this  time  her  legs  were  still  stiff  and  numb.  There  were  no  knee- 
jerks,  and  sensations  of  pain,  heat,  and  cold  were  much  less  quickly 
and  keenly  perceived  below  the  knees  than  above,  or  upon  the  hands. 
In  April,  1896,  she  had  recovered  entirely. 

MULTIPLE  NEURITIS  DUE  TO  POISONING  BY  LEAD. 

Etiology. — It  is  said  that  the  use  of  lead  enters  into  111  industries. 
Poisoning  by  lead  is  always  a  chronic  poisoning  and  usually  occurs  in 
painters,  in  plumbers,  in  typesetters,  or  in  workers  in  lead  objects  such 
as  toys,  who  come  in  contact  with  lead,  either  in  the  form  of  metallic 
lead  or  of  some  of  the  salts  of  lead  which  are  used  in  mixed  paints. 
Painters  who  work  in  the  open  air  are  less  liable  to  lead  poisoning  than 
those  who  labor  in  rooms ;  and  those  are  most  liable  who,  like  the 
varnishers  in  carriage  factories  and  furniture  establishments,  scrape  off 
old  paint  preparatory  to  putting  on  a  new  coat.  Any  work  which 
results  in  the  production  of  dust  which  may  be  mixed  with  lead,  or  its 
salts,  may  be  productive  of  lead  poisoning.  It  is  evident  that  the 
inhalation  of  the  dust  is  one  method  of  the  entrance  of  the  poison  into 
the  body.  Laborers  who  work  in  factories  where  china  earthenware 
is  produced  and  where  glazes  and  fretted  glass  are  manufactured  are 
very  liable  to  lead  poisoning.  In  the  midland  counties  of  England, 
where  such  industries  are  common,  in  Limoges,  France  ;  in  Dresden, 
Germany,  and  in  Trenton,  New  Jersey,  many  cases  are  seen  annually. 

The  usual  method  of  poisoning,  however,  is  by  swallowing  the  lead, 
and,  as  many  painters  do  not  properly  clean  the  hands  before  taking 
their  noonday  meal,  the  poisoning  is  often  acquired  in  this  way. 
Some  individuals,  however,  appear  to  be  extremely  susceptible  to  lead 
poisoning.  Thus,  I  have  known  most  serious  cases  in  children  of 
painters  who  could  only  have  obtained  the  poison  by  inhalation  of  the 
fumes  of  paints  that  were  kept  in  the  room.  I  have  known  small 
amounts  of  lead  to  produce  severe  poisoning  when  taken  into  the 
stomach.  Beer  bottles  are  frequently  cleaned  by  being  shaken  with 
lead  shot  within  them,  and  I  have  known  lead  poisoning  to  occur 
from  drinking  the  beer  from  such  bottles.  I  have  known  lead  poison- 
ing to  occur  in  servants  who  drank  the  water  first  drawn  from  the 
spigot  early  in  the  morning,  water  which  had  stood  in  the  lead  pipes 
all  night  and  had  thus  become  impregnated  with  some  of  the  soluble 
salts  of  lead.  I  have  known  severe  cases  in  typesetters  whose  fingers 
were  stained  by  the  constant  handling  of  metallic  lead.  I  have  also 
known  it  in  plumbers  after  long  labor  in  laying  lead  pipes.  Horses 
are  subject  to  lead  palsy.     Lead  is  not  frequently  used  as  a  medicine, 


MULTIPLE  NEUBITIS  DUE   TO   POISONING  BY  LEAD.        231 

and  I  have  never  known  a  case  of  poisoning  from  this  source.  Alco- 
holic subjects  and  those  who  use  tobacco  in  excess  are  more  liable  to 
develop  lead  palsy  than  others. 

Pathology.  —  The  pathology  of  lead  palsy  has  been  a  matter  of 
dispute  among  authorities  for  many  years.  Many  writers  have  found 
changes  in  the  cells  of  the  anterior  horns  of  the  spinal  cord,  while 
others,  more  recently,  have  maintained  that  the  changes  are  limited  to 
degeneration  of  the  peripheral  nerve  trunks.  The  establishment  of 
the  neurone  theory  has  thrown  light  upon  these  differences  of  opinion, 
and  the  discovery  of  more  delicate  methods  of  staining  by  Nissl  and 
Marchi  have  demonstrated  that  the  entire  motor  neurone  may.  be 
affected  in  this  disease.  The  most  evident  changes  are  a  degenerative 
neuritis  with  atrophy .  in  the  peripheral  nerves,  chiefly  in  the  radial 
nerve  but  not  infrequently  in  many  of  the  peripheral  branches  of  the 
brachial  plexus.  The  degree  of  this  degeneration  and  its  extent  de- 
pends upon  the  severity  of  the  poisoning.  There  are  few,  if  any, 
changes  in  the  neurilemma  or  connective  tissue  sheath,  hence  this  form 
of  neuritis  is  purely  a  parenchymatous  neuritis.  While  the  majority 
of  these  changes  are  limited  to  the  terminal  filaments  of  the  nerves, 
there  may  be  slight  changes  detected  in  the  trunk  of  the  nerves  and  in 
the  anterior  nerve  roots.  The  method  of  Nissl  demonstrates  chroma- 
tolysis  in  the  cells  of  the  spinal  cord  and  in  those  of  the  spinal  ganglia, 
but  these  are  not  usually  of  a  sufficient  degree  to  produce  degeneration 
or  complete  atrophy  that  is  irreparable.  In  the  severer  cases,  how- 
ever, vacuolization  of  the  cell  and  final  degenerative  changes  may  take 
place.  Changes  of  a  similar  kind  have  also  been  found  in  the  large 
cells  of  the  brain  cortex.  The  serious  lesions  are  more  commonly 
found  in  the  peripheral  nerves  than  in  the  spinal  cord. 

Symptoms. — In  93  per  cent,  of  cases  the  onset  of  the  paralysis  is 
preceded  by  a  severe  attack  of  lead  colic.  The  patient  suffers  from 
intense  pain  in  the  region  of  the  umbilicus,  attended  by  obstinate  con- 
stipation, very  often  by  nausea  and  vomiting.  The  pain  is  supposed 
to  be  due  to  an  irritation  of  the  visceral  nerves  by  the  lead.  It  may 
also  be  due  to  distention  of  the  intestines  from  paralysis  of  these  nerves 
and  cessation  of  peristaltic  action.  There  is  a  characteristic  blue  line 
seen  along  the  gums,  not  to  be  mistaken  for  staining  of  the  teeth  be- 
cause of  its  location  and  clear  blue  color.  Anaemia  is  almost  always 
present  in  patients  who  have  been  poisoned  by  lead.  Another  com- 
mon affection  in  persons  exposed  to  lead  poisoning  is  chronic  articular 
rheumatism.  Thus,  Tanqueral  found  that  in  1,217  patients  who  suf- 
fered from  lead  colic,  755  developed  painful  joints,  107  developed 
paralysis,  and  72  suffered  from  headache. 

A  few  days  after  the  onset  of  an  attack  of  colic,  or  sometimes  not 
until  two  or  three  weeks  after  the  attack  has  subsided,  the  patient  is 
suddenly  seized  with  paralysis  of  the  extensor  muscles  of  the  fingers, 
thumb,  and  wrist.  It  is  a  rule  for  one  hand  to  be  affected  several 
days  before  the  other,  and  occasionally  both  hands  are  unequally 
affected  throughout  the  disease.  It  is  quite  commonly  found  that 
the  paralysis  of  the  extensors  of  the  index  finger  or  of  the  thumb 


232  TBE   TOXIC  FORMS   OF  MULTIPLE   NEURITIS. 

precedes  the  paralysis  of  the  other  muscles.  As  a  consequence  of  this 
paralysis  the  wrists  drop  and  cannot  be  raised,  and  from  lack  of  ful- 
crum power  the  flexors  are  apparently  weakened.  If,  however,  the 
wrists  be  extended  by  the  examiner  and  held,  it  will  be  found  that  the 
flexor  power  in  the  hand  and  fingers  is  not  impaired.  The  supinator 
longus  muscle  is  rarely,  if  ever,  affected,  and  hence  it  is  apparent  that 
the  lesion  is  limited  to  the  distribution  of  the  radial  branch  of  the 
musculospiral  nerve  below  the  point  where  the  nerve  to  this  muscle  is 
given  ofi;  There  is  very  often  a  tremor  in  the  hands  and  fingers, 
which  comes  on  early  and  remains  until  the  paralysis  subsides.  There 
is  no  affection  of  sensation  as  a  rule,  though  sometimes  tingling  and 
numbness  is  felt  in  the  back  of  the  hand  or  in  the  fingers.  The 
muscles  that  are  affected  rapidly  undergo  atrophy  of  an  extreme  type, 
so  that  within  a  couple  of  weeks  of  the  onset  the  back  of  the  forearm 
presents  a  very  atrophic  appearance  and  the  muscles  show  the  reaction 
of  degeneration. 

As  a  result  of  the  drop-wrist  a  deformity  of  the  back  of  the  hand 
soon  appears,  due  to  a  displacement  backward  of  the  internal  bones  of 
the  wrist,  forming  a  hard  protuberance  upon  the  back  of  the  hand 
near  the  base  of  the  metacarpal  bones ;  but  firm  contractures  or  per- 
manent shortening  in  the  paralyzed  muscles,  such  as  occurs  in  alcoholic 
or  arsenical  cases,  does  not  develop  in  lead  palsy.  Not  infrequently, 
if  the  disease  is  a  severe  one,  the  intrinsic  muscles  of  the  hands  also 
suffer  early  and  the  hands  become  quite  useless,  apposition  of  the 
thumb  to  the  fingers  and  any  abduction  or  extension  of  the  thumb  and 
fingers  being  impossible.  Then  it  is  evident  that  branches  of  the  ulnar 
and  median  nerves  are  also  involved. 

The  reaction  of  degeneration  appears  early  in  the  muscles,  and  the 
faradic  reaction  does  not  return  until  the  patient  has  been  well  for 
some  months. 

In  the  majority  of  cases  the  paralysis  does  not  go  beyond  the  mus- 
cles of  the  forearms  and  hands,  and  after  a  period  of  five  to  six  months 
there  is  a  gradual  return  of  power  and  complete  recovery.  The 
patients  often  complain  of  severe  cramps  in  the  muscles  —  a  form  of 
pain  which  may  even  prevent  sleep  —  but  pain  is  not  a  common  symp- 
tom in  lead  palsy. 

The  disease  is  a  very  slow  one  and  the  patients  are  rarely  able  to 
return  to  work  within  six  or  seven  months  of  the  onset.  As  their 
livelihood  depends  upon  their  labor,  it  is  well  that  this  should  be  un- 
derstood from  the  outset,  so  that  during  the  period  of  incapacity  they 
may  seek  some  other  occupation  which  will  enable  them  to  support 
life.     Relapses  are  common  when  patients  return  to  their  work. 

In  more  serious  cases  the  paralysis  may  appear  in  the  muscles  of  the 
shoulder,  the  deltoid  being  first  affected,  and  then  the  biceps,  brachialis 
anticus  and  supinator  longus  being  involved.  Occasionally  the  mus- 
cles of  the  legs  below  the  knees  become  paralyzed,  the  peroneal  group 
being  more  commonly  invaded,  though  in  severe  cases  the  extensors 
of  the  toes  and  even  the  anterior  tibial  group  may  be  equally  paralyzed. 


MULTIPLE   NEURITL^  DUE   TO   POISONING   BY  LEAD.  233 

This  only  occurs  in  4  per  cent,  of  the  cases  and  has  been  observed 
chiefly  in  children.  It  .causes  drop-feet  and  a  stepping  gait.  In  the 
most  severe  type  the  patient  is  almost  totally  paralyzed.  Fibrillary 
twitchings  are  occasionally  seen  in  the  muscles,  but  they  are  not  the 
rule.  A  general  sensory  disturbance  is  common,  the  patients  suffering 
from  intense  pain  in  the  arms  and  legs,  from  anaesthesia  of  the  hands 
and  feet,  and  from  very  disagreeable  sensations  of  pricking  and  ting- 
ling. The  sphincters  are  never  involved.  Vasomotor  and  trophic 
symptoms  are  usually  absent,  though  the  hands  and  arms  may  be  cold, 
cyanotic,  and  may  perspire  too  freely. 

In  the  severe  cases  it  is  possible  to  demonstrate  a  condition  of 
tenderness  along  the  affected  nerves,  but  in  slighter  cases  no  tender- 
ness is  felt.  A  type  of  lead  palsy  has  been  described  in  which  the 
symptoms  appear  first  or  exclusively  in  the  cranial  nerves ;  the  laryn- 
geal, the  pharyngeal,  the  lingual,  the  ocular,  the  facial,  and  the  optic 
nerves  having  been  affected.  In  these  cases  headache,  vertigo,  and 
mental  apathy  indicate  that  the  brain  as  well  as  the  nerves  is  invaded 
by  the  poison.  This  type  is  very  rare.  It  is  termed  lead  encephal- 
opathy by  the  German  writers. 

The  marked  pallor  and  evident  anaemia  present  in  all  cases  is  con- 
firmed by  the  examination  of  the  blood  ;  the  hemoglobin  is  low  and  a 
peculiar  granular  appearance  of  the  red  cells  is  found. 

Prognosis  is  good  as  to  recovery,  but  the  duration  of  the  disease,  as 
already  stated,  even  in  the  lighter  type,  is  six  months,  and  in  the  more 
severe  type  very  often  a  year.  The  following  case  illustrates  a  severe 
condition  of  chronic  poisoning : 

Male,  aged  thirty-eight  years,  after  suffering  from  an  attack  of  lead 
colic,  began  to  feel  numbness  and  pain  in  his  legs  and  feet,  which  soon 
extended  to  his  hands  and  forearms.  This  steadily  increased  for  two 
weeks,  and  to  it  was  added  paralysis  of  the  extensors  of  both  hands 
and  both  feet,  so  that  at  the  end  of  that  time  he  was  unable  to  use  his 
hands  or  to  stand.  With  the  paralysis  there  was  rapid  atrophy  of  the 
muscles,  and  a  decline  in  the  faradic  excitability.  The  muscles  became 
more  and  more  tender,  and  the  spontaneous  pains  gradually  increased 
until  it  was  necessary  to  use  opium  freely  to  quiet  him.  By  the  end 
of  a  mouth  a  well-marked  anaesthesia  had  developed  in  the  hands  and 
below  the  knees,  and  it  was  noticed  that  the  atrophied  muscles  were  in 
a  constant  tremor.  There  were  wrist-drop  and  foot-drop  on  both  sides, 
and  all  reaction  to  faradism  ceased.  It  required  a  very  strong  gal- 
vanic current  to  produce  contraction  and  AGO  was  greater  than  KCG. 
The  knee-jerk  was  preserved. 

The  muscular  sense  was  impaired.  For  five  months  his  condition 
remained  stationary,  in  spite  of  treatment  by  strychnine,  iodide  of 
potash,  massage,  and  electricity.  During  a  month  of  his  illness  he 
was  delirious  at  night,  was  excited  in  the  daytime,  and  subject  to  de- 
lusions and  had  little  memory  or  mental  capacity  ;  but  these  symptoms 
gradually  subsided.  Then  a  gradual  improvement  set  in,  the  pains 
became  less  severe,  sensation  returned,  the  muscles  regained  their  con- 


234  TEE    TOXIC    FOBMS    OF    MULTIPLE    NEURITIS. 

tour  and  strength,  and,  finally,  the  electric  contractility  returned  to  the 
normal  standard.  About  a  year  from  the  onset  of  the  symptoms  the 
recovery  was  complete. 

Treatment.  —  Treatment  of  the  lead  colic  is  oy  means  of  full  doses 
of  opium  to  quiet  the  pain,  and  by  laxative  saline  salts,  such  as  sul- 
phate of  magnesia,  to  relieve  the  constipation.  Large  draughts  of  water 
should  be  given  constantly,  in  order  to  eliminate  the  poison,  which 
passes  off  partly  through  the  kidneys.  During  the  stage  of  chronic 
poisoning  the  patient  should  be  instructed  to  drink  a  great  deal  of 
water,  and  five  grains  of  iodide  of  potash  may  be  given  three  times  a 
day  to  assist  in  the  elimination  of  the  lead.  I  have  never  been  able 
to  see  that  strychnine,  which  is  usually  recommended,  has  any  effect 
upon  the  progress  of  regeneration,  though  general  tonics  —  cod-liver 
oil,  iron,  and  quinine  —  may  well  be  employed,  as  these  patients  are 
uniformly  anaemic.  Treatment  of  the  paralysis  is  by  massage  and 
electricity,  galvanism  being  the  only  current  which  will  produce  any 
effect. 

Multiple  neuritis  of  the  ataxic  type,  due  to  poisoning  by  phosphorus, 
has  been  recorded  by  Henschen  in  one  case,  and  in  seven  other  cases 
of  acute  poisoning  he  found  mild  nervous  symptoms  of  parsesthesia 
and  tenderness  along  the  nerves.  The  condition  must  be  a  very  rare 
one,  as  it  has  not  been  seen  by  others.  Inquiry  among  employes  in 
match  factories  failed  to  elicit  any  knowledge  of  a  special  disease 
peculiar  to  this  occupation. 

Mercurial  poisoning  has  been  said  to  cause  multiple  neuritis,  but  no 
case  can  be  found  which  is  not  open  to  objection.  The  tremor  which 
is  produced  by  acute  mercurial  poisoning,  and  which  is  observed  in 
workers  in  looking-glass  factories,  is  accompanied  by  headache,  vertigo, 
and  occasionally  by  hemiplegia  or  monoplegia  of  cerebral  type,  and  is 
not  followed  by  multiple  neuritis.  Chronic  mercurial  poisoning  after 
or  during  the  treatment  of  syphilis  also  fails  to  cause  neuritis. 

Gowers^  has  described  a  case  of  poisoning  by  silver,  in  which  par- 
alytic symptoms  developed  in  the  arms  with  drop-wrists,  resembling 
closely  those  due  to  lead  poisoning.  The  characteristic  blue  coloring 
of  the  skin  was  present.  The  patient  died  of  cancer  and  no  autopsy 
was  made.     This  is  the  only  case  thus  far  observed. 

A  few  cases  have  been  recorded  ^  of  multiple  neuritis  due  to  poison- 
ing by  copper  occurring  among  brass  workers.  In  these  cases  the 
symptoms  resembled  those  of  the  ataxic  type  of  neuritis  seen  in  alco- 
holics.    They  are  so  rare  as  to  require  mention  only. 

When  platinum  is  dissolved  in  aqua  regia  red  fumes  of  oxides  of 
nitrogen  are  evolved  which  are  very  toxic.  In  a  workman  affected  by 
these  fumes  a  painful  condition  of  multiple  neuritis  developed  which 
ran  a  long  and  severe  course  but  finally  recovered. 

^Diseases  of  the  Nervous  System,  German  edition,  vol.  iii.,  p.  ooo. 
'^  Suckling.     British  Medical  Journal,  1888,  vol.  ii.,  p.  1334.     Walton  and  Carter, 
American  Journal  of  the  Medical  Sciences,  1892,  vol.  ii.,  p.  61. 


CHAPTER  X. 

THE   INFECTIOUS   AND   TOXEMIC   FORMS   OF   MULTIPLE 

NEURITIS. 

'  Multiple  Neuritis  Subseguent  to  Diphtheria  ;  to  the  Grippe  ;  to  Typhoid,  Typhus,  and 
Malarial  Fever ;  to  Scarlet  Fever,  Measles,  Whooping-cough,  and  Smallpox  ;  to 
Erysipelas  and  Septicaemia  ;  to  Gonorrhoea  and  Puerperal  Fever.     Leprous  Neuritis. 

MULTIPLE  NEURITIS  SUBSEQUENT  TO  DIPHTHERIA. 

Diphtheritic  paralysis  is  one  of  the  most  common  forms  of  mul- 
tiple neuritis,  but  does  not  occur  as  frequently  as  a  sequel  in  cases  that 
are  treated  by  antitoxin  as  in  those  which  are  allowed  to  run  a  normal 
course.  Rosenau  and  Anderson  ^  have  shown  that  in  guineapigs  one 
unit  of  antitoxin  injected  on  the  first  day  prevents  paralysis  while  4000 
units  injected  after  forty-eight  hours  fails  to  prevent  paralysis.  After  the 
paralysis  has  appeared  antitoxin  fails  to  affect  it  in  any  way.  The 
number  of  cases  of  diphtheritic  paralysis  appearing  at  my  clinic  since 
the  use  of  antitoxin  has  been  much  less  than  in  any  previous  period. 
Rothe  ^  found  64  cases  of  diphtheritic  paralysis  in  744  cases  of  diph- 
theria treated  at  the  Charite  Clinic  in  Berlin,  which  gives  a  frequency 
of  8.7  per  cent,  of  the  cases  of  diphtheria  followed  by  paralysis.  This 
observation  is  in  accord  with  that  of  other  observers.^  Bernhardt 
called  attention  to  the  fact  that  in  many  cases  of  diphtheria  there  is  a 
loss  of  the  knee-jerk,  a  fact  which  seems  to  him  to  point  to  a  probable 
susceptibility  of  the  entire  nervous  system  to  the  diphtheritic  poison, 
but  Rothe  found  a  loss  of  the  knee-jerk  in  but  one-half  of  the  cases 
which  were  paralyzed.  It  is  certainly  a  fact  that  for  many  weeks 
after  the  disappearance  of  all  symptoms  of  diphtheria,  even  in  cases 
which  show  no  paralysis,  the  knee-jerk  may  be  absent. 

Pathology.  —  The  pathology  of  diphtheritic  paralysis  has  been  a 
matter  of  much  discussion.  Some  authors  maintain  that  the  lesion  is 
a  degenerative  neuritis  only,  while  others  maintain  that  .the  lesions  are 
central  in  the  spinal  cord  and  brain  axis.  A  parenchymatous  neuritis 
is  the  chief  lesion,  but  occasionally  a  diffuse  process  with  involvement 
of  the  interstitial  tissues  has  been  observed.  In  the  finest  nerves  of 
the  muscles  and  in  the  skin,  the  destructive  process  is  more  fully  de- 
veloped, as  a  rule,  than  in  the  trunks  of  the  nerves  or  in  the  larger 
nerves.  The  neuritis  is  more  pronounced  in  the  cranial  nerves  in 
diphtheritic  paralysis  than   in   any  other  form  of  multiple  neuritis. 

»  P>ulletin  No.  38,  U.  S.  Marine-Hospital  Service,  1907. 
2  Inaug.  Dissert.,  Berlin,  1899. 
»  Wollacott.     Lancet,  August  20,  1899. 
235 


236  INFECTIOUS   AND   TOXEMIC  POUUS   OF   NEURITIS. 

Lesions  of  the  anterior  and  posterior  nerve  roots  have  been  found  by 
Dejeriue,  and  many  authors  have  described  parenchymatous  degenera- 
tion in  the  anterior  horn  cells  and  in  the  posterior  spinal  ganglia. 

Jlecent  experimental  investigations  by  Murawjeff^  show  that  the 
toxin  of  diphtheria  produces  chromatolysis  and  degeneration  in  the 
cell  bodies  of  both  motor  and  sensory  neurones.  He  also  finds  that 
it  attacks  the  peripheral  nerves  as  well ;  in  some  cases  before,  in 
others  after  it  has  attacked  the  neurone. 

Symptoms. — In  the  lighter  forms  of  diphtheritic  paralysis  the  soft 
palate  is  the  only  part  that  is  affected.  In  472  cases  of  diphtheritic 
paralysis  observed  by  Wollacott  the  soft  palate  was  affected  in  413, 
the  difficulty  of  swallowing,  with  regurgitation  of  fluids  through  the 
nose  and  a  thickening  and  indistinctness  of  the  voice  being  the  only 
symptoms  present.  There  is  a  loss  of  reflex  in  the  throat  in  these 
cases  and  a  lowered  sensitiveness  of  the  pharynx,  so  that  irritation 
does  not  cause  acts  of  swallowing.  The  limitation  of  the  paralysis  to 
the  palate  has  been  explained  by  supposing  that  the  poison  of  the  dis- 
ease has  a  direct  action  upon  the  terminal  filaments  of  the  nerves 
which,  in  this  position,  are,  as  it  were,  dipped  constantly  in  the  poison. 
This  theory  is  supported  by  a  case  in  which  paralysis  of  the  abdominal 
muscles  was  associated  with  a  diphtheritic  inflammation  of  the  navel 
in  a  newborn  child.  But  the  more  serious  cases  prove  that  it  is 
through  the  blood  that  the  poison  is  carried  to  nerves  far  removed  from 
the  diphtheritic  inflammation. 

Next  in  frequency  to  paralysis  of  the  palate  occurs  paralysis  of 
some  of  the  ocular  muscles,  producing  internal  squint  and  double 
vision.  This  paralysis  may  affect  any  one  or  many  of  the  muscles 
moving  the  eyeball  of  one  or  both  eyes,  though  it  is  less  common  for 
the  levator  palpebrse  to  be  affected  than  any  other  muscle.  The  ex- 
ternal recti  are  more  often  affected  than  the  internal,  and  it  is  not 
common  to  find  all  the  muscles  supplied  by  the  oculomotor  nerve 
affected  together.  Among  Wollacott's  472  cases,  104  had  some  ocular 
palsy.  The  paralysis  of  accommodation  prevents  reading.  The 
paralysis  may  not  extend  to  any  other  muscles  than  those  already 
named,  and  in  the  lighter  cases  remains  in  them  for  a  period  of  four 
to  ten  weeks  and  then  gradually  passes  off. 

Not  infrequently,  however,  the  disease  extends  much  more  widely 
and  after  a  week  or  more  of  local  palsy  of  the  throat  or  eyes,  or  both, 
the  individual  develops  within  twenty-four  hours  a  widespread  paral- 
ysis of  both  arms  and  both  legs.  This  is  usually  preceded  or  attended 
by  incoordination  of  movement  in  all  the  finer  adjusting  actions  of  the 
hands  and  by  ataxia  in  the  act  of  walking.  Associated  with  this 
ataxia  and  with  the  weakness  of  the  muscles  there  are  frequently  drop- 
wrist  and  drop-foot,  so  that  the  patient  exhibits  the  stepping  gait 
already  described  as  occurring  in  alcoholic  neuritis. 

There  are  also  sensory  symptoms  consisting  of  numbness  in  the  ex- 
tremities, disturbance  of  sensation  both  of  touch,  temperature,  and  pain 
and  of  the  muscular  sense  and  delayed  sensations  of  pain.     Shooting 

^Neurolog.  Centralbl.,  1898,  p.  475. 


MULTIPLE    NEURITIS   SUBSEQUENT    TO    DIPHTHERIA.  237 

pains  are  sometimes  felt,  but  are  not  very  prominent  and  there  is 
rarely  tend^ness  along  the  nerve  trunks.  If  the  paralysis  is  not  so 
extreme  as  to  suspend  all  movement,  a  tremor  is  not  uncommonly  ob- 
served in  the  motions  of  the  fingers.  The  tendon  reflexes  are  lost  and 
mechanical  excitability  of  the  muscles  disappears  ;  a  rapid  atrophy 
with  reaction  of  degeneration  develops  in  the  paralyzed  muscles. 
Paralysis  of  both  sides  of  the  face  may  occur. 

In  a  still  more  severe  type  of  case,  either  soon  after  the  onset  or 
after  the  paralysis  has  been  present  for  several  weeks,  bulbar  symp- 
toms may  appear,  with  great  difficulty  in  swallowing,  atrophy,  and 
weakness  of  the  tongue,  spasms  of  coughing  (which  may  be  danger- 
ous), weakness  of  the  face,  and  paralysis  of  the  vocal  cords.  Such  a 
complication  is  extremely  grave,  as  the  patient  may  die  of  respiratory 
paralysis,  of  suffocation,  or  of  pneumonia  due  to  the  inhalation  of  food. 
This  occurs  in  10  per  cent,  of  the  cases.  The  onset  of  the  paralysis 
may  occur  within  a  week  of  the  infection  or  it  may  be  delayed  for 
several  weeks.  In  Rothe's  cases  the  paralysis  developed  in  the  major- 
ity in  the  second  week,  but  in  a  few  over  forty  days  had  elapsed 
between  the  initial  symptoms  and  the  development  of  paralysis. 

Diphtheritic  paralysis  is  not  by  any  means  confined  to  children,  but 
occurs  in  adults  and  even  in  old  people.  Not  infrequently  it  is  impos- 
sible to  ascertain  a  definite  history  of  diphtheria,  and  a  very  light 
attack  of  diphtheritic  sore  throat  may  be  followed  by  a  severe  attack 
of  diphtheritic  paralysis.  The  occurrence  of  paralysis  of  the  throat 
and  eye  muscles  and  a  marked  ataxic  condition  of  the  limbs  enable 
one  to  make  a  diagnosis,  even  in  cases  where  no  sore  throat  has 
occurred. 

Prognosis. — The  prognosis  in  diphtheritic  paralysis  is  good,  pro- 
vided the  condition  is  limited  to  paralysis  of  the  throat,  or  of  the  eyes, 
or  to  a  general  diphtheritic  ataxia.  If,  however,  a  true  paralysis  of 
the  muscles  of  the  limbs  ensues ;  if  the  respiratory  muscles  are  aflFected, 
or  if  the  pneumogastric  nerve  is  invaded,  causing  frequency  of  the 
pulse,  the  prognosis  is  very  serious. 

Treatment.  —  The  treatment  of  diphtheritic  paralysis  is  that  of 
multiple  neuritis.  The  danger  of  inhalation  of  food  or  of  imperfect 
nutrition  from  difficulty  of  swallowing  is  not  to  be  forgotten,  and  in  the 
extreme  cases  it  may  be  well  to  feed  the  patient  with  a  stomach  tube 
rather  than  to  allow  them  to  attempt  to  swallow,  if  this  be  productive 
of  fits  of  choking  and  coughing.  Inasmuch  as  all  effiart  seems  to  in- 
crease the  severity  of  the  symptoms,  it  is  well  that  these  patients 
should  be  kept  in  bed  as  long  as  there  is  any  difficulty  of  motion. 
Strychnine  is  of  a  great  deal  of  service  in  the  treatment  of  diphtheritic 
paralysis.  It  was  for  a  time  supposed  to  be  a  specific  for  the  disease, 
but  this  was  at  a  time  when  the  lesion  was  thought  to  be  central.  It 
is  not  to  be  used  in  very  large  doses,  -^wo  ^^  ^  grain  every  three  hours 
being  sufficient  for  an  adult.  It  has  not  seemed  in  my  experience  to 
shorten  the  duration  of  the  paralysis. 

Tiie  following  cases  are  good  examples  of  the  ordinary  type  of 
diphtheritic  paralysis : 


238  INFECTIOUS   AND    T0X2EMIC   FORIIH   OF   NEURITIS. 

Male,  two  and  a  half  years  of  age,  suffered  from  measles  during  the 
month  of  January,  1894,  and  on  the  first  of  February  developed  symp- 
toms of  diphtheria.  This  ran  its  ordinary  course,  though  it  was  nec- 
essary to  perform  intubation  and  the  tube  was  retained  about  one  week. 
He  then  gradually  recovered  and  after  a  week  appeared  to  regain  his 
ordinary  strength  ;  his  appetite  improved,  and  he  could  stand  aud  run 
about.  On  the  20th  of  March  he  was  suddenly  seized  with  paralysis, 
which  within  two  days  had  become  most  extensive.  He  then  had  ex- 
treme strabismus  due  to  a  paralysis  of  both  internal  recti.  There  was 
no  paralysis  of  the  face,  tongue,  or  palate,  and  he  could  talk  well ;  but 
there  was  complete  paralysis  of  the  muscles  moving  the  head  and  of 
both  arms  and  both  legs,  and  of  the  body.  He  was  unable  to  sit  up, 
to  move  his  head,  or  to  move  any  of  the  extremities.  There  was  no 
knee-jerk.  There  was  no  disturbance  of  the  bladder  or  rectum,  there 
was  no  pain  or  tenderness ;  but  respiration  was  difficult  and  his  pulse 
was  140.     On  the  24th  of  March  he  died  of  respiratory  paralysis. 

A  boy  aged  thirteen  years,  after  an  attack  of  diphtheria  developed 
paralysis  of  the  soft  palate.  One  week  after  the  paralysis  of  the  pal- 
ate he  noticed  a  disturbance  of  vision  which  proved  on  examination  to 
be  due  to  a  total  paralysis  of  all  the  muscles  of  the  eyeballs.  The 
pupil  reflex  to  light  was  preserved ;  but  there  was  no  reaction  in  ac- 
commodation. This  paralysis  of  the  eyeballs  came  on  within  twenty- 
four  hours,  and  at  the  same  time  he  developed  a  very  marked  degree 
of  ataxia.  He  swayed  in  standing  with  eyes  closed,  and  his  gait  was 
exactly  like  that  of  a  well-marked  case  of  tabes.  The  motions  of  the 
hands  were  also  disturbed,  and  all  the  tests  demonstrated  an  extreme 
degree  of  inco5rdination.  The  knee-jerks  were  lost ;  the  skin  reflexes 
were  present.  In  addition  to  the  ataxia  there  was  actual  weakness  of 
the  muscles,  which  was  more  evident  in  the  arms  and  hands  than  in 
the  lower  extremities.  He  had  formication  in  the  legs,  but  not  in  the 
hands,  and  there  were  no  lightning  pains.  He  had  great  difficulty  in 
swallowing  solids  as  well  as  liquids,  which  seemed  to  point  to  some  in- 
volvement of  the  muscles  of  deglutition  as  well  as  of  the  soft  palate. 
This  condition  remained  stationary  for  about  three  weeks,  and  then  all 
the  symptoms  began  to  pass  off  gradually.  His  recovery  was  not 
complete  until  six  months  after  the  onset. 

A  girl,  aged  thirteen  years,  after  suffering  for  a  few  days  from  a  slight 
sore  throat,  found  herself  unable  to  see  clearly  because  of  the  develop- 
ment of  a  paresis  of  accommodation.  There  followed  a  slowly  pro- 
gressing ataxia  of  the  upper,  and  later  of  the  lower,  limbs,  with  un- 
certain gait,  disturbance  of  touch,  pain,  temperature,  and  muscular 
senses,  with  delayed  sensation  of  pain  aud  loss  of  tendon  reflexes.  At 
first  she  felt  shooting  pains  in  the  limbs,  but  later  these  ceased.  There 
was  at  no  time  tenderness  of  the  nerves  to  pressure.  After  three 
months  bulbar  symptoms  appeared,  atrophy  with  paresis  of  the  tongue, 
difficulty  in  swallowing,  spasms  of  coughing,  weakness  of  voice,  and 
paralysis  of  the  palate.  The  muscles  of  the  hands  began  to  atrophy 
about  the  time  that  the  bulbar  symptoms  commenced  and  soon  were 
useless.     In  the  atrophic  muscles  the  faradic  reaction  was  absent,  the 


MULTIPLE    NEURITIS    SUBSEQUENT    TO    THE  GRIPPE.  239 

galvanic  much  reduced  and  very  slow.     Nine  months  after  the  onset 
she  died  of  pneumonia. 

The  autopsy  showed  the  brain  and  spinal  cord  to  be  normal.  In 
all  the  peripheral  nerves  of  the  extremities,  as  well  as  in  the  hypo- 
glossal and  recurrent  laryngeal  nerves,  well-marked  atrophic  degenera- 
tion was  found,  with  destruction  of  axis  cylinders  and  medullary 
sheaths,  and  with  thickening  and  increase  of  nuclei  in  the  connective- 
tissue  sheaths.^ 

MULTIPLE  NEURITIS  SUBSEQUENT  TO  THE  GRIPPE. 

Severe  attacks  of  neuralgia  accompany  or  follow  the  grippe  in  about 
15  per  cent,  of  the  cases,  and  are  an  evidence  of  direct  action  of  the 
poison  upon  the  nerve  trunks.  Thus  trigeminal  neuralgia,  occipital 
neuralgia,  intercostal  neuralgia,  and  sciatica  frequently  develop  and 
sometimes  run  a  very  severe  and  long  course.  They  are  occasionally 
bilateral  and  symmetrical,  which  is  rare  under  other  conditions. 

Many  cases  of  local  neuritis  appearing  in  one  or  in  several  nerves 
in  the  body  at  once  have  been  observed  after  the  grippe.  Any  of  the 
cranial  or  spinal  nerves  may  be  involved. 

It  is  not  uncommon  for  such  a  neuritis  to  attack  symmetrical  nerves 
on  both  sides  of  the  body.  Thus  I  have  seen  both  perouei  affected, 
both  ulnar  nerves  affected,  both  median  nerves  affected,  and  the  brach- 
ial plexus  affected  on  both  sides.  In  all  these  cases  the  neuritis  ran  a 
typical  course. 

Draper,^  Remak,^  and  Bernhardt  ^  recorded  cases  of  multiple  neuritis 
accompanied  by  acute  ataxia  following  the  first  epidemic  of  influenza, 
1890.  Mills,^  Buzzard,^  Eisenloch,''  Savage,^  and  others  subsequently 
reported  cases  of  multiple  neuritis  with  sensory  symptoms  and  with 
paralysis  without  ataxia,  and  various  writers,  in  confirming  these  obser- 
vations, have  described  cases  in  which  tlie  cranial  nerves  of  one  or 
both  sides,  the  ocular  nerves,  the  facial  nerves  of  one  or  both  sides, 
and  the  palate  have  been  involved.  In  a  few  cases  oedema  in  the 
limbs  has  been  noticed  as  a  prominent  symptom.  These  cases  have 
developed  within  one  or  two  weeks  of  the  attack  of  influenza,  have 
reached  their  height  about  one  month  after  the  attack,  and  have  re- 
mained for  many  months.  A  few  cases  with  rapid  recovery  within 
two  months  have  been  seen.  There  are  no  particular  features  to  dis- 
tinguish these  cases  from  the  ordinary  types  of  neuritis,  and  there  is 
no  special  treatment  for  them. 

iRast.     Deut.  Arch.  f.  klin.  Med.,  1886,  Bd.  xl.,  S.  41. 

2  New  York  Medical  Kecord,  1890,  p.  239. 

3  Berliner  klin.  Woclien.,  1890,  p.  181. 
nbid.,  p.  643. 

^Boston  Medical  and  Surgical  Journal,  1892,  pp.  349,  405. 
*  Transactions  of  tlie  Philadelphia  Medical  Society,  1892. 
''Neurolog.  Centralbl.,  1893,  p.  380. 
^  Journal  of  the  American  Medical  Association,  July  24,  1897. 


240  INFECTIOUS   AND    TOXMMIC  FORMS   OF   NEURITIS. 

MULTIPLE   NEURITIS    SUBSEQUENT   TO   VARIOUS   INFECTIONS. 

It  is  still  somewhat  a  matter  of  conjecture  whether  cases  of  paralysis 
following  typhoid,  typhus,  and  malarial  fevers  are  due  to  an  affection 
of  the  peripheral  nerves  or  of  the  spinal  cord.  The  researches  of 
Pitres  and  Vaillard  ^  have  shown  that  extensive  degeneration  of 
peripheral  nerves  is  to  be  found  in  the  bodies  of  patients  who  have 
died  of  typhoid  fever,  and  they  have  also  demonstrated  that  these 
fevers  are  often  followed  by  local  neuritis  as  a  sequel.  Cases  of  mul- 
tiple neuritis  with  autopsies  have  not  as  yet  been  reported  after  typhoid 
or  typhus.  It  is  so  common  in  severe  cases  of  these  diseases  to  use 
alcoholic  stimulants  as  a  remedy  that  it  is  not  impossible  that  in  some 
of  these  cases  the  real  cause  of  the  disease  was  alcoholic  poisoning.  In 
several  hospital  cases  which  I  have  seen  this  has  been  undoubtedly  the 
cause  of  the  supposed  post-typhoid  neuritis,  the  symptoms  being  iden- 
tical with  those  occurring  in  alcoholic  cases  and  including  cerebral 
symptoms,  delusions,  and  loss  of  memory.  Hence  great  care  should 
be  taken  in  reaching  a  conclusion  that  any  post-typhoid  case  is  really 
due  to  the  infection,  although  all  writers  on  typhoid  emphasize  the  pos- 
sibility of  multiple  neuritis  as  a  sequel. 

Buzzard  recorded  ^  two  cases  of  paralysis  following  malarial  fever, 
in  which  all  the  symptoms  pointed  to  an  affection  of  the  peripheral 
nerves.  In  this  country  Gibney  has  described  several  cases  of  paral- 
ysis of  the  extremities,  of  sudden  onset,  rapid  course,  and  prompt 
recovery  under  large  doses  of  quinine,  which  he  considered  malarial. 
The  following  case,  seen  with  Holt,  was  of  this  nature  : 

A  healthy  boy,  aged  ten  years,  was  suddenly  seized  with  a  chill  and 
fever  and  with  pains  of  a  severe  kind  in  both  legs,  associated  with 
weakness  so  that  he  could  hardly  stand.  The  pain  was  referred  to 
the  sciatic  nerves  and  was  accompanied  by  numbness  and  tingling  of 
the  feet  and  legs.  There  was  marked  tenderness  both  in  the  course 
of  the  sciatics  and  in  the  muscles  of  the  thigh  and  leg.  There  was  loss 
of  tendon  reflexes,  preservation  of  skin  reflexes,  and  marked  paresis  in 
all  the  muscles  of  the  legs,  so  that  he  required  help  in  walking. 
There  was  no  ataxia  and  no  incontinence  of  urine.  The  symptoms 
lasted  about  twenty-four  hours  and  then  decreased  in  severity  during 
the  following  day,  but  recurred  on  the  third,  and,  after  a  remission,  on 
the  fifth  day,  with  lessened  severity.  The  periodicity  of  the  affection 
and  the  rapid  cure  under  quinine  left  no  doubt  as  to  its  malarial 
nature.  During  the  third  day,  at  the  time  when  I  saw  him,  there  was 
redness  along  the  course  of  the  sciatics  and  in  the  region  of  the  right 
median  nerves  and  extreme  tenderness  along  these  nerve  trunks,  in 
addition  to  the  symptoms  mentioned.  The  electric  condition  was,  un- 
fortunately, not  tested ;  but  in  WestphaPs  case  faradic  contractility 
was  totally  abolished  during  the  attack.^  In  this  case  the  active  mani- 
festation of  the  malarial  poisoning  was  by  general  neuritis.     In  Buz- 

^Rev.  de  Med.,  1885,  p.  980,  Des  Ndvrites  peripheriques. 
^Paralysis  from  Peripheral  Neuritis,  p.  104,  Loudon,  1886. 
^Neurolog.  Ceutralbl.,  1885,  p.  187. 


NEURITIS    SUBSEQUENT    TO    VARIOUS    INFECTIONS.  241 

zard's  cases  the  malarial  attacks  preceded  the  nervous  affection.  The 
tenderness  iij*.muscles  and  nerves  left  no  doubt  regarding  the  peripheral 
nature  of  the  disease. 

I  have  recently  seen  a  very  severe  case  of  general  neuritis  with  total 
paralysis  in  both  arms  and  legs  lasting  many  months,  and  paralysis  of 
the  diaphragm  which  lasted  for  two  weeks  and  was  attended  by  rapid 
pulse  and  great  exhaustion.  In  this  case  the  only  cause  ascertainable 
was  an  attack  of  severe  dengue  fever  which  immediately  preceded  the 
paralysis.  The  patient  was  an  engineer  and  contracted  the  disease  in 
South  America  and  was  brought  to  New  York  with  great  difficulty. 
He  had  cerebral  symptoms  for  some  weeks  at  the  height  of  the  disease. 

Neuritis  following  variola  is  a  rare  complication,  and  the  following 
case  is  the  only  one  on  record  in  which  an  autopsy  showed  the  lesion 
to  be  a  multiple  neuritis : 

A  young  man  had  varioloid  in  November,  1881,  and  while  con- 
valescing, six  weeks  later,  began  to  suffer  from  severe  pains  in  his  four 
extremities,  especially  in  the  joints  of  his  arms,  which  were  diagnosti- 
cated as  rheumatic,  although  there  was  no  fever.  Soon  after  there 
followed  a  true  paresis,  with  progressive  atrophy  of  the  muscles  of  the 
forearms  and  legs.  The  muscles  at  the  same  time  became  very  tender 
to  touch  or  pressure.  The  tendon  reflexes  were  much  diminished. 
Reaction  of  degeneration  developed  in  all  the  paretic  muscles.  The 
pains  in  the  joints  and  limbs  continued,  but  were  less  severe  than  at 
the  outset.  The  sensibility  of  the  skin  was  about  normal.  The  nerve 
trunks  were  tender  to  pressure.  Profuse,  offensive  perspiration  in  all 
four  extremities  was  a  distressing  symptom.  There  was  no  tendency 
to  bed-sores,  but  an  extensive  pemphigus  developed  in  the  legs,  and 
then  the  pains  became  more  severe.     The  patient  died  of  pneumonia. 

Autopsy  showed  the  brain  oedematous  and  the  cord  in  a  state  of 
hypostatic  congestion.  The  pathological  changes  of  importance  were 
found  in  the  nerves  and  muscles.  The  majority  of  the  nerves  of 
all  the  extremities  were  found  in  a  state  of  degeneration  and  atrophy. 
There  was  marked  degenerative  atrophy  and  fatty  degeneration  of  the 
muscles.^ 

Scarlet  fever,^  measles,^  whooping-cough,^  and  mumps  have  been, 
known  to  be  followed  by  neuritis,  both  local  and  multiple,  but  the 
cases  are  so  rare  as  to  require  only  a  mention. 

Multiple  neuritis  following  erysipelas  or  of  septicsemic  origin  has  been 
reported  by  French''  and  German^  authorities.  I  have  seen  a  severe 
case  in  which  the  only  cause  found  was  a  long-continued  suppurating 
tooth.  This  patient  had  a  continued  fever  during  the  early  part  of  the 
disease.     Bury^  reported  a  case  in  which  an  infected  wound  of  the 

ip,  Grocco,  Milano.     Centralbl.  f.  med.  Wissen.,  1885,  p.  693. 
*M.  J.  Bassette.     Journal  of  Nervous  and  Mental  Diseases,  1892,  p.  461. 
^Allyn.     Medical  News,  1891,  p.  617. 
*Leroux.     Allg.  Wiener  med.  Zeitsch.,  1898,  Nr.  29. 
*  Charcot.     Revue  neurologique,  1893,  Nr.  1  et  2. 

^Gerhardt.  Deutsche  med.  Wochen.,  1898,  p.  14.  Kraus,  Wiener  klin.  Wochen., 
1897,  Nr.  40. 

'  Eoss  and  Burj.     Peripheral  Neuritis,  p.  291,  1893, 
X6 


242  INFECTIOUS   A^'D    TOXEMIC  FORMS   OF   NEURITIS. 

finger  gave  rise  to  secondary  abscesses,  and  then  multiple  neuritis  de- 
veloped. 

Both  local  and  multiple  neuritis  have  been  known  to  develop  subse- 
quent to  attacks  of  gonorrhoea.     They  have  no  special  characteristics. 

German  ^  authors  have  described  many  cases  of  so-called  puerperal 
multiple  neuritis ;  cases  that  appeared  to  be  of  the  infectious  type  and 
due  to  septicsemia  following  abortion,  miscarriage,  or  delivery.  These 
cases  are  not  to  be  confounded  with  the  local  neuritis  of  the  sacral 
plexus  consequent  upon  compression  of  this  plexus  during  labor,  nor 
are  they  to  be  confounded  with  cases  of  myelitis  consequent  upon 
anaemia  of  the  spinal  cord  subsequent  to  confinement.  The  cases  re- 
corded by  the  Germans  have  been  of  general  widespread  multiple  neu- 
ritis of  the  septic  type.  Such  cases  have  not  been  frequently  observed 
in  this  country,  though  I  have  knowledge  of  four  such  cases,  and  in  the 
large  material  at  the  Sloane  Maternity  Hospital  there  is  no  record  of 
multiple  neuritis  occurring  in  the  course  of  pregnancy  or  after  labor. 
Cases  of  neuritis  developing  during  pregnancy  and  traced  to  the  exhaus- 
tion following  long-continued  vomiting  are  not  unknown,^  but  are  not 
of  the  septic  type. 

LEPROUS  NEURITIS. 

Multiple  neuritis  occurring  in  leprosy  is  a  very  rare  affection  in  this 
country,  but  is  of  some  interest  on  account  of  the  fact  that  it  is  the 
only  form  of  neuritis  in  which  the  bacillus  of  any  disease  has  been 
found  in  nerves.  This  bacillus  locates  itself  entirely  in  the  interstitial 
tissue  of  the  nerve  trunks  within  the  sheath,  in  the  perineurium,  and 
by  its  direct  irritation  produces  hyperplasia  of  connective  tissue.  This 
causes  a  compression  of  the  nerve  fibres  and  a  secondary  degeneration 
in  them.  Here  and  there  along  the  course  of  an  affected  nerve  are 
found  small  bulbous  enlargements  where  the  connective  tissue  is  par- 
ticularly thick.  The  changes  in  the  nerves  themselves  are  those  of 
simple  degeneration.  The  lesion  is  due  to  the  direct  effect  of  the 
bacilli  and  is  not  secondary  to  any  infection  or  any  poisoning  conveyed 
to  the  nerves  through  the  blood.  For  this  reason  it  is  a  localized  neu- 
ritis, but  is  multiple  in  its  character,  inasmuch  as  very  many  of  the 
terminal  branches  of  the  filaments  of  the  nerves  are  involved.  (See 
Plate  XVII.) 

Symptoms.  —  The  symptoms  of  leprous  neuritis  are  the  gradual 
development  of  irregular  areas  of  anaesthesia,  the  occurrence  of  par- 
alysis and  atrophy  in  the  peripheral  parts  of  the  limbs,  and  the  pro- 
duction of  trophic  disorders.  The  anaesthesia  is  extremely  irregular 
in  its  distribution,  depending  wholly  upon  the  branches  of  the  nerves 
which  are  affected  by  the  disease,  irregular  plaques  of  anaesthesia 
appearing  here  and  there  upon  the  hands  and  feet  or  upon  the  forearms 
and  legs,  and  on  the  trunk,  and  not  infrequently  upon  the  face.     These 

'  Moebius.  Munchener  med.  Wochen.,  1890,  Nr.  14.  Mader,  Wiener  klin.  Wochen., 
1895,  p.  537.     Jolly,  Arch.  f.  Psych.,  1897,  p.  650. 

^Stiefel.  New  York  Polyclinic,  1893,  March.  Stembo,  Deutsche  med.  Wochen., 
1895,  p.  461.  ^  ^  ^  » 


PLATE  XVII 


'.^Z  -^ 


m    :4Mi 


Cross-section  of  Sciatic  Nerve  in  a  Case  of  Leprous  Neuritis. 
( Marchi  stain.) 

The  degenerated  nerve  fibres  in  the  various  bundles  of  nerves  are  stained  black.  The 
Infiltration  of  the  perineurium  and  epineurium  with  granulation  cells,  not  shown  by  this 
stain,  has  forced  the  bundles  apart. 

(Flatau,  Spec.  Pathol,  u.  Therap.,  Nothnagel,  Bd.  xi.,  Taf.  viii.) 


LEPROUS    NEURITIS. 


243 


anaesthetic  areas  do  not,  as  a  rule,  correspond  to  the  distribution  of 
any  one  nerjte,  nor  are  they  symmetrical,  as  in  ordinary  cases  of  mul- 
tiple neuritis  ;  but  are  extremely  irregular.  They  are  attended  by 
numbness  and  tingling  and  burning  sensations  or  a  pain  of  slight  de- 
gree. The  loss  of  sensibility  may  affect  touch,  temperature,  and  pain- 
ful sensations  equally,  or  any  one  of  these  may  be  affected  alone.     A 

Fig.  105. 


Atrophy  and  trophic  changes  in  hand  and  fingers  in  a  case  of  leprous  neuritis. 

loss  of  pain  and  temperature  senses,  while  touch  is  preserved,  is  not 
uncommon,  hence  the  disease  has  been  mistaken  for  syringomyelia. 
The  paralysis  is  usually  found  in  the  very  small  muscles  of  the  hands 
and  feet,  but  may  extend  to  the  longer  muscles  of  the  extremities,  and 
not  uncommonly  a  few  of  the  facial  muscles  are  paralyzed.  A  pro- 
gressive atrophy  occurs  in  the  paralyzed  muscles,  with  reaction  of  de- 
generation. There  is  a  loss  of  mechanical  excitability  in  the  muscles 
affected.  The  trophic  disturbances  present  in  the  disease,  viz.,  whitish 
colored  plaques  on  the  skin,  peculiar  thickening  or  atrophy  of  the  skin, 
the  glossy  surface,  the  ulcerations,  and  the  gradual  loss  of  substance  — 
are  to  be  regarded  rather  as  a  part  of  the  disease  leprosy  than  as  evi- 
dence of  the  local  affection  of  the  nerves. 

Fig.105  illustrates  the  condition  occasionally  resulting  from  an  ex- 
treme condition  of  anaesthetic  leprosy,  the  photograph  being  taken  from 
a  patient  seen  by  me  in  consultation  with  Piffard  —  one  of  the  few 
cases  of  the  kind  seen  in  New  York  within  the  past  twenty  years.  The 
ends  of  the  thumb  and  forefinger  had  been  eroded. 

The  disease  is  a  slowly  progressive  one  aud  treatment  appears  to  be 
of  no  avail. 

The  differential  diagnosis  between  lepra  ansesthetica  and  syringo- 
myelia is  sometimes  somewhat  difficult,  as  the  trophic  disturbances, 
anaesthetic  areas,  and  nauscular  palsies  of  irregular  distribution  occur 


244  INFECTIOUS   AND    TOXEMIC   FORMS   OF   NEURITIS. 

in  both  diseases ;  but  the  distribution  of  the  ansesthesia  is  usually 
greater  in  syringomyelia  and  begins  in  (if  it  is  not  entirely  limited  to) 
the  upper  extremities.  White  plaques  are  not  common  upon  the  skin 
in  syringomyelia.  Bulbous  swellings  upon  a  nerve  trunk  are  not  felt 
in  syringomyelia,  while  in  lepra  anaesthetica  there  is  an  absence  of 
those  symptoms  of  spastic  paraplegia,  disturbance  of  the  bladder  and 
rectum,  and  increased  reflex  activity  of  the  legs,  which  are  commonly 
found  in  syringomyelia. 

References  may  be  made  to  the  articles  of  Schultze,^  Dehio,^  Zam- 
bacho,^  Laehr,*  and  Blaschko.^ 

^ Deutsche  Arch,  f,  klin.  Med.,  1888,  Bd.  43. 
2  St.  Petersburg  Wochen.,1889,  No.  42,  and  1890_,  No.  48. 

^  Verharidhingen  des  Internationale  wissenschaftlichen  Lepra  Conferenzes  zu  Berlin, 
October,  1897. 

*Arch.  f.  Psych.,  Bd.  xxx.,  323. 

^  Die  Nervenkrankheitserscheinungen  der  Lepra,  Berlin,  1899. 


CHAPTER  XI. 

EPIDEMIC   MULTIPLE   NEURITIS. 
BERIBERI   OR   KAKKE. 

In  1882  Professor  Scheiibe,  of  Tokio,  Japan,  called  the  atteutiou  of 
European  physicians  to  the  existence  of  a  peculiar  affection  prevailing 
among  the  Japanese.^  It  was  called  kakke,  from  the  two  Chinese 
words — kiaku,  meaning  legs,  and  ke,  meaning  disease.  It  had  been 
known  among  the  Chinese  for  centuries,  being  mentioned  by  name  in 
Chinese  medical  books  written  200  B.  C,  and  fully  discussed  by  an 
eminent  author  in  640  A.  D.  It  ceased,  however,  to  prevail  in  China 
about  two  hundred  years  ago,  and  of  late  it  is  found  chiefly  in  Japan. 
There  its  importance  is  considerable,  since  it  is  so  prevalent  that  in 
1877,  14  per  cent,  and  in  1878,  38  per  cent,  of  the  meu  serving 
in  the  army  suffered  from  it.  It  is  considered  a  miasmatic  infectious 
disease  by  Scheube,  although  Takaki  considered  it  due  in  some  way  to 
the  diet  of  rice  and  fish.  Braddon  has  pointed  out  that  every  beri- 
beric  in  the  Orient  is  a  rice-eater ;  that  persons  on  rice-free  diet  do 
not  get  beri-beri ;  that  the  importation  of  machine-milled  rice  into  any 
part  of  the  world  is  followed  by  beri-beri ;  that  the  users  of  home- 
milled  rice  do  not  get  beri-beri ;  that  if  from  any  cause  the  home 
supply  is  exhausted,  beri-beri  will  break  out  on  the  importation  of 
machine-milled  rice.  He,  therefore,  concludes  that  beri-beri  is  due  to 
a  saprophyte  in  the  rice  pericarp  which  invades  the  dead  decorticated 
rice-grain  or  aleurone. 

In  the  primitive  preparation  the  rice  is  husked  in  a  mortar  and  then 
boiled,  causing  the  pericarp  to  adhere  to  the  aleurone,  but  giving  the 
rice  a  dull  appearance.  The  adhesion  of  the  pericarp  prevents  the 
invasion  of  the  aleurone  by  the  toxic  agent,  and  rice  so  prepared  does 
not  cause  beri-beri.  But,  on  the  other  hand,  a  final  step  in  the  machine 
milling  of  j-ice  is  its  being  passed  through  leather  polishers,  thus  re- 
moving the  pericarp,  whose  broken  parts  mix  with  the  kernels  and 
allow  the  toxic  agent  free  access  to  the  rice  grain,  a  step  which  pro- 
duces a  finer-looking  grain,  but  a  more  dangerous  article. 

It  has  been  found  that  a  toxin  called  "  arsin  "  exists  in  the  pericarp 
of  rice,  possibly  of  fungoid  origin,  and  this  can  cause  beri-beri.  If 
the  rice  is  properly  prepared  or  "  cured  "  this  is  removed,  but  "  paddy 
rice  "  not  so  cured  contains  it  and  is  the  cause  of  the  disease  among 
the  Chinese.  Cooking  does  not  "cure"  the  rice.  Since  wheat  has 
been  substituted  for  rice  in  the  diet  of  some  of  the  barracks  and  prisons 

*  Deutsche  Arch.  f.  klin.  Med. ,  vols,  xxvii.  and  xxxi. 
245 


246  RPlDEMtC    MULTIPLE    NEVRlTlS. 

in  Japan  the  disease  has  been  less  common.  It  occurs  in  epidemics, 
but  is  always  endemic  in  Japan.  It  rarely  attacks  Europeans.  While 
not  contagious,  the  disease  is  transmitted  by  human  beings,  for  out- 
breaks have  occurred  on  islands  formerly  free  after  the  landing  of  per- 
sons who  were  affected.  It  aifects  females  rarely,  only  9  per  cent  of 
the  cases  being  in  women  ;  and  it  is  the  youth  of  the  land,  between  the 
ages  of  sixteen  and  twenty-five  years,  who  suifer.  Exposure  to  damp 
and  cold  in  crowded  dwellings,  such  as  barracks,  increases  the  liability 
to  the  affection.  The  majority  of  the  cases  occur  during  the  hot,  damp 
months,  but  some  are  always  under  observation. 

This  disease  is  not,  however,  confined  to  Japan.  It  has  been 
observed  for  many  years  in  the  islands  of  the  Pacific  Ocean,  espe- 
cially in  the  Philippines,  in  India,  Ceylon,  on  the  west  coast  of  the 
Red  Sea,  in  Borneo  and  New  Guinea,  in  Brazil  and  Cuba,  on 
the  isthmus  of  Panama,  and  in  the  Dutch  possessions  in  the  China 
Sea.^  It  is  there  known  under  the  name  of  beriberi.  It  is  endemic 
in  these  regions,  but  occasionally  occurs  as  an  epidemic.  An  interest- 
ing account  of  such  an  epidemic,  occurring  in  1882-83  in  Manila,  in 
the  Philippine  Islands,  has  been  given  by  Koeniger.^  It  appears  that 
in  the  fall  of  1882  an  epidemic  of  cholera  occurred  in  Manila  of  such 
severity  that  20,000  persons,  in  a  population  of  400,000  were  affected. 
As  a  precaution  against  this  disease  the  native  population  lived  for 
several  months  exclusively  upon  rice,  refusing  to  eat  fruit  or  fish, 
which  are  their  other  chief  articles  of  diet.  As  the  epidemic  was  sub- 
siding a  terrible  cyclone  devastated  the  city,  destroying  the  light 
wooden  houses,  and  leaving  60,000  families  homeless ;  and  these  poor 
people  were  exposed  for  several  weeks  to  the  inclemency  of  the 
weather,  which  at  this  time  of  the  year  was  rainy.  A  few  days  after 
the  cyclone  the  epidemic  of  beriberi  began,  and,  as  the  disease  had 
never  before  appeared  in  Manila,  the  unknown  affection  excited  great 
alarm.  This  was  increased  by  its  fearful  mortality,  60  per  cent,  of 
the  early  cases  proving  fatal.  Europeans  were  exempt,  with  two  ex- 
ceptions, and  the  Chinese  population  did  not  suffer  greatly,  but  among 
the  natives  the  epidemic  was  widespread.  Thus,  in  one  suburb  of 
Manila,  of  25,000  inhabitants,  300  died  in  the  course  of  eight  weeks. 
Men  and  women  were  equally  affected  and  persons  of  all  ages,  except 
young  children,  were  attacked.  The  disease  terminated  fatally  in  from 
ten  days  to  five  weeks  after  its  onset ;  but  as  time  went  on  the  propor- 
tion of  recoveries  increased,  and  by  the  end  of  March,  1883,  it  had 
almost  disappeared.  The  months  from  October  to  March  are  the  dry, 
cool  season  in  the  Philippine  Islands,  although  the  climate  is  tropical. 
Exposure  to  heat  could  hardly  be  considered  a  cause  of  this  epidemic, 
but  the  exposure  to  cold  and  damp  after  an  exclusive  diet  of  rice  was 
the  probable  cause. 

^  B.  Scheube.  Diseases  of  Warm  Countries,  p.  190,  London,  1903,  where  a  complete 
literature  is  to  be  found. 

^Deutsche  Arch.  f.  klin.  Med.,  xsxiv. 


BERIBERI   OR   KAKKE.  247 

Sporadic  cases  of  beriberi,  or  kakke,  occasionally  appear  in  our 
hospitals,  usually  in  the  persons  of  Chinese  or  Malay  sailors,  or  in  the 
persons  of  travellers  from  tropical  climates  who  have  been  exposed  to 
the  infection  in  the  place  from  which  they  came.^  In  1881  a  Brazilian 
naval  vessel  entered  San  Francisco  with  a  large  number  of  the  crew 
affected  by  the  disease.  They  were  sent  to  the  United  States  Marine 
Hospital  and  attended  by  Dr.  Hebersmith,  who  gave  ap  interesting 
account  of  the  circumstances  leading  to  the  development  of  the  disease 
in  the  United  States  Marine  Hospital  Report  for  1881. 

In  1886  a  commission  was  appointed  by  the  Dutch  Government  to 
investigate  the  subject  of  its  nature,  and  the  published  report  contains 
the  following  statements.^  The  disease  is  caused  by  a  micro5rganism 
resembling  the  bacillus  of  splenic  fever,  though  somewhat  smaller, 
which  colors  with  fuchsin  and  gentian-violet,  and  can  be  seen  with 
a  power  of  560  diameters.  These  bacilli  are  found  in  the  blood,  lungs, 
heart,  brain,  cord,  and  nerves  of  the  patients,  and  can  be  cultivated 
outside  of  the  body.  They  are  seen  in  pairs  and  can  be  separated 
from  the  cerebro-spinal  fluid  in  the  acute  stage  of  the  disease.  The 
germs  infect,  wooden  dwellings  chiefly.  They  may  be  conveyed  by 
articles  of  clothing,  and  probably  enter  the  body  by  the  lungs.  Direct 
contagion  has  not  been  observed.  A  potent  predisposing  cause  to  their 
reception  in  the  body  and  to  the  development  of  the  disease  is  lack  of 
nutrition,  consequent  upon  exposure  to  damp  and  cold,  and  upon  in- 
sufficient or  bad  food.  This  view  is  supported  by  the  recent  studies 
of  Hamilton  Wright  ^  who  has  proven  that  the  organism  gains  entrance 
to  the  body  through  the  alimentary  canal  and  multiplies  in  the  duo- 
denum. Wheate  *  affirms  that  the  infection  is  always  through  a  skin 
abrasion.  It  is  probably  a  toxin  from  this  microorganism  which 
produces  the  neuritis,  as  in  cases  of  diphtheria. 

Symptoms.  —  The  cases  of  beriberi  are  divided  into  two  general 
classes,  according  to  their  severity  : 

There  are,  first,  slight  cases,  in  which  the  onset  is  gradual,  being 
usually  preceded  by  a  little  fever,  coryza,  and  conjunctivitis,  which 
cease  when  the  actual  symptoms  commence.  The  patient  first  notices 
a  loss  of  appetite,  a  dull  oppressive  feeling  in  the  epigastrium,  nausea 
and  vomiting,  a  weak  and  heavy  feeling  in  the  legs,  and  finds  that  he 
tires  so  easily  that  he  cannot  walk  as  much  as  usual.  The  fatigue  is 
soon  associated  with  numbness  and  pain  in  the  legs,  and  with  a  slight 
cedematous  swelling.  Then,  if  not  before,  palpitation  of  the  heart, 
and  general  malaise  are  felt,  and  the  patient  finds  it  necessary  to  apply 
for  treatment.  An  examination  then  shows  some  diminution  of  power 
in  the  feet  and  legs  and  also  in  the  hands,  with  loss  of  tendon  reflexes 

'Seguin.  Medical  News,  Dec.  11,  1886.  Bondurant,  Jour.  Nerv.  and  Ment.  Dis., 
Dec,  1900. 

2  Deutsche  med.  Wochen.,  December  9,  1886.  See  also  Harada,  Neurolog.  Cen- 
tralbl,  1885,  p.  826. 

^  On  the  Classification  and  Pathology  of  Beriberi.  John  Bale  Sons,  London,  De- 
cember, 190.3. 

*N.  Y.  Med.  .Jour.,  April  6,  1907. 


248  EPIDEMIC    MULTIPLE    NEURITIS. 

and  much  tenderness  in  the  muscles,  which  show  a  diminished  electric 
excitability.  There  is  never  any  ataxia,  though  the  patient  sways  when 
his  eyes  are  closed.  There  is  discovered  a  slight  degree  of  anaesthesia 
of  irregular  distribution,  chiefly  in  the  legs  and  in  the  radial  nerve 
region  on  the  forearms.  Though  the  patients  look  pale,  examination 
of  the  blood  does  not  show  any  marked  anaemia. 

The  circulation  in  the  extremities  is  sluggish.  The  heart  is  irregular 
and  rather  rapid,  and  the  oedema  of  the  extremities  indicates  a  failure 
of  its  power.  Wallace  Taylor^  found  that  a  sphygraographic  tracing 
is  characterized  by  a  sudden  upstroke  in  ventricular  systole,  by  a  pre- 
cipitous descent  from  the  apex  of  the  percussion  wave,  and  by  dicrotism. 
Beyond  this  point  these  cases,  which  make  up  the  majority,  do  not 
advance.  They  usually  recover  in  a  few  days,  or  at  most  a  month, 
although  a  few  become  chronic  and  require  several  months  before  the 
cure  is  complete. 

There  are,  secondly,  severe  cases.  These  may  present  three  differ- 
ent types,  though  Wheate  believes  that  these  types  are  merely  stages  of 
the  disease.  There  is,  first,  the  atrophic  or  dry  type,  in  which,  after  an 
onset  similar  in  nature  to  that  in  the  slight  cases,  but  much  more 
rapid,  the  weakness  develops  into  a  true  paralysis  associated  with 
marked  wasting  of  the  muscles  and  reaction  of  degeneration,  with 
great  diminution  of  galvanic  excitability.  Within  a  week  the  patient 
has  to  go  to  bed,  and  then  the  paralysis  soon  spreads  from  the  legs  to 
the  arms,  and  may  involve  the  trunk  and  even  the  face.  The  entire 
muscular  system  wastes  away  until  the  patient  is  a  mere  skeleton.  In 
addition  to  the  motor  synrptoms  there  is  great  sensory  disturbance. 
The  suffering  from  pain,  paveesthesise,  and  general  muscular  tenderness 
is  extreme,  and  the  patient  lies  totally  helpless  and  unable  to  tolerate 
the  lightest  touch.  The  skin  may  be  glossy.  There  is  usually  some 
anaesthesia,  but  it  is  never  complete,  and  it  does  not  involve  the  trunk. 
The  temperature  sense  is  seldom  affected.  Pain  may  be  delayed  in 
transmission.  There  are  no  gastric  symptoms  and  no  oedema.  Some 
cases  prove  fatal  from  general  exhaustion  or  intercurrent  disease,  but 
the  majority  recover  after  a  convalescence  which  lasts  a  year  or  more, 
during  which  the  muscular  system  is  rebuilt. 

There  is,  secondly,  the  hydropic  or  wet  type.  In  these  heart  failure 
appears  early  and  is  associated  with  a  marked  decrease  of  arterial  ten- 
sion and  much  oedema  of  the  entire  body,  the  effusion  into  the  cavities 
being  added  to  that  beneath  the  integument.  The  swelling  of  the 
oedematous  parts  conceals  the  atrophy  which  is  going  on  in  the  mus- 
cles, but  this  is  indicated  by  the  paralysis,  which  is  as  severe  as  in  tlie 
preceding  form,  and  it  becomes  evident  during  recovery  when  the 
oedema  has  subsided. 

There  is,  thirdly,  the  acute  pernicious  type.  In  this  all  the  symp- 
toms of  the  two  former  types  appear  in  rapid  succession,  and,  in  addi- 
tion, gastro-intestinal  symptoms  and  a  suppression  of  urine  combine  to 
make  the  condition  an  alarming  one.     Effusions  into  the  pleura  and 

^Studies  in  Japanese  Kakke,  by  Wallace  Taylor,  M.D.     Osaka,  1886. 


BERIBERI    OR    KAKKE.  249 

pericardium  appear  early.  The  pulse  becomes  small  and  irregular, 
and  cyano^  indicates  the  heart  failure  which  precedes  death.  There 
are  no  constant  changes  in  the  blood,  and  leucocytosis  has  not  been 
found. 

In  this  form  the  disease  may  run  its  course  in  two  weeks  to  a  fatal 
termination.  This  was  the  form  which  chiefly  prevailed  in  Manila, 
the  cases  of  the  atrophic  form  being  the  ones  which  recovered. 

The  severity  differs  much  in  different  epidemics,  the  mortality  vary- 
ing from  2  per  cent,  in  Japan  to  60  per  cent,  in  Manila.  It  is  usually 
not  above  3  per  cent.  In  all  the  forms  there  is  some  danger  of  a 
sudden  heart  failure,  and  this  is  usually  the  cause  of  death. 

Treatment.  —  As  to  its  treatment,  it  may  be  mentioned  that  qui- 
nine fails  to  influence  its  course  and  that  heart  stimulants  to  combat 
the  dangerous  complications,  hypnotics  to  counteract  the  pain  and 
insomnia,  and  general  tonic  treatment  have  proved  of  the  greatest  ser- 
vice. Change  of  climate  is  often  attended  by  recovery.  In  the  stage 
of  recovery  electricity  and  massage  have  been  employed  with  advantage. 


CHAPTER   XII. 

MULTIPLE  NEURITIS   ASSOCIATED   WITH   OTHER  DISEASED 
STATES   OF   THE   BODY. 

Multiple  Neuritis  and  Rheumatism,  Gout,  Diabetes,  Tuberculosis,  Syphilis,  and  Carci- 
noma.    Multiple  Neuritis  of  Unknown  Origin.     Senile  Polyneuritis. 

The  occurrence  of  multiple  neuritis  in  connection  with  certain  other 
diseases  has  been  observed  in  so  many  cases  as  to  have  led  to  the 
hypothesis  that  there  must  be  some  causal  relation  between  the  two 
conditions.  The  diseases  in  which  neuritis  develops  are  of  a  nature  to 
affect  the  general  health,  to  impair  the  nutrition,  and  to  produce  a 
constitutional  disturbance ;  but  it  becomes  evident,  from  the  fact  that 
only  a  small  percentage  of  the  patients  suffering  from  these  diseases 
develop  neuritis  that  some  other  cause  must  be  active  in  conjunction 
with  the  disease.  A  congenital  or  acquired  weakness  of  the  nervous 
system  is  probably  present  in  certain  individuals  and  predisposes  them 
to  this  complication,  when  they  fall  victims  to  other  affections.  The 
diseases  with  which  neuritis  is  frequently  associated  are  rheumatism 
and  gout,  diabetes,  tuberculosis,  syphilis,  carcinoma,  senility,  and 
arterial  sclerosis. 

MULTIPLE  NEURITIS  AND  RHEUMATISM. 

Multiple  neuritis  is  very  rarely  to  be  traced  to  rheumatism.  The 
so-called  "  rheumatic  "  cases  of  the  Germans  are  cases  due  to  exposure 
to  cold  and  do  not  present  the  characteristic  symptoms  of  rheumatic 
fever  and  joint  affections.  In  many  cases  of  neuritis  some  pain  in  the 
joints  on  motion  may  be  present,  but  it  is  very  seldom  that  the  red, 
swollen,  oedematous,  hot  joints  of  rheumatism  are  found.  A  critical 
review  of  the  cases  of  multiple  neuritis  supposed  to  be  rheumatic  ^ 
shows  no  conclusive  evidence  of  the  coexistence  of  the  two  diseases. 
It  is  not  enough  to  consider  pain  and  tenderness  about  the  rheumatic 
joint  as  evidence  of  neuritis  when  anaesthesia  and  paralysis  in  the 
domain  of  these  tender  nerves  fail  to  appear.  Nor  is  it  sufficient  evi- 
dence of  rheumatism  to  find  painful  joints  in  cases  of  neuritis  without 
the  constitutional  effects  of  the  disease.  Hence  it  seems  very  ques- 
tionable whether  we  are  justified  in  considering  rheumatism  as  a  cause 
of  multiple  neuritis.  It  is  admitted  that  local  neuritis  is  not  infre- 
quently produced  in  the  vicinity  of  a  rheumatic  joint,  as  in  the  cases 
fully  described  by  Bury.^    Thus,  in  rheumatism  of  the  elbow,  the  ulnar 

^E.  g.,  the  cases  cited  by  Steiner.     Deut.  Arch.  f.  klin.  Med.,  Iviii.,  p.  240. 
^  Medical  Chronicle,  June,  1888. 

250 


MULTIPLE  NEURITIS  FOLLOWING  GOUT.  251 

nerve  may  become  inflamed,  swollen,  tender  to  pressure,  and  paralysis 
and  atrophy  of  the  interossei  and  thenar  eminences  may  ensue,  with 
anaesthesia  in  the  hand.  So,  too,  peroneal  neuritis  may  result  from  a 
rheumatism  of  the  knee.  I  have  seen  several  cases  of  this  kind.  In 
such  cases  there  is  a  localized  neuritis  due  to  extension  of  the  inflam- 
matory processes  directly  from  the  joint  to  the  nerves  passing  over  it. 
This  cannot  be  considered,  however,  as  a  neuritis  due  to  the  state  of 
the  blood  which  produces  the  rheumatism.  Furthermore,  an  error  is 
often  made  when  the  tender,  stiif  .joints  which  develop  late  in  the 
course  of  neuritis  are  termed  rheumatic.  In  the  later  stages  of  alco- 
holic neuritis,  when  the  tenderness  of  the  skin  and  muscles  and  the 
pain  produced  by  any  motion  have  led  the  patient  to  keep  the  hands 
and  fingers  and  toes  perfectly  fixed  and  immovable  for  weeks,  an 
ankylosis  of  the  smaller  joints  often  develops,  and  this  may  be  accom- 
panied by  some  thickening  of  the  articular  surfaces  and  is  often 
attended  by  thickening  of  the  skin  over  the  knuckles,  and  by  profuse 
sweating  of  the  hands  and  feet.  This  may  give  rise  to  a  condition  not 
unlike  that  resulting  from  chronic  rheumatism.  It  is  not,  however, 
rheumatic,  but  is  the  natural  sequel  of  inactivity  in  a  joint  which  is 
fixed,  and  is  a  trophic  symptom  of  neuritis. 

It  is  evident,  therefore,  that  it  is  a  mistake  to  regard  neuritis  as 
a  frequent  result  of  rheumatism,  or  to  establish  any  causal  relation 
between  the  two  diseases.  In  case  the  two  diseases  coincide  or  follow 
one  another,  each  should  be  treated  separately. 

In  the  course  of  a  case  of  chronic  articular  rheumatism  or  of 
chronic  arthritis  deformans,  multiple  neuritis  may  develop  from  any 
cause.  I  have  seen  several  such  cases ;  but  here,  again,  there  is  never 
any  certain  evidence  of  a  causal  relation  between  the  two  affections. 

MULTIPLE  NEURITIS  FOLLOWING  GOUT. 

Neuralgia  and  localized  neuritis  in  single  nerves  have  been  fre- 
quently observed  as  a  complication  of  gout.  Of  late,  English  writers 
have  called  attention  to  the  frequency  of  attacks  of  multiple  neuritis 
affecting  symmetrical  nerves  on  both  sides  of  the  body  as  an  occa- 
sional sequel  or  accompaniment  of  this  constitutional  affection.  Sciat- 
ica and  brachial  neuritis  are  frequently  traced  to  this  cause.  Buzzard  ^ 
in  particular,  has  described  several  cases  in  which  a  sudden  attack  of 
pain  and  numbness  in  the  hands  has  been  followed  by  weakness  and 
loss  of  faradic  excitability  in  the  muscles  of  the  hands  and  forearms, 
and  in  one  case  this  condition  soon  appeared  in  the  feet.  These 
patients  were  gouty  persons  and  acute  attacks  of  gout  had  preceded  or 
accompanied  the  nervous  symptoms.  Ebstein^  and  Grube  ^  and  Remak  * 
have  published  similar  cases,  and  all  agree  that  a  direct  relationship 

^Buzzard.  Harveian  Lectures,  1885,  Lancet,  vol.  ii.,  p.  983.  Dyce  Duckworth, 
Gout,  London,  1893,  p.  247. 

^Ebstein.  Deutsche  med.  Wochen.,  1898,  p.  489. 
'Grabe.  Miinchcner  med.  Wochen.,  1899,  p.  23. 
^Keraak.     Nothnagel's  spec.  Path.  u.  Then,  vol.  xi.,  Th.  iii.,  p.  623. 


252  MULTIPLE  NEURITIS  AND   OTHER  DISEASES. 

between  gout  and  neuritis  is  proven.  A  general  peripheral  neuritis, 
such  as  the  form  occurring  after  alcoholic  poisoning  or  after  exposure 
to  cold,  has  not  been  observed.  For  this  reason  the  following  case, 
in  which  both  peroneal  and  anterior  tibial  nerves  were  affected  and 
the  smaller  branches  of  the  nerves  in  the  hands  were  also  involved, 
may  be  recorded  as  of  interest : 

The  disease  began  with  a  severe  itching  and  burning  sensation  about 
the  toe-nails  and  upon  the  dorsum  of  the  right  foot,  which  soon  devel- 
oped in  the  left  foot  also.  This  itching  and  burning  sensation  then 
extended  up  both  legs  to  the  knee,  but  was  not  at  first  attended  by  any 
change  in  the  appearance  of  the  skin,  or  by  actual  anaesthesia.  After 
several  remissions  the  symptoms  became  more  intense,  and  the  burn- 
ing and  itching  were  accompanied  by  the  appearance  of  a  crop  of  mi- 
nute vesicles.  The  feet  then  became  swollen,  the  skin  became  glossy, 
red,  and  extremely  tender  to  the  slightest  touch,  and  the  sensation  was 
one  of  great  pain  throughout  both  lower  extremities.  Any  moist 
dressing  upon  the  surface  intensified  the  pain,  but  dry  dressings  or 
oily  applications  seemed  to  give  some  relief.  Large  serous  blebs  formed 
upon  the  soles  of  the  feet  and  about  the  toes,  and  there  was  consider- 
able eczematous  exudation  and  a  scaly  appearance  of  the  skin  of  the 
entire  legs.  After  this  condition  had  lasted  for  three  months  similar 
itching  and  burning  began  in  the  hands  about  the  fingers  with  very 
marked,  deep-seated  parsesthesia.  Fine  vesicular  pin-point  elevations 
in  the  skin  of  the  hands  also  appeared,  but  these  did  not  go  on  to 
the  formation  of  blebs,  as  in  the  feet.  After  two  months  the  skin  of 
the  legs  had  become  thickened,  cracked,  and  shiny  with  crusts  all  over 
the  surface,  and  when  these  had  peeled  off  the  surface  was  red  and 
very  sensitive  to  light  pressure.  The  nails  of  the  toes  were  thickened, 
rough,  striated,  and  black.  It  was  said  that  they  had  not  grown  at 
all  during  six  months.  Tactile  sensibility  was  diminished  over  both 
feet  and  both  legs,  but  any  touch  was  attended  by  great  pain.  The 
muscles  of  the  feet  and  legs  had  become  extremely  atrophied  and  were 
almost  entirely  paralyzed  ;  no  electrical  examination  could  be  made  on 
account  of  the  extreme  sensitiveness.  At  this  time  sciatica  was  com- 
plained of  as  high  as  to  the  buttocks.  The  knee-jerks,  at  first  exag- 
gerated, were  subsequently  lost. 

When  I  saw  the  patient,  a  woman  of  about  fifty  years  of  age,  seven 
months  after  the  onset,  she  was  unable  to  stand  or  bear  her  feet  upon 
the  floor,  but  the  paralysis  had  subsided  and  she  could  move  her  ankles ; 
but  could  not  move  her  toes.  There  was  very  marked  tenderness  to 
touch  over  both  legs  and  upon  both  feet,  and  the  tenderness  of  the 
nerves  upon  the  soles  of  the  feet  was  extreme.  The  skin  of  the  feet 
was  dry,  scaly,  and  cracked.  The  nails  were  badly  discolored,  ridged, 
and  dark  and  rough,  excepting  near  the  matrix,  where  a  new  growth 
of  nail,  about  one-half  inch  in  depth,  had  begun.  Any  covering  ap- 
plied to  the  feet  caused  intense  burning  and  itching,  so  that  she  kept 
the  feet  entirely  uncovered  both  by  day  and  night.  There  was  noth- 
ing in  the  way  of  local  treatment  that  relieved  this  itching  and  burn- 


MULTIPLE  NEURITIS  FOLLOWING  DIABETES.  253 

ing,  though  every  form  of  local  application  had  been  tried.  The  ball 
of  the  foot  was  red,  the  rest  of  the  foot  white ;  but  during  an  attack 
of  pain  the  feet  became  scarlet,  or  sometimes  became  very  much  more 
pallid  than  usual.  It  was  evident  that  the  condition  in  the  feet  was 
that  of  erythromelalgia. 

There  was  a  similar  burning  sensation  and  itching  felt  in  the  hands 
from  time  to  time,  but  there  was  no  apparent  malnutrition  of  the  skin. 

The  patient  came  of  a  very  gouty  family  and  had  many  indications 
of  the  presence  of  gout,  and  all  these  symptoms  gradually  subsided  in 
the  course  of  a  year  under  treatment  directed  exclusively  to  the  gouty 
state,  namely,  dietetic  treatment  and  alkalies  given  freely.  The  coal- 
tar  products  appeared  to  give  some  little  relief  to  the  local  symptoms. 
I  have  seen  the  patient  several  times  in  the  past  five  years  and  there 
has  been  no  return  of  the  aiFection. 

I  have  seen  several  other  cases  that  were  quite  similar  to  this  in 
their  symptoms  and  course  and  have  no  doubt  that  they  can  be  classed 
together  as  gouty  neuritis. 

MULTIPLE  NEURITIS  FOLLOWING  DIABETES. 

Neuralgia  in  the  course  of  diabetes  is  a  very  common  occurrence, 
and  probably  is  an  indication  that  the  condition  of  the  blood  in  this 
disease  produces  an  imperfect  nutrition  of  the  nerves.  The  loss  of 
knee-jerk  often  observed  in  this  disease  (30  per  cent,  of  cases)  supports 
this  hypothesis.^  The  sciatic  nerve  seems  to  be  the  one  more  com- 
monly affected  than  any  other  nerve.^  I  have  seen  many  cases,  and 
think  it  important  to  look  for  sugar  in  the  urine  in  every  case  of  sci- 
atica. Bruns  has  called  attention  to  the  frequency  of  crural  and  obtu- 
rator neuralgia  and  neuritis  in  the  course  of  diabetes,  and  Buzzard  ^ 
has  observed  several  cases  of  brachial  plexus  neuritis  following  dia- 
betes. In  some  cases  the  attacks  of  neuralgia  have  been  bilateral.^ 
In  some  cases  they  have  gone  on  to  neuritis.  The  nervous  symptoms 
occurring  in  the  course  of  diabetes  may  be  not  only  neuralgic  pains  of 
a  sharp,  shooting  character,  but  also  various  forms  of  parsesthesia, 
numbness,  and  burning  sensations.  Patients  often  complain  of  severe 
cramps  in  the  legs,  especially  at  night,  and  these  may  precede  or  may 
accompany  sciatica. 

Multiple  symmetrical  neuritis  of  extensive  distribution,  not  unlike 
that  occurring  after  poisoning  by  alcohol,  both  of  the  paralytic  and  of 
the  ataxic  types,  has  been  ascribed  to  diabetes,  but  is  exceptional.® 
Several  authors  have  described  cases  of  so-called  neurotabes  peripherica 
diabetica,  which  have  resembled  locomotor  ataxia,  but  have  gone  on 
to  recovery.^    In  some  of  these  cases  the  diagnosis  from  true  tabes  was 

'Ziemssen.     Miinchener  med.  Wochen.,  1885,  p.  618. 

^Auerbach.     Deutsches  Arch.  f.  klin.  Med.,  1887,  p.  484. 

» Buzzard.     British  Medical  Journal,  1890,  vol.  i.,  p.  1421. 

^Williamson.     Medical  Chronicle,  1892,  November;  Lancet,  1897,  vol.  ii.,  p.   138, 

^Pryce.     Brain,  1893,  vol.  xvi.,  p.  416  ;  also  Pavy,  Lancet,  July  9,  1904. 

»Leichtentrett,  Inaug.  Diss.,  Berlin,1893  ;  PwT,  Williamson, Rev,  deM^d.,  1911,  Dec, 


254  MULTIPLE  NEURITIS  AND   OTHER  DISEASES. 

difficult,  especially  as  perforating  ulcer  of  the  foot  occurred/  The 
tendency  to  gangrene  in  diabetes  is  not  traceable  to  neuritis.  The  in- 
tensity of  the  neuritis,  according  to  Gowers,  is  not  related  to  the  amount 
of  the  sugar  in  the  urine. 

The  prognosis  is  good  in  all  tliese  forms  of  neuritis,  as  they  recover 
when  the  sugar  disappears  from  the  urine. 

The  treatment  will  necessarily  be  directed  to  the  original  disease  as 
well  as  to  the  complication. 

MULTIPLE  NEURITIS  AND  TUBERCULOSIS. 

Cases  of  multiple  neuritis  occurring  in  tuberculous  patients  require 
some  consideration.  If  a  review  of  cases  of  multiple  neuritis  with  au- 
topsy be  made  it  will  be  found  that  quite  a  number  of  the  patients 
died  of  phthisis.  There  is  little  evidence  to  prove  that  the  neuritis 
was  due  in  these  cases  to  the  tuberculous  disease.  No  one  has  found 
tubercles  in  the  nerve  trunks  in  these  cases,  and  bacteriological  exami- 
nation has  not  demonstrated  the  presence  of  bacilli  in  the  nerves.  For 
this  reason  it  is  not  possible  to  speak  of  a  tuberculous  multiple  neu- 
ritis. There  is  no  doubt,  however,  that  the  existence  of  a  grave  con- 
stitutional affection  may  lead  to  such  a  general  impairment  of  nutri- 
tion in  the  nerves,  as  well  as  in  other  organs,  as  to  predispose  such  a 
patient  to  an  attack  of  neuritis.  Hence  especial  mention  must  be 
made  of  neuritis  as  a  complication  of  phthisis.  It  should  not  be  for- 
gotten, however,  that  in  phthisical  patients  who  have  used  alcohol 
freely  the  neuritis  may  owe  its  origin  to  this  poison.  Oppenheim  ^  has 
reported  cases  of  multiple  neuritis  in  tuberculous  patients  which  went 
on  to  recovery.     The  following  case  died  : 

A  young  girl  who  was  suffering  from  phthisis  took  a  severe  cold  and 
began  to  have  pains  in  the  joints,  weakness,  and  numbness  of  the 
lower  extremities.  The  weakness  of  the  legs  increased  rapidly  and 
was  attended  by  rapid,  diffuse  atrophy.  The  same  symptoms  soon  de- 
veloped in  the  arms.  Four  weeks  after  the  onset  partial  reaction  of 
degeneration  was  present  in  all  the  extremities,  and  a  total  paralysis 
of  the  legs  and  nearly  complete  paralysis  of  the  arms  were  present. 
In  the  course  of  the  disease,  which  lasted  five  months,  there  was  little 
pain,  but  great  tenderness  of  the  muscles  and  hypersesthesia  of  the  skin. 
The  sensation  was  diminished  in  the  feet  and  hands,  but  this  was 
slight  in  comparison  with  the  motor  symptoms.  The  paralysis  finally 
attacked  the  trunk  muscles  and  the  diaphragm,  while  the  beginning  of 
rapid  heart  action  was  thought  to  indicate  paralysis  of  the  pueumo- 
gastric  nerve.  Great  prostration,  delirium,  incontinence,  bed-sores, 
and  oedema  of  extremities,  with  fever,  preceded  death. 

A  high  degree  of  degeneration  of  the  peripheral  nerves,  including 
the  phrenic  and  pneumogastric,  was  found  at  the  autopsy,  with  a  mod- 
erate degree  of  atrophy  of  the  muscles.     Spinal  cord  and  anterior 

^Raymond.     Lefons  sur  les  malad.  du  syst.  nerv.,  1895,  vol.  ii.,  p.  331, 
22eitscb.  f.  klin.  Med.,  1886,  p.  230, 


MULTIPLE  NEURITIS  AND  SYPHILIS.  255 

motor  roots  were  normal.  The  changes  in  the  nerves  were  similar  to 
those  after  ^ection,  viz.,  a  simple  degeneration  of  the  fibres  with  some 
increase  in  the  connective  tissue,  but  there  were  no  evidences  of 
changes  in  the  vessel  walls,  no  infiltration  with  cells.  The  nerves 
contained  very  few  normal  fibres.  The  myelin  sheaths  were  found  in 
all  stages  of  destruction  even  to  an  entire  absence  of  contents  of  the 
sheath  of  Schwann.  Throughout  the  fibres  fatty  and  granular  masses 
were  found.  The  sheath  of  Schwann  did  not,  however,  show  an  in- 
crease of  nuclei.^ 

Such  a  case  as  this  would  have  been  ascribed,  a  few  years  ago,  to  a 
tuberculous  spinal  meningitis.  It  is  not  my  purpose  to  dispute  in  any 
way  the  fact  that  many  cases  of  paralysis  occurring  in  the  course  of 
phthisis  are  due  to  lesions  of  the  central  nervous  system  and  its  mem- 
branes, but  I  desire  to  emphasize  the  fact  that  a  multiple  neuritis  may 
produce  paralysis  in  tuberculous  individuals,  and  the  importance  of 
appreciating  this  possibility  cannot  be  too  strongly  urged,  since  the 
treatment,  as  well  as  the  prognosis,  will  differ  widely,  according  to  the 
diagnosis  made. 

It  has  been  known  for  some  time  that  local  neuritis  may  complicate 
phthisis,  and  the  researches  of  Pitres  and  Vaillard  have  confirmed  this 
fact.  They  have  described  (a)  a  latent  neuritis  in  which  lesions  in  the 
nerves  were  found  post-mortem,  but  no  symptoms  had  appeared  ;  (6) 
neuritis  causing  paralysis  and  atrophy  in  the  muscles,  and  (c)  neuritis 
with  sensory  symptoms  only.  They  are  inclined  to  ascribe  many  of 
the  nervous  disturbances  arising  in  the  course  of  consumption  to  an 
aflFectiou  of  the  peripheral  nerves ;  and  other  writers  have  confirmed 
these  views  and  have  recorded  cases  in  which  all  forms  of  cranial  and 
spinal  neuritis  have  appeared.  But  here,  again,  the  neuritis  must  not 
be  termed  tuberculous,  as  it  has  nothing  characteristic  of  that  affection 
in  its  pathology. 

MULTIPLE  NEURITIS  AND   SYPHILIS. 

There  has  been  much  discussion  in  regard  to  the  existence  of  a 
syphilitic  multiple  neuritis.  The  admitted  frequency  of  syphilitic 
exudations  in  nerve  trunks,  especially  in  the  cranial  nerves  and  about 
the  roots  of  the  spinal  nerves,  and  the  admitted  existence  of  gummy 
growths  in  the  plexuses  and  in  the  nerves,  and  of  syphilitic  endarteritis 
in  the  vessels  accompanying  the  nerves  have  made  it  seem  not  improb- 
able that  a  true  syphilitic  multiple  neuritis  might  occur.  The  preva- 
lence of  syphilis  has  resulted  in  the  development  of  multiple  neuritis 
in  many  patients  who  were  syphilitic,  and  this  has  also  awakened  the 
suspicion  that  syphilis  might  be  the  cause  of  the  neuritis.  The  syphi- 
litic poison  has  been  designated  by  some  authors  as  the  cause  in  these 
cases ;  1)y  others  the  effects  have  been  ascribed  to  the  post-syphilitic 
toxin  which  seems  to  play  such  a  prominent  part  in  the  production  of 
tabes.  Cases  have  been  reported  in  all  stages  of  the  disease.  It  is 
only  in  a  few  of  the  reported  cases,  however,  that  a  true  syphilitic 

^Vierordt.     Arch.  f.  Psych.,  1883,  vol.  xiv.,  p.  3, 


256  MULTIPLE  NEURITIS  AND   OTHER  DISEASES. 

multiple  neuritis  can  be  admitted.  In  the  cases  of  Fordyce/  Spell- 
mann  and  Etienne/  and  Fry,^  the  development  of  multiple  neuritis  in 
the  course  of  the  second  stage  of  syphilis,  the  accompanying  numerous 
syphilitic  deposits  in  the  skin  and  periosteum,  and  the  rapid  improve- 
ment under  antisyphilitic  treatment  give  a  certain  probability  to  the 
assertion  that  these  cases  were  of  specific  origin.  It  must  be  acknowl- 
edged, however,  that  such  cases  are  extremely  rare.  At  a  discussion 
at  the  New  York  Neurological  Society  it  was  found  that  no  one  of  the 
members  had  seen  a  case  of  multiple  neuritis  undoubtedly  syphilitic, 
and  a  review  of  the  literature  has  convinced  me  that  many  of  the  cases 
reported  as  such  were  not  actually  due  to  the  disease. 

MULTIPLE  NEURITIS  AND  CANCER. 

Auche  *  collected  from  the  literature  and  published  a  number  of  cases 
of  neuritis  developing  in  the  course  of  carcinoma,  especially  of  the 
stomach.  Some  of  these  were  local  neuritis  due  to  a  direct  extension 
of  the  carcinoma  into  adjacent  nerve  trunks.  Others  were  general 
multiple  neuritis  in  the  peripheral  branches.  The  lesions  were  found 
post-mortem  to  be  of  the  type  of  degeneration  in  the  finer  nerve  fila- 
ments, not  unlike  those  found  by  Pitres  and  Vaillard  in  tuberculous 
patients.  In  some  of  these  cases  no  symptoms  had  appeared  during 
life.  In  others  minor  sensory  symptoms  were  noticed :  parsesthesise, 
and  ansesthesise,  and  pain.  In  a  few  cases  the  symptoms  of  a  general 
multiple  neuritis,  typical  sensory  and  motor  paralysis  developed.  This 
was  seen  in  cases  of  Miura  and  Remak,^  where  the  autopsy  confirmed 
the  diagnosis.  In  this  connection  it  is  to  be  remembered  that  proc- 
esses of  degeneration  are  going  on  continually  in  normal  nerves  in  a 
state  of  health,  that  these  processes  are  undoubtedly  intensified  and 
regeneration  is  delayed  in  conditions  of  wasting  disease,  such  as  tuber- 
culosis and  carcinoma,  and  also  in  senile  conditions  or  where  the  circu- 
lation and  nutrition  are  interfered  with,  as  in  states  of  extensive  arterial 
sclerosis.  Hence,  it  is  not  proper  to  lay  too  much  stress  upon  lesions 
of  this  kind  found  after  death  when  no  symptoms  have  appeared  dur- 
ing life.  And  while  "  latent  neuritis "  may  awaken  interest  as  a 
pathological  curiosity,  it  is  of  no  importance  to  the  clinician. 

MULTIPLE  NEURITIS  OF  UNKNOWN  ORIGIN. 

A  certain  number  of  cases  of  multiple  neuritis  develop  after  expo- 
sure to  cold.  A  few  develop  after  unusual  exertion,  and  quite  a 
number  occur  without  any  ascertainable  cause. 

It  is  true  that  the  more  closely  so-called  idiopathic  or  spontaneous 
cases  are  scrutinized,  in  the  light  of  our  knowledge  that  a  great  variety 

^Boston  Medical  and  Surgical  Journal,  1890,  p.  39. 
^Eevue  neurologique,  1897,  p.  28. 
^Journal  of  Nervous  and  Mental  Disease,  1898,  p.  594. 
*Kevue  de  m^.,  October,  1890. 

5 Berliner  klin.  Wochen.,  1891,  p.  905,  JSTothnagel's  spec.  Path.  ll.  Ther.,  Th,  ii., 
p.  312. 


MULTIPLE  NEURITIS  OF  UNKNOWN  ORIGIN.  257 

of  causes  are  competent  to  produce  multiple  neuritis,  the  more  likely 
we  are  to  discover  some  cause.  Thus,  many  cases  which  have  been 
reported  as  idiopathic  can  now  be  traced  to  tuberculous,  carcinomatous, 
gouty,  rheumatic,  or  diabetic  conditions  known  to  exist  in  the  patients, 
but  not  formerly  supposed  to  have  any  causal  relation  to  neuritis. 
There  are  numerous  cases  of  "idiopathic"  neuritis  which  are  preceded 
by  severe  gastro-enteritis.  Is  it  not  possible  that  an  acute  intoxication  or 
ptomaine  poisoning  capable  of  causing  the  gastro-intestinal  symptoms 
may  produce  a  general  neuritis?  This  question,  which  has  occurred  to 
several  recent  writers  on  the  subject,  seems  to  be  answered  affirmatively 
by  such  observations  as  are  collected  by  Remak  from  recent  literature. 

It  is  possible  that  in  some  of  these  "idiopathic"  cases  there  may  be 
acute  infection  as  the  basis  of  the  neuritis,  inasmuch  as  some  of  the 
cases  develop  with  an  acute  febrile  attack,  a  chill,  temperature  rising 
to  102°  or  104°  F.,  and  continuing  for  several  days  with  the  general 
manifestations  of  the  febrile  movement,  a  rapid  pulse,  nausea  and 
vomiting,  diarrhoea,  occasionally  jaundice,  malaise,  catarrhal  symptoms 
in  the  various  mucous  membranes,  and  a  general  condition  of  acute 
sickness,  such  as  is  indicative  of  an  infection.  Remak  ^  has  laid  great 
stress  upon  these  spontaneous  cases  and  has  argued  from  the  general 
constitutional  disturbance  that  they  are  always  infectious.  He  cites 
cases  by  Striimpell,  Rosenheim,  and  Putnam,  which  were  attended  by 
a  marked  swelling  of  the  spleen,  in  support  of  this  hypothesis  of  infec- 
tion. I  am  inclined  to  agree  with  Raymond  [Legons,  1897)  that  very 
many  cases  of  paralysis  hitherto  ascribed  to  spinal-cord  disease,  but 
resulting  in  recovery,  are  actually  cases  of  this  nature,  such  as  the 
cases  recently  described  by  Dana^  as  acute  ataxia,  and  many  cases  of 
so-called  acute  poliomyelitis  in  adults. 

Symptoms.  —  The  symptoms  arising  in  these  cases  do  not  differ  in 
any  particular  manner  from  the  symptoms  occurring  in  alcoholic  neu- 
ritis, though  Schultze  is  inclined  to  divide  the  cases  into  three  categories, 
according  to  the  prevalence  of  sensory^  motor,  or  ataxic  symptoms. 
The  general  description,  therefore,  of  the  symptoms  of  multiple  neu- 
ritis already  given  is  sufficient  for  these  cases,  and  if  the  points  that 
are  mentioned  under  the  head  of  diagnosis  (page  201)  are  carefully 
considered  it  will  be  possible  to  separate  these  cases  from  other  affec- 
tions producing  somewhat  similar  symptoms.  The  cerebral  nerves  do 
not  escape  in  these  cases,  and,  in  fact,  any  of  the  symptoms  which 
have  already  been  considered  under  the  heads  of  alcoholic  multiple 
neuritis,  arsenical  multiple  neuritis,  and  diphtheritic  multiple  neuritis 
may  develop  in  the  course  of  these  cases.  In  a  few  cases  optic  neuritis 
has  been  observed,  though,  as  already  stated,  this  complication  is  rare. 
Sometimes  painful  swelling  of  the  joints  has  been  noticed  at  the 
beginning  or  in  the  course  of  the  cases  of  idiopathic  polyneuritis,  and 
it  has  been  a  matter  of  discussion  whether  such  joint  affection  was 
secondary  to  the  neuritis  or  an  independent  rheumatic  state. 


'  Nothnagel'H  spec.  Pathol,  u.  Therapie,  Bd.  xi.,  Tli.  ii. 
^Jour.  Ncrv.  and  Mont.  Dis.,  1901,  p.  105. 


17 


258  MULTIPLE  NEURITIS  AND   OTHER  DISEASES. 

Korsakow^  has  affirmed  that  cerebral  symptoms,  such  as  have  al- 
ready been  described  as  occurring  in  alcoholic  neuritis,  namely  loss  of 
memory,  or  delusions  of  memory,  or  disturbances  of  memory,  or  even 
a  state  approaching  dementia,  may  develop  in  the  course  of  these 
cases.  *  I  have  never  seen  these  psychical  symptoms,  however,  in  any 
but  the  alcoholic  types  of  the  disease. 

SENILE  POLYNEURITIS. 

A  form  of  multiple  neuritis  occurring  in  old  age  and  not  produced  by 
any  of  the  known  causes  of  neuritis,  and  not  attended  by  any  of  the 
acute  symptoms,  has  been  observed.  This  type  of  neuritis  has  been 
described  chiefly  by  Oppenheim.^  I  have  seen  several  cases  of  this 
affection.  Patients  are  usually  persons  over  the  age  of  seventy  years 
and  are  often  the  subjects  of  arterial  sclerosis.  The  symptoms  develop 
slowly.  There  is  increasing  weakness,  with  numbness  in  the  lower 
extremities,  and  then  in  the  upper  extremities ;  but  the  patients  do 
not  suflFer  from  sharp  pain  or  from  tenderness  along  the  nerves.  There 
is  a  progressive  condition  of  paresis,  which  is  much  more  marked  in 
the  hands  and  forearms  and  in  the  feet  and  legs  than  in  the  proximal 
portion  of  the  extremities,  and  is  rarely  attended  by  any  symptoms  in 
the  nerves  of  the  head,  eyes,  or  face.  In  some  cases  atrophy  has  at- 
tended the  paresis  and  there  has  been  a  diminution  in  the  contractility 
of  the  muscles  to  both  currents.  Knee-jerks  are  lost.  There  is  rarely 
any  ataxia.  Very  frequently  the  general  weakness  leads  to  a  tremor, 
which,  however,  may  be  considered  as  one  of  old  age.  In  one  case 
that  I  observed,  where  the  neuritis  occurred  in  a  person  who  had  long 
suflPered  from  chronic  arthritis,  the  joints  that  had  previously  been  stiif 
and  deformed  became  quite  relaxed,  so  that  very  abnormal  positions 
could  be  given  to  the  fingers  and  toes  and  to  the  wrists  and  ankles, 
without  any  perception  of  pain.  In  this  case  there  was  a  complicating 
gangrene  of  one  leg.  It  remained  for  a  long  time  after  the  symptoms 
of  the  neuritis  had  subsided  and  the  paralysis  disappeared.  The  fixed 
deformity  of  the  joints  returned  with  the  subsidence  of  the  paralysis. 
In  spite  of  their  age,  these  patients  do  not  always  die  of  the  disease, 
and  may  recover  quite  completely.  Stein  ^  has  recently  recorded  a  case 
in  which  the  muscles  of  the  eyes  were  paralyzed  for  a  short  time. 

In  the  treatment  of  this  form  of  multiple  neuritis  the  importance  of 
good  food  should  be  remembered,  and  the  free  use  of  stimulants  may 
be  required. 

iZeitsch.  f.  Psych.,  vol.  xlvi.,  p.  475,  1890.     Arch.  f.  Psych.,  vol.  xxi.,  p.  669. 
2  Berliner  klin.  Wochen.,  1893,  p.  589. 
'Munchener  raed.  Wochen.,  1897,  p.  463. 


SECTION  II. 
DISEASES  OF  THE  SPINAL  CORD. 


CHAPTER  XIII. 

THE   SPINAL   BLOODVESSELS. 

The  maiu  arteries  of  the  spinal  cord  are  three  in  number.  They 
lie  on  the  anterior  and  posterior  surfaces  of  the  organ  along  its  entire 
length.  The  anterior  spinal  artery  is  formed  by  the  junction  of  two 
vessels  which  arise  from  the  vertebral  arteries  and  it  extends  to  the 
lowest  part  of  the  spinal  cord.     The  posterior  spinal  arteries  also  arise 

Fig.  106. 


a.  spin,  postf 


a.  spin.  post. 


Scheme  to  show  the  course  and  distribution  of  the  terminal  branches  of  the  arterial  plexus  of  the 
pia  mater,  a.  spin. post.,  posterior  spinal  arteries;  a.  spin,  ant.,  anterior  spinal  arteries ;  a.  sil.,  ante- 
rior median  fissure  ;  rac.  ant.,  anterior  root  arteries.     (After  Van  Gehuchten.) 

from  the  vertebral  arteries,  but  do  not  often  join.  They  pass  down- 
ward along  the  surface  of  the  cord  on  each  side  near  the  entry  of  the 
posterior  spinal  nerve  roots.  (See  Plate  VIII.)  In  addition  to  these 
main  arteries  there  are  a  large  number  of  smaller  arteries,  branches  of 

259 


260  TEE    l^PINAL    BLOODVESSELS. 

the  intercostal  arteries,  wliich  enter  the  spinal  canal  at  the  sides  of  the 
spinal  nerves  and  are  distributed  to  the  antero-lateral  surfaces  of  the 
cord,  freely  anastomosing  with  one  another.  These  have  been  called 
anterior  and  posterior  radicular  arteries.  They  divide  into  ascending 
and  descending  branches  and  anastomose  freely  with  the  anterior  and 
posterior  spinal  arteries,  making  a  sort  of  arterial  network  about  the 
spinal  cord  from  which  numerous  small  branches  penetrate  the  surface 
of  the  organ.  The  number  of  these  radicular  arteries  is  variable,  but 
they  are  never  present  on  all  the  nerves,  every  second  or  third  nerve 
having  an  accompanying  vessel.  There  is  a  fold  of  pia  mater  in  the 
anterior  fissure  of  the  spinal  cord,  and  in  this  fold  a  large  branch  of 
the  anterior  spinal  arteries  is  usually  found  running  longitudinally. 
From  this  branch  secondary  branches  enter  the  gray  matter  of  the 
spinal  cord  and  pass  down  in  it,  forming  the  central  arteries  of  the 
cord.  Thus  the  supply  of  the  gray  matter  and  white  matter  of 
the  cord  is  from  different  branches.  Fig.  107  demonstrates  this  dis- 
tribution. All  of  the  spinal  arteries  which  enter  the  cord  are  terminal 
arteries  like  the  basal  arteries  of  the  brain ;  that  is,  they  do  not  anas- 
tomose. Hence  an  embolus  in  a  spinal  vessel  always  leads  to  an  area 
of  softening.  The  peripheral  spinal  branches  which  penetrate  the 
organ  from  its  surface  enter  with  the  connective-tissue  septa  or  with 

Fig.  107  . 


The  distribution  of  the  anterior  sjiinal  artery  and  vein  to  the  gray  matter  of  the  spinal  cord.    An 
injected  preparation.     (Adamkiewicz.) 

the  nerve  roots.  They  supply  the  white  matter  of  the  cord,  and  their 
terminal  twigs  reach  the  edge  of  the  gray  matter  and  sometimes  enter 
it,  but  they  do  not  anastomose  with  the  branches  of  the  central  artery. 
The  fine  capillaries  within  the  gray  matter  empty  into  veins  which 
accompany  the  central  arteries  and  empty  into  larger  veins  which  lie 
in  the  anterior  fissure.  The  majority  of  the  spinal  capillaries,  how- 
ever, empty  into  fine  venous  twigs  which  make  their  exit  on  the  lateral 
and  posterior  surfaces  of  the  cord  where  a  venous  network  surrounds 
the  cord.  This  in  turn  empties  into  larger  veins  which  make  their 
exit  from  the  spinal  canal  with  the  spinal  nerves,  and  thus  reach  the 


TEE    SPINAL    BLOODVESSELS.  261 

vena  cava.     Others  ascend  to  empty  into  the  branches  of  the  jugular 
vein  withi^  the  skull. 

While  little  attentioO  has  hitherto  been  paid  to  diseases  of  the  spinal 
bloodvessels  and  to  the  results  of  endarteritis,  it  is  probable  that  these 
play  a  large  part  in  the  production  of  various  forms  of  spinal-cord 
disease.  I  have  seen  a  number  of  cases  of  injury  to  the  spine  in  which 
a  sudden  development  of  symptoms  indicating  a  transverse  total  lesion 
were  followed  by  a  gradual  and  complete  recovery.  I  have  also  seen 
similar  cases  without  injury.  I  saw  a  college  football  player  after  an 
injury  on  the  field  who  was  totally  paralyzed  below  the  neck.  On  the 
third  day,  when  I  saw  him,  he  lay  with  the  arms  abducted  and  fore- 
arms flexed,  the  deltoids  alone  not  being  paralyzed,  and  he  was  anaesthetic 
below  the  fifth  rib.  There  was  retention  of  urine  and  priapism.  There 
was  no  spinal  fracture.  The  prognosis  was  considered  a  bad  one  as 
the  probability  of  an  injury  to  the  cord  seemed  great.  But  after  a 
week  improvement  began,  by  the  end  of  a  month  he  could  move  his 
limbs  freely  and  had  recovered  sensation  and  at  the  end  of  three 
months  he  was  quite  well.  In  these  cases  the  only  explanation  of  the 
symptoms  possible  was  that  a  hemorrhage  either  outside  or  inside  of 
the  spinal  dura  had  occurred  ;  had  produced  pressure  enough  to  suspend 
the  function  of  the  spinal  cord  below  the  lesion  ;  but  had  been  grad- 
ually absorbed ;  and  as  the  pressure  of  the  clot  was  removed  the  spinal 
functions  were  resumed.  Since  the  discovery  of  the  characteristic 
symptoms  of  syringomyelia  it  has  been  possible  to  diagnosticate  hem- 
orrhages within  the  gray  matter  of  the  cord  by  the  sudden  appearance 
of  similar  symptoms  ;  ^  and  recent  pathological  study  ^  seems  to  indicate 
that  many  supposed  cases  of  myelitis  are  really  cases  of  softening  in 
the  cord  due  to  thrombosis  in  diseased  bloodvessels,  and  that  some 
forms  of  sclerosis  may  originate  in  obliterating  endarteritis  of  the  per- 
ipheral vessels  that  enter  the  posterior  and  lateral  columns  of  the  spinal 
cord.  It  will  be  necessary  in  the  chapters  upon  tabes  and  upon  lateral 
sclerosis  to  call  attention  more  particularly  to  endarteritis  as  a  possible 
cause  of  some  cases  of  these  diseases. 

1  Wm.  Browning.     Medical  News,  October  7,  1905. 

^E/.  J.  Williamson.     Manchester  Medical  Chronicle,  1895. 


CHAPTER  XIV. 

POLIOMYELITIS. 

Poliomyelitis  ,  Infantile  spinal  paralysis  ;  Epidemic  and  sporadic  forms . 

Poliomyelitis,  infantile  spinal  paralysis,  acute  atrophic  paralysis, 
atrophic  spinal  paralysis  or  regressive  paralysis  is  an  acute  infectious 
disease,  observed  chiefly  among  children  but  also  among  adults,  char- 
acterized by  sudden  complete  loss  of  power  in  one  or  more  limbs, 
usually  in  the  legs ;  occasionally  in  the  trunk  or  face ;  followed  by 
rapid  atrophy  of  the  paralyzed  muscles  and  by  an  imperfect  growth  of 
the  limb  affected,  and  attended  at  the  onset  by  pain  -but  not  by  per- 
manent sensory  disorder. 

Etiology.  —  The  cause  of  the  disease  is  the  presence  in  the  body  of  a 
micro5rganism  of  such  minute  size  as  to  be  invisible  under  our  present 
microscopes.  The  microorganism  is  soluble,  and  may  be  obtained  from 
the  cerebrospinal  fluid  or  from  the  gray  matter  of  the  spinal  cord  in 
inoculated  monkeys  or  in  persons  suffering  from  the  disease. 

The  disease  is  infectious  and  probably  contagious,  and  as  the  con- 
tagious material  has  been  found  in  the  mucus  of  the  mouth  and  nose 
it  is  probably  disseminated  through  this  mucus  and  after  drying  gets 
into  the  air  and  is  inhaled.  From  the  nasal  mucosa  it  passes  along 
the  filaments  of  the  olfactory  nerves  through  the  cribriform  plate  into 
the  brain.  The  disease  may  be  carried  from  place  to  place  by  persons 
not  affected.  In  epidemics  it  always  follows  the  lines  of  travel,  town 
after  town  being  invaded.  It  is  supposed  that  it  may  also  be  carried 
by  insects  or  by  animals,  as  animals  of  all  kinds  are  susceptible  to  it. 
The  period  of  incubation  is  from  5  to  13  days.  It  is  uncertain  how 
long  a  person  affected  may  convey  the  disease  to  others,  but  the  virus 
has  been  found  active  in  the  mucus  of  monkeys  40  days  after  the  active 
febrile  symptoms  had  ceased  and  can  survive  an  indefinite  time  when 
dried.  One  attack  confers  immunity.  This  is  associated  with  the 
appearance  in  the  blood  of  the  patient  of  definite  principles  which  have 
the  power  of  destroying  the  activity  of  the  virus ;  so  that  if  some  of 
this  blood  is  mixed  with  the  virus  obtained  from  a  fatal  case  and  a 
monkey  is  inoculated  with  the  mixture  it  will  not  develop  the  disease. 
This  test  may  determine  when  a  suspected  person  has  had  the  disease.^ 

The  disease  has  been  frequently  observed  in  connection  with  other  in- 
fectious diseases.  Thus  it  is  an  occasional  sequel  of  diphtheria,  menin- 
gitis, measles,  pneumonia,  scarlet  fever,  and  acute  malarial  poisoning. 
It  has  also  appeared  during  an  attack  of  furunculosis. 

'  Flexner.     N.  Y.  Med.  Kecord,  Oct.  28,  1911. 
262 


MtOLOG^. 


^63 


Exposure  to  cold  or  to  a  sudden  check  of  perspiration  has  been  sup- 
posed to  be^an  exciting  cause  in  certain  cases.  I  have  seen  several 
children  who  were  attacked  with  infantile  spinal  paralysis  subsequent 
to  long-continued  bathing  in  cold  water  during  the  sununer.  Trauma- 
tism is  frequently  assigned  as  a  cause  by  parents,  and  a  few  cases  are 
on  record,  which  I  can  confirm  by  my  own  observations,  in  which  the 
disease  has  developed  immediately  after  a  severe  fall  or  blow  on  the 
back.  These  contributing  causes  act  by  weakening  the  resistance  of 
the  body  to  the  invasion  of  the  germ  of  the  disease.  In  some  cases  no 
cause  can  be  discovered. 

The  frequency  with  which  the  disease  appears  in  children  who  are 
learning  to  walk,  together  with  the  fact  that  the  symptoms  are  located 
much  more  commonly  in  the  legs  than  in  the  arms,  has  led  to  the  sup- 
position that  a  functional  hyperemia  of  the  cord  in  its  lumbar  region, 
due  to  overexertion,  going  on  to  a  pathological  congestion  and  hemor- 
rhage, may  be  an  etiological  factor. 

The  disease  occurs  in  both  sexes  with  about  equal  frequency.  There 
is  no  history  of  its  being  inherited.  The  following  table  demonstrates 
that  the  age  of  maximum  liability  is  between  the  first  and  fourth  years, 
but  children  at  all  ages  are  liable  to  the  disease,  and  it  occurs  in  adult 
life  : 

Table  V.  — Age  of  Onset. 


1st  year. 

M. 

Sd. 

hth. 

5lh. 

6th. 

7th. 

8th. 

9lh. 

loth. 

271 

560 

436 

183 

120 

67 

36 

31 

17 

17 

The  youngest  case  on  record  is  mentioned  by  Collins  in  a  child  four 
days  old.  The  youngest  patient  in  my  own  records  was  four  weeks 
old. 

It  has  long  been  noticed  (Barlow  1878)  that  the  majority  of  the 
cases  were  seen  during  the  summer  months  and  the  following  table 
confirms  this. 


January 
February 
March  . 
April   , 
May     . 
June     . 
July     . 
August. 
September 
October 
November 
December 


Table  VI.  — Month  of  Onset. 


Barlow.        Gowers.      Sinkler. 


53 


Starr.        Collins.        Lovett. 


70 


509 


202 


343 


239 


Total. 


1 

14 

5 

2 

8 

31 

0 

8 

2 

1 

4 

16 

4 

11 

9 

1 

5 

31 

2 

21 

4 

0 

5 

23 

4 

21 

5 

1 

6 

38 

5 

11 

61 

10 

22 

13 

122 

16 

13 

109 

42 

77 

36 

293 

11 

13 

124 

57 

109 

43 

359 

4 

15 

79 

41 

125 

47 

311 

3 

6 

45 

18 

35 

39 

146 

1 

2 

12 

6 

10 

29 

60 

2 

5 

12 

3 

0 

4 

26 

1,456 


Epidemics  of  the  disease  have  now  been  observed  in  many  countries 
of  Europe  and  in  many  parts  of  America.  Some  years  ago  such  epi- 
demics were  a  curiosity  and  in  previous  editions  of  this  work  a  number 
of  these  epidemics  were  fully  described.     They  have  however  so  in- 


264  POLIOMYELITIS. 

creased  in  frequency  and  have  extended  all  over  the  world  with  such 
rapidity  that  description  of  single  epidemics  would  now  require  an 
entire  volume.^  They  have  always  occurred  in  warm  weather,  usually 
in  the  months  of  July  and  August,  but  in  Australia  they  have  occurred 
in  the  corresponding  warm  months  of  January  and  February.  The 
earliest  epidemics  of  any  size  were  noticed  in  Sweden  and  Norway  and 
it  has  been  thought  probable  that  the  disease  has  been  spread  all  over 
the  world  by  emigrants  from  Sweden  and  Norway,  though  this  is  not 
by  any  means  proven.  It  is  only  within  the  last  year  that  the  disease 
has  appeared  in  epidemic  form  in  England.  In  America  these  epidem- 
ics have  been  wide  spread  all  over  the  United  States,  and  they  have 
attracted  much  attention  because  of  the  facts  that  the  epidemic  type  of 
disease  is  much  more  fatal  and  the  cases  vary  very  much  in  severity, 
while  in  the  sporadic  cases  death  is  rare.  The  knowledge  of  the  disease 
has  been  very  much  extended  by  the  study  of  cases  appearing  in  these 
epidemics.  It  is  probable  that  many  mild  sporadic  cases  occur  which 
are  not  recognized  as  poliomyelitis. 

Pathology.  —  The  lesions  that  have  been  described  in  the  earlier 
text-books  were  those  that  are  seen  in  the  terminal  condition  after  the 
disease  had  been  present  for  some  time,  and  for  a  long  time  it  was  sup- 
posed that  they  were  limited  to  the  anterior  horns  of  the  spinal  cord 
and  consisted  chiefly  in  a  degeneration  of  the  motor  cells  in  these  horns 
and  a  replacement  of  the  cells  by  connective  tissue.  This  was  the 
pathological  appearance  described  by  Charcot.  Later  on  the  observa- 
tions of  Dreschfeld  and  others  established  the  fact  that  the  disease 
began  with  an  acute  congestion  of  the  entire  gray  matter  of  the  cord 
with  hemorrhages  from  the  capillaries  and  an  interstitial  inflammation  as 
well  as  a  degeneration  of  the  motor  neurones. 

The  study  of  material  secured  in  the  recent  epidemics  has  again 
modified  our  ideas  of  the  pathology  of  the  disease  and  it  is  now  agreed 
that  the  microorganism  acting  as  an  irritant  produces  an  active  conges- 
tion and  inflammation  of  the  meninges  and  of  the  spinal  cord  itself  both 
in  the  gray  and  in  the  white  matter,  the  gray  matter  being  however 
chiefly  affected  on  account  of  its  greater  supply  of  blood  through  the 
branches  of  the  anterior  spinal  artery.  The  cerebral  axis,  the  medulla, 
pons  and  crura  cerebri  are  often  involved  as  well  as  the  spinal  cord. 
The  degree  of  inflammation  in  the  meninges  appears  to  vary  greatly  in 
accordance  with  the  severity  of  the  disease,  being  so  intense  in  the 
severer  cases  as  to  produce  the  clinical  picture  of  a  meningitis.  In  the 
milder  cases  it  is  probable  that  the  meninges  are  merely  congested  and 
that  the  chief  pathological  changes  are  limited  to  the  gray  matter  in 
the  domain  of  the  anterior  spinal  arteries.  In  the  mildest  of  all  cases, 
now  known  as  the  abortive  type,  it  is  probable  that  the  lesion  is  merely 
an  intense  congestion  of  these  vessels,  possibly  with  capillary  hemor- 
rhages which  are  subsequently  absorbed  so  that  no  permanent  lesion  is 
left.  There  is  every  probability  that  many  cases  occur  of  this  last 
type  which  are  never  recognized  as  cases  of  poliomyelitis. 

*See  Epidemic  Infantile  Paralysis.    Starr,  Joum.  Amer.  Med.  Assoc,  July  11,  1908. 


PATHOLOGY.  265 

Figures  108  to  111  demonstrate  the  lesions  that  are  found  in  cases 
that  succumb  soon  after  the  onset.  There  is  a  distension  of  the  blood 
vessels  botn  in  the  meninges  and  in  the  gray  matter  of  the  spinal  cord 
and  many  minute  hemorrhages  in  the  gray  matter.  There  is  an  infil- 
tration of  the  meninges  and  of  the  cord  with  serum  and  with  emigrat- 
ing leucocytes.  The  serum  fills  the  lymph  spaces  about  the  vessels 
and  about  the  nerve  cells,  the  leucocytes  infiltrate  the  tissues  every- 
where, cluster  about  the  cells,  and  make  their  way  into  the  cells. 
Throughout  the  neuroglia  there  is  a  great  increase  of  small  cells  and 
nuclei  which  may  be  so  intense  as  to  obscure  the  other  elements.  The 
degenerative  processes  in  the  ganglion  cells  or  motor  neurones  appear 
early,  all  varieties  of  degenerative  changes  being  seen. 

Fig.  108. 


Acute  poliomyelitis.  Death  10  days  after  onset.  Lumbar  region.  Distension  of  blood  vessels 
and  absence  of  motor  cells  is  seen.  The  serious  infiltration  obscures  the  outline  of  gray  matter. 
(Larkin.) 

The  cell  may  have  a  cloudy  appearance  and  be  slightly  swollen, 
staining  more  deeply  by  reagents,  the  chromophile  granules  appearing 
to  be  larger  than  in  the  normal  cells,  as  seen  by  the  Nissl  stain,  and 
the  nucleus  appearing  granular.  This  is  shown  in  Plate  III.,  B 
(page  34).  A  further  stage  of  degeneration  is  shown  by  the  fact  that 
the  protoplasm  no  longer  absorbs  stains ;  the  cell  is  swollen,  has  lost  its 
sharp  outline,  has  a  homogeneous  appearance,  and  the  nucleus  is  faint, 
as  are  also  the  outlines  of  some  of  the  dendrites.  It  is  probable  that 
in  both  these  stages  of  degeneration  an  arrest  of  the  process  and  a 
gradual  regeneration  and  a  return  to  the  condition  of  health  with  re- 
sumption of  the  function  of  the  cell  is  possible.  If  the  process  of  de- 
generation proceeds  beyond  this  point,  however,  no  repair  is  possible, 
and  the  function  of  the  cell  is  forever  lost. 


266  POLIOMTELlTtS. 

Then  the  cell  appears  to  be  changed  into  a  swollen,  irregular,  oi* 
spherical  mass  of  material ;  its  protoplasm  becomes  cloudy  and  its 
nucleus  is  not  visible,  if  stained  at  all ;  the  chromophile  granules  have 
lost  their  regular  arrangement  in  concentric  rings  about  the  nucleus 
or  in  radiating  lines  toward  a  neuraxone,  and  the  homogeneous  mass  is 
seen  to  be  permeated  with  vacuoles,  the  dendrites  having  dropped  off. 
(See  Plate  III.,  c,  d.)  In  the  last  stage  the  protoplasm  shrinks,  the 
cell  body  being  reduced  so  as  to  be  no  larger  than  its  original  nucleus. 
It  stains  deeply  and  has  a  granular  appearance.  During  the  later 
stages  of  this  degeneration  leucocytes  may  be  observed  penetrating  the 

Fig.  109. 


Acute  poliomyelitis.  The  distended  vessels,  the  capillary  hemorrhages,  the  infiltration  of  the 
entire  gray  matter  with  small  cells,  and  the  destruction  of  motor  cells  is  evident.  Contrast  this  figure 
with  Fig.  20,  showing  normal  appearance  at  this  level.     (Larkin.) 

pericellular  spaces  and  encroaching  upon  the  cell  body.  Both  in  the 
dendrites  and  in  the  neuraxone  similar  degenerative  processes  may  be 
observed  in  progress,  and  they  are  destroyed  before  the  cell  undergoes 
its  final  degeneration.     (See  Plate  III.,  E,  r,  g,  page  34.) 

While  in  the  majority  of  cases  there  is  a  parallel  degree  of  change 
in  the  interstitial  tissues  and  in  the  ganglion  cells,  so  that  there  is  a 
shrinkage  and  progressive  destruction  of  the  neuroglia  as  well  as  of 
the  nerve  elements,  there  are  a  few  cases  in  which  the  cellular  degenera- 
tion is  attended  by  few  changes  in  the  interstitial  tissues.  The  degree 
to  which  various  groups  of  cells  are  affected  varies  greatly  at  different 
levels  of  the  cord,  and  in  some  groups  a  larger  number  of  cells  may 
be  affected  than  in  others.  The  extent  of  the  paralysis  and  the  degree 
of  the  paralysis   in  any  one  muscle  will  necessarily  depend  upon  the 


PATSOLOGT. 


267 


number  of  groups  of  cells  affected  and  upon  the  number  of  cells  de- 
stroyed in  ajAj  one  group. 

The  result  of  the  atrophy  of  cells  and  of  the  neuroglia  is  a  gradual 
shrinkage  of  the  entire  area  of  the  anterior  horn,  leading  secondarily 
to  a  collapse  inward  of  the  white  columns  surrounding  the  gray  matter 
and  of  the  nervous  fibre  issuing  from  the  horn  through  these  white 
columns  into  the  anterior  nerve  roots.     There  is  also  a  degenerative 

Fig.  110. 


Acute  poliomyelitis  several  years  after  onset.     Upper  lumbar  region.    Diffuse  degeneration  in  both 
anterior  horns  and  central  gray  matter  with  liquefaction  of  tissue  in  the  left  anterior  horn.    (Larkin.) 

atrophy  of  fibres  in  the  anterior  nerves.  Many  of  the  cells  in  the 
anterior  horns  of  the  cord  send  their  axones  to  the  antero-lateral 
column,  where  they  turn  upward  and  downward  to  pass  to  other  levels 
and  terminate  in  the  anterior  horn,  thus  serving  to  associate  the  action 
of  different  cells  lying  at  different  levels  of  the  cord.  These  associa- 
tion cells  as  well  as  the  motor  cells  suffer  from  degeneration,  and 
hence  there  is  a  secondary  degenerative  process  in  their  axones,  leading 
to  a  shrinkage  and  slight  sclerosis  in  the  antero-lateral  column  of  the 
cord  for  a  varying  distance  above  and  below  the  seat  of  the  lesion. 
The  extent  of  the  lesion  in  the  gray  matter  varies  in  various  cases. 
In  a  few  cases  tlie  pathological  change  is  strictly  limited  to  the  anterior 
horn,  being  particularly  severe  in  its  peripheral  region.  In  the  majority 
of  cases  the  central  gray  matter  which  lies  between  the  anterior  and 
posterior  horns  is  also  affected.  It  is  in  these  hitter  cases  that  the 
growth  of  the  limbs  is  intcirfered  with  in  the  chronic  stage,  the  central 
gray  matter  of  the  cord  having  a  closer  relation  to  the  growth  of  the 
tissues  tlian  other  parts. 


268  POLIOMYELITIS. 

Similar  lesions  have  been  found  in  the  medulla  oblongata,  in  the  pons 
and  crura  cerebri,  involving  all  parts,  but  chieflj  affecting  the  motor 
nerve  nuclei. 

In  addition  to  the  pathological  changes  in  the  meninges  and  cord  and 
brain  it  has  been  found  that  the  cerebrospinal  fluid  undergoes  changes. 
It  becomes  opalescent  and  then  cloudy.  It  contains  lymphocytes  both 
small  and  large,  and  it  contains  also  iDolynucloar  leucocytes.  It  gives 
a  protein  reaction  when  tested  by  Noguchi's  butyric  acid  test.  This 
condition  of  the  cerebrospinal  fluid  appears  early  in  the  disease  and 

Fig.  111. 


'^^^jS^^^^^^^^^^^k 

Poliomyelitis.  Higher  power,  samecase  as  Fig.  104,  showing  complete  ahsence  of  cells  in  the  horn; 
hlood  Tessels  and  scar  tissue  remaining,  and  liquefaction  of  the  tissue.  The  child's  thigh  and  leg  were 
atrophic  and  totally  jiaralyzed.     (Larkin.) 

may  be  utilized  as  a  means  of  diagnosis ;  the  fluid  being  drawn  by 
lumbar  puncture  and  tested.  In  a  case  under  Flexner's  care  the  diag- 
nosis was  reached  a  day  before  the  appearance  of  paralysis  by  this  test.^ 

Recent  observations  ^  have  shown  that  in  addition  to  the  nervous 
lesions  in  poliomyelitis  the  general  results  of  any  infectious  disease  in 
characteristic  changes  in  the  liver,  spleen,  kidneys  and  other  organs 
are  present.     These  need  not  be  described  here. 

Symptoms.  —  The  disease  usually  begins,  like  any  acute  infectious 
disease,  with  fever,  sometimes  attended  by  convulsions  and  delirium, 
sometimes  by  considerable  pain  in  the  back,  body,  and  limbs ;  occa- 
sionally by  a  rigidity  of  the  neck  and  back  suggestive  of  acute  menin- 
gitis ;  and  by  digestive  disturbances,  vomiting,  and  diarrhoea ;  some- 

^Frissell.     Jour.  Amer.  Med.  Assoc,  July  11,  1911." 

^Flexner  and  Draper.     Jour.  Anier.  Med.  Assoc.,  January  13,  1912. 


SYMPTOMS.  269 

times  merely  by  general  malaise.  The  temperature  rises  rapidly  to 
102°  or  10^°  F.,  and  the  patient  may  have  a  chill  followed  by  sweat- 
ing. The  temperature  remains  about  101°  or  102°  for  several  days, 
with  slight  morning  remission,  then  gradually  sinks  to  normal,  the 
entire  febrile  movement  rarely  lasting  more  than  a  week.  Within 
two  or  three  days  of  the  onset  paralysis  sets  in.  This  may  be  in  both 
legs,  or  in  both  arms ;  rarely  in  one  limb  alone,  or  in  all  four  extrem- 
ities. If  the  child  is  young  and  is  confined  to  bed  by  the  fever, 
the  paralysis  may  not  be  noticed  until  the  third  or  fourth  day.  In 
older  children  and  adults  the  paralysis  is  well  developed  within  twenty- 
four  hours  of  the  onset.  It  is  observed  that  infants  cry  a  good  deal 
during  the  period  of  invasion,  and  those  who  are  old  enough  to  com- 
plain say  that  they  suffer  from  pain  in  the  back  and  in  the  affected 
limbs.  This  pain  may  remain  for  some  weeks.  There  is  usually  no 
disturbance  of  the  bladder  or  rectum,  although  in  a  few  cases  retention 
of  urine  has  been  noticed  for  a  few  days.  There  is  no  tendency  to  the 
development  of  bed-sores.  In  a  few  cases  herpes  zoster  has  appeared 
on  the  paralyzed  limb.  There  is  no  complaint  of  numbness  and  there 
is  never  any  loss  of  sensation,  but  the  limbs  are  sometimes  painful  upon 
any  movement,  especially  in  the  joints. 

In  some  cases  the  patient  is  so  sick  during  the  first  week  that  ty- 
phoid fever  is  suspected,  and  it  is  only  on  the  appearance  of  the  paral- 
ysis that  the  real  condition  is  recognized. 

After  such  an  onset  the  fever  and  general  symptoms  subside  rapidly, 
so  at  the  end  of  a  week  or  at  most  two  weeks  the  child  appears  to  be 
well. 

After  the  fever  with  its  attendant  malaise  and  digestive  disturbances 
has  subsided  and  the  general  health  has  been  restored,  there  remains 
a  paralysis  more  or  less  extensive.  This  paralysis  is  usually  more 
extensive  at  the  onset  than  it  is  destined  to  be  permanently.  Thus 
the  child  may  at  first  be  completely  helpless,  and  later  recover  power 
in  all  but  one  limb  ;  or  the  trunk  may  be  paralyzed  at  the  onset,  but 
not  permanently  affected.  Both  legs  are  commonly  affected  together, 
but  the  final  paralysis  is  usually  limited  to  one  limb.  Occasionally 
the  neck  muscles  are  distinctly  weak,  and  there  may  be  difficulty  in 
swallowing.  This  is  seen  in  cases  in  which  the  arms  are  paralyzed, 
and  yet  the  final  paralysis  may  affect  but  one  arm.  The  face  has 
been  paralyzed  with  the  arms,  and  the  ocular  muscles  also,  but  either 
is  a  rare  occurrence.  In  a  number  of  cases  in  which  the  final  paral- 
ysis has  been  limited  to  two  or  three  muscles  the  original  paralysis 
was  widespread,  involving  all  the  limbs.  These  facts  should  be  remem- 
bered in  giving  a  prognosis  in  the  early  stage.  Sometimes  the  onset 
of  the  paralysis  is  not  sudden,  but  there  is  a  gradual  increase  during 
a  week  or  ten  days,  then  a  stationary  period,  and  then  a  regression. 
The  subsidence  of  tlie  paralysis  begins  from  a  week  to  two  months 
after  tlie  onset,  and  then  goes  on  steadily,  but  it  is  not  until  after  three 
months  that  it  is  quite  possible  to  determine  what  muscles  will  eventu- 
ally recover.     There  is  usually  a  certain  amount  of  permanent  paralysis. 


270 


POLIOMYELITIS. 


The  muscles  which  are  paralyzed  undergo  atrophy.  This  is  more 
rapid  and  complete  in  those  that  are  to  be  permanently  paralyzed,  and 
there  is  a  change  in  the  size  of  the  limbs  which  is  well  marked  within 
a  month.  The  paralyzed  muscles  are  relaxed,  never  rigid,  and  show 
a  reaction  of  degeneration  to  the  electrical  tests.  The  reaction  of  de- 
generation consists  of  a  loss  of  the  response  in  both  muscle  and  nerve 
to  faradic  stimulus,  and  a  loss  of  response  in  the  nerve  to  galvanic 
stimulus.  The  galvanic  reaction  of  the  muscle  remains,  but  in  such  a 
muscle  there  is  found  an  alteration  of  its  normal  contractility  to  gal- 

FiG.  112. 


Extieme  atrophy  of  the  shoulder,  arm  and  forearm  in  an  adult  who  suflfered  from  an  attack  o 
infantile  paralysis  at  the  age  of  three. 


vanic  currents.  For  the  first  few  weeks  the  muscle  responds  too 
strongly  to  galvanism  and  contracts  under  the  positive  pole  more 
quickly  than  under  the  negative  pole  when  the  current  is  sent  through 
it.  Later  the  contractility  to  galvanism  is  progressively  decreased, 
until  in  a  totally  paralyzed  muscle  it  is  lost.  It  may  be  stated  as  a 
prognostic  sign  that  the  muscles  in  which  the  faradic  reaction  is  pre- 
served will  recover  though  paralyzed  for  a  time  at  the  onset.  Such 
muscles  also  preserve  their  tone,  so  that  they  contract  when  percussed 
sharply  with  a  hammer. 

The  circulation  in  the  aflPected  limb  is  considerably  impaired,  and 
it  is  cold,  blue  and  flabby,  but  not  oedematous.  In  some  cases  the 
bone  is  subsequently  hampered  in  its  growth,  so  that  the  limb  is 
shorter  and  more  slender  than  its  fellow  in  after  life.      (See  Fig.  112.) 

While  the  description  just  given  of  an  acute  onset  with  fever  applies 
to  about  three-quarters  of  the  cases  of  anterior  poliomyelitis,  there  re- 


SYMPTOMS. 


271 


mains  one-quarter  in  which  there  is  no  febrile  onset.     Of  166  cases  in 
my  clinic,  ^0  began  with  fever  and  46  began  without  fever.     Sink- 


riG.  113. 


Infantile  paralysis,  with  atrophy  and  impaired  growth  of  the  right  leg,  and  drop-foot;  four  years 

after  the  onset. 

lor  reported  178  with  fever,  40  without  fever.  Collins  and  Romeiser  ^ 
reported  336  cases  with  fever  and  29  without  fever.  In  these  cases  the 
child  while  in  a  state  of  perfect  liealth  is  suddenly  paralyzed  in  one  or 

'Jour,  Ainer,  Mod.  Aksoc,  Ma/ 30,  1908, 


272 


POLIOMYELITIS. 


more  limbs.  It  gives  no  sign  of  pain,  it  does  not  appear  to  be  ill,  and 
the  paralysis  surprises  the  mother  hy  its  sudden  onset.  In  these  cases 
the  paralysis  is  soon  followed  by  atrophy  and  by  vaso-motor  paralysis. 
It  is  not  attended  by  pain  or  tenderness  on  motion,  and  usually  de- 
creases to  some  extent,  leaving  the  limb,  however,  in  part  permanently 
paralyzed.  These  two  types  of  onset  of  the  disease  are  evidently  quite 
distinct  from  one  another. 

After  the  onset  is  over  there  is  a  slow  progressive  improvement  up 
to  a  certain  point,  and  then  the  permanent  condition  of  paralysis  is 
found  to  vary  greatly  in  different  cases. 

The  location  of  the  paralysis  is  usually  in  the  legs,  and  here  two 
types  of  the  disease  may  be  recognized  —  the  leg  type  and  the  thigh 
type.     In  the  leg  type  the  peronei  alone  or  with  the  anterior  tibial 

Fig.  114. 


Infantile  paralysis  and  atrophy  of  the  left  arm  two  years  after  onset.    The  partial  luxation  of  the 
humerus  is  evident ;  and  also  the  main  en  griffe. 

muscles  are  commonly  affected,  although  the  posterior  tibial  group  may 
share  in  the  paralysis  or  may  even  be  as  fully  paralyzed  as  the  others. 
As  the  paralysis  remains,  deformities  of  the  ankle  and  foot  will  appear, 
the  form  of  talipes  developed  depending  upon  the  muscles  chiefly  para- 


SYMPTOMS. 


273 


lyzed.  In  the  thigh  type,  the  psoas  and  iliacus  muscles  and  the  glutei 
and  musclegk  about  the  thigh  are  those  chiefly  aifected,  the  muscles  on 
the  inner  side  of  the  thigh  and  the  muscles  below  the  knee  often  escap- 
ing. In  these  cases  the  leg  hangs  like  a  flail  from  the  body,  and  can- 
not support  the  weight  at  all.  In  some  cases  nearly  all  of  the  muscles 
of  the  lower  extremity  are  paralyzed,  and  the  atrophy  is  uniform 
throughout  the  limb.  (Fig.  113.)  In  these  severe  cases  it  is  not 
uncommon  for  the  muscles  of  the  back  and  abdomen  to  share  in  the 
paralysis  and  atrophy. 

When  the  arms  are  attacked  two  types  of  paralysis  have  been  des- 
cribed, the  upper-arm  type  and  the  lower-arm  type.  In  the  upper-arm 
type  the  muscles  about  the  scapula  and  the  deltoid,  the  biceps,  and 
supinator  longus  are  paralyzed  and  atrophic,  and  consequently  the 
motions  of  the  shoulder  joint  and  elbow-joint  are  seriously  hampered. 
In  these  cases  the  shoulder-joint  is  unduly  movable  and  the  head  of 
the  humerus  falls  out  of  the  socket.  In  the  lower-arm  type  the  mus- 
cles below  the  elbow  are  invaded,  the  flexors  or  extensors  of  the  wrist 
and  fingers,  or  both  together,  are  affected,  the  supinator  longus  escap- 
ing. In  other  cases  the  interossei  and  the  thenar  and  hypothenar 
muscles  of  the  hand  are  paralyzed,  while  the  long  flexors  and  extensors 
escape.  Occasionally  a  combination  of  upper-arm  and  lower-arm  types 
occurs,  in  which  case  the  entire  extremity  is  useless.  (Fig.  114.) 
The  upper  part  of  the  trunk  is  occasionally  involved  in  the  paralysis 
together  with  the  arms.  The  muscles  of  the  back  and  trunk  are  rarely 
the  only  ones  permanently  paralyzed. 

In  a  very  few  cases  the  entire  muscular  system  of  the  body  appears 
to  be  affected  by  this  disease ;  both  legs,  the  trunk,  and  both  arms  and 
even  the  facial  muscles  are  more  or  less  paralyzed ;  but  even  in  these 
cases  a  careful  examination  will  show  that  the  degree  of  the  paralysis 
and  atrophy  is  not  the  same  in  all  the  muscles.  The  relative  frequency 
of  paralysis  in  different  parts  of  the  body  is  shown  in  the  following 
table  (Table  YII.).  The  paralysis  is  rarely,  if  ever,  exactly  sym- 
metrical when  both  legs  or  both  arms  are  involved. 


Table  VII.  —  The  Distribution  of  Permanent  Paralysis, 


Duchenne.^   Seeligmuller.^  SinJder. 


Both  legs. 

Kight  leg. 

Left  leg    . 

Right  arm 

Left  arm  . 

Both  arms 

All  extremities 

Arm  and  leg  same  side 

Arm  and  leg  opposite  sides 

Trunk       .       .       . 

Three  extremities . 


9 
25 
7 
5 
5 
2 
5 
11 
2 
1 


14 
15 
27 
9 
4 
1 
2 
2 
1 


107 

63 

62 

5 

8 

1 

35 

26 

1 

22 

10 


Starr. 

54 
31 
37 
11 

6 

5 

9 

9 

6 

4 

5 


Lovett.''' 

130 
216 
239 

5 

5 

0 

3 
15 

7 

6 

2 


Total 

314 

350 

372 

35 

28 

9 

54 
63 
17 
33 
17 


1  Archives  gdn.  de  Med.,  1864,  p.  38. 

^Gerhardt's  Handlmeh  d.  Kinderkrankheiten,  1880,  vol.  v.,  p.  1. 
'Keating's  Cyclop,  of  Children's  Diseases,  1890,  vol.  iv.,  p.  695, 
*Lovett.     Trans.  Amer,  Med.  Assoc,  1908,  p.  1680, 


18 


274  POLIOMYELITIS. 

Occasionally  the  cranial  nerve  nuclei  are  invaded  by  the  disease,  and 
as  a  result  strabismus  of  various  types,  facial  palsy,  paralysis  of  the 
larynx  and  tongue,  and  of  the  pharynx  have  been  observed. 

In  addition  to  the  paralysis  and  atrophy,  there  is  a  loss  of  reflex  ac- 
tion at  the  level  of  the  lesion  in  every  case.  The  skin  reflexes  usually 
return  after  a  time,  but  the  tendon  reflexes  are  absent  for  a  long  period, 
even  when  a  partial  recovery  of  the  muscle  involved  has  taken  place. 
Thus  the  knee-jerk  is  uniformly  absent  when  the  thigh  muscles  are 
paralyzed,  and  the  elbow-jerk  and  wrist-jerk  when  the  arms  are 
affected. 

Sensation  is  preserved  in  almost  every  case,  but  I  have  so  frequently 
observed  a  permanent  hypersensitive  condition  to  painful  impressions 
in  the  paralyzed  limb  that  I  cannot  but  believe  that  the  lesion  in  the 
gray  matter  aifects  the  pain-sense  tracts  in  their  passage  through  the 
cord  at  the  level  of  their  entrance,  and  has  a  relation  to  this  symptom. 
There  is  marked  vasomotor  paralysis  and  lack  of  vasomotor  response 
in  the  limb  to  applications  of  heat  and  cold. 

Deformities  of  the  joints  are  a  common  sequel  in  infantile  spinal 
paralysis.  The  approximation  of  articular  surfaces  is  secured,  in  part, 
by  the  normal  tension  of  the  muscles,  especially  at  the  shoulder,  hip, 
and  knee,  and  hence  paralysis  of  the  muscles  controlling  these  joints  is 
attended  by  relaxation  and  a  greater  degree  of  mobility  than  is  normal. 
Thus  the  head  of  the  humerus  falls  from  its  socket  when  the  deltoid  is 
paralyzed,  and  abnormal  extension  of  the  knee  is  often  seen  in  the 
upper-leg  type  of  paralysis.  After  some  months  of  paralysis  the 
muscles  which  are  the  natural  opponents  of  the  paralyzed  muscles  are 
apt  to  become  permanently  contractured,  and  this  also  occasions  defor- 
mities. The  action  of  gravitation  on  a  flaccid  part  of  the  limb  com- 
bines with  the  contracture  in  the  case  of  the  foot  to  increase  the 
deformity  there,  and  hence  all  forms  of  talipes  may  ensue  upon  infan- 
tile paralysis.  Deformities  of  the  wrist  are  also  observed,  but  these 
are  not  common.  Curvature  of  the  spine  from  paralysis  of  the  muscles 
of  the  back  is  frequently  seen,  all  varieties  having  been  described. 
Its  most  common  cause  is  the  shortness  of  one  leg,  due  to  arrest  of  its 
growth.  Such  curvatures  differ  from  those  due  to  bone  disease  in  the 
fact  that  they  do  not  persist  during  suspension  of  the  body  by  the 
head  and  arms.  It  is  one  of  the  most  important  points  in  treatment 
to  prevent  the  development  of  these  deformities. 

The  progress  of  the  disease  in  any  case  may  be  divided  into  stages. 
After  an  acute  onset  there  is  a  stage  of  maximum  intensity  lasting 
from  one  to  six  weeks,  and  followed  by  a  period  of  steady  improve- 
ment which  may  extend  from  six  months  to  a  year.  Then  follows  the 
permanent  chronic  condition,  in  which  the  normal  growth  of  the  child 
may  lead  to  a  slow  development  of  the  limb,  but  not  to  any  change  in 
its  power  of  use.  It  is  very  rare  for  a  complete  recovery  to  take 
place  after  an  attack  of  infantile  paralysis.  Even  in  the  lightest  cases 
there  is  usually  some  weakness,  slight  atrophy,  and  coldness  left,  and 
one  or  two  muscles  will  be  particularly  feeble.     In  the  majority  of 


SYMPTOMS. 


275 


cases  considerable  permanent  paralysis  remains,  requiring  the  use  of 
apparatus  to  assist  the  action  of  the  limb  and  to  prevent  deformities. 
Death  occurs  during  the  acute  onset,  in  about  7  per  cent,  of  the  cases 


Fig.  115. 


Infantile  paralysis  with  atrophy  of  the  right  leg.    The  curvature  of  the  spine  is  secondary  to  the 

shortening  of  the  leg. 

in  epidemics  of  the  disease,  but  in  sporadic  cases  is  very  rare,  and  once 
this  stage  is  passed  there  is  nothing  in  the  disease  to  threaten  life. 

It  is  the  chief  characteristic  of  the  atrophic  paralysis  in  this  disease 
that  it  selects  certain  muscles  to  the  exclusion  of  others.  This  selec- 
tion bears  no  relation  to  the  arrangement  of  muscles  in  the  limb  or  to 
the  conjoint  action  of  muscles  in  producing  any  definite  movement. 
It  is  wholly  dependent  upon  the  arrangement  of  the  groups  of  cells 
controlling  the  muscles  in  the  anterior  horns  of  the  spinal  cord,     Jh^ 


276  POLIOMYELITIS. 

exact  localization  of  the  disease  can  be  determined  by  referring  the 
symptoms  in  any  case  to  Table  I.  in  the  chapter  on  the  Diagnosis  of 
Spinal-cord  Diseases,  page  56. 

Features  of  Epidemics.  —  When  the  disease  occurs  in  epidemics  the 
symptoms  are  similar  to  those  occurring  in  the  infectious  type  of 
sporadic  cases,  there  being  a  high  fever,  104°,  with  chill,  vomiting, 
malaise,  severe  general  sweating,  severe  pains  in  the  back  and  limbs, 
sometimes  attended  by  rigidity  of  the  spine  and  retraction  of  the  head, 
giving  rise  to  the  suspicion  of  meningitis.  Tlie  excessive  sweating 
implies  an  involvement  of  the  vasomotor  centres  in  the  spinal  cord. 
Diarrhoea  occurs  on  the  second  day  and  continues  for  two  or  three  days. 
Delirium  is  a  frequent  symptom,  occasionally  convulsions,  and  aphasia 
have  been  noted.  The  febrile  symptoms  last  for  five  or  ten  days.  On 
the  third  or  fourth  day  the  paralysis  appears,  usually  suddenly  and  at 
its  maximum  extent.  When  the  legs  are  paralyzed  it  is  not  unusual 
to  have  retention  of  urine  and  loss  of  control  of  the  bladder  lasting 
several  days.  When  the  arms  are  affected  the  respiratory  muscles  may 
be  paralyzed  and  in  the  fatal  cases  death  occurs  from  respiratory  paral- 
ysis. Sometimes  the  paralysis  extends  to  the  neck,  throat  and  face, 
and  even  to  the  eyes,  giving  rise  to  the  symptoms  of  bulbar  palsy,  the 
polio-encephalitis  inferior  and  superior  of  Wernicke.  If  the  face  is 
affected  all  the  muscles  are  involved  and  the  patient  cannot  close  the 
eye.  If  the  ocular  muscles  are  paralyzed  strabismus  appears.  Pain 
is  a  more  prominent  symptom  in  the  epidemic  than  in  the  sporadic  cases. 
The  variation  in  the  onset  and  in  the  distribution  of  the  paralysis  have 
led  writers  to  distinguish  between  spinal,  bulbar,  cerebral,  neuritic, 
meningitic,  and  abortive  forms  of  the  disease.  These  forms  differ  some- 
what in  the  fact  that  in  each  one  set  of  symptoms  predominates.  They 
are  rarely  without  common  symptoms  and  do  not  require  separate 
description. 

In  epidemics  the  mortality  has  been  from  7  to  10  per  cent. ;  death 
occurring  from  the  fourth  to  the  tenth  day,  usually  on  the  sixth  day. 
In  sporadic  cases  death  is  very  rare. 

In  epidemics  abortive  cases  have  been  recognized,  where  the  fever 
has  produced  great  muscular  weakness,  but  has  not  been  followed  by 
permanent  paralysis,  complete  recovery  having  ensued  after  two  or 
three  weeks.  In  epidemics  there  is  complete  recovery  in  25  per  cent, 
of  the  cases.  Various  cases  of  slight  indefinite  temporary  illness  with 
prostration  but  no  paralysis,  much  like  the  prostration  of  gripj)e,  have 
been  observed  in  families  and  communities  where  this  disease  was  epi- 
demic. These  are  probably  cases  of  very  mild  type  and  the  diagnosis 
can  be  made  only  by  an  examination  of  the  cerebrospinal  fluid  or  by 
an  experimental  inoculation  of  the  blood  already  described  on  page  262. 
The  cases  are  so  insignificant  that  neither  procedure  is  as  a  rule  justi- 
fiable, as  lumbar  puncture  is  not  without  danger. 

Diagnosis.  —  There  is  no  difficulty  in  recognizing  the  disease,  and 
it  is  hardly  likely  to  be  mistaken  for  anything  else.  Occasionally 
a  child  will  be  attacked  with  acute  articular  rheumatism,  and,  on 


SYMPTOMS.  277 

account  of  the  pain  in  the  joints,  will  be  unwilling  to  move  the  limbs, 
and  thus  ipay  be  thought  to  be  paralyzed.  A  careful  examination 
should  soon  demonstrate  tlie  real  condition,  for  acute  rheumatism 
never  causes  any  atrophy  or  paralysis,  and  the  local  tenderness  in  the 
joints,  the  sweating,  and  the  lack  of  coldness  of  the  limbs  may  also 
aid  in  the  diagnosis. 

Rachitis,  sometimes  caused  in  infants  living  in  healthful  and  com- 
fortable surroundings  by  the  use  of  artificial  patent  foods  containing 
considerable  sugar,  may  lead  to  a  sudden  febrile  onset,  with  much  pain 
and  tenderness  in  the  limbs  and  unwillingness  to  move.  But  the  child 
is  not  really  paralyzed,  and  the  tenderness  of  its  bones,  the  appearance 
of  the  gums  and  the  sweating,  as  well  as  the  lack  of  limitation  of  the 
pain  and  immobility  to  one  or  two  limbs,  should  prevent  this  disease 
from  being  mistaken  for  infantile  paralysis. 

In  some  cases  of  anterior  poliomyelitis  there  is  considerable  pain 
felt  in  the  limbs,  and  some  tenderness  of  the  surface  and  of  the  mus- 
cles. The  existence  of  pain  during  the  first  two  days  of  the  disease 
occasionally  leads  to  mistakes  in  diagnosis.  Thus  Marsh  ^  records  a 
case  of  a  child  aged  five  years,  who  was  suddenly  attacked  with  pain 
in  the  left  leg  extending  down  the  thigh  to  the  knee.  The  limb  was 
flexed,  abducted,  and  rotated  outward,  and  any  motion  was  painful ; 
hence  the  case  was  recorded  as  acute  hip  disease,  but  closer  examina- 
tion showed  the  hip-joint  to  be  quite  freely  movable,  and  after  two 
days,  when  the  pain  had  passed  away,  the  case  was  found  to  be  one 
of  infantile  paralysis.  The  fever  and  general  constitutional  distur- 
bances present  at  the  onset  had  rendered  the  diagnosis  obscure. 

In  painful  cases  it  has  been  suggested  that  a  neuritis  may  accom- 
pany the  poliomyelitis.  The  existence  of  pain  alone  is  not  sufficient 
to  warrant  this  conclusion,  inasmuch  as  we  now  know  that  in  the  early 
stages  there  is  a  congestion  of  the  meninges  and  central  gray  matter  of 
the  cord  which  is  sufficient  to  explain  the  pain.  If,  however,  the  pain 
continues  and  tenderness  develops  in  the  muscles  and  nerves,  it  is 
probable  that  a  neuritis  has  developed  due  to  the  same  infectious  agent 
which  has  caused  the  poliomyelitis.  It  is  to  be  remembered  that  poly- 
neuritis is  usually  a  disease  affecting  the  extremities  symmetrically  and 
causing  drop-wrist  and  drop-foot ;  that  the  distal  parts  of  the  extrem- 
ities are  more  severely  paralyzed  than  the  proximal  parts  ;  that  there 
is  no  such  selection  of  muscles  paralyzed  as  in  poliomyelitis,  and  that 
there  are  usually  sensory  disturbances  of  a  permanent  nature,  anaes- 
thesia and  analgesia,  or  ataxia,  in  addition  to  the  pain  and  tenderness 
along  the  nei-ves ;  hence  in  the  acute  stage  of  onset  a  polyneuritis 
should  not  be  confounded  with  a  poliomyelitis.  When  polyneuritis 
accompanies  poliomyelitis  the  clinical  picture  will  be  made  up  of  a 
combination  of  the  symptoms  of  both  affections  (see  page  205). 

A  condition  of  weakness  of  the  muscles  observed  in  infancy  has 
been  described  by  Oppcnheim  and  by  Spiller,  which  they  term  mya- 
tonia  conr/enita    and    which    resembles    slightly    infantile    paralysis. 

^Lancet,  January  16,  1897. 


278  ■  POLIOMYELITIS. 

Twenty-six  cases  have  been  collected  by  Haberman.^  The  infant 
makes  very  few  movements  from  the  time  of  birth,  all  the  muscles 
seemiug  to  be  weak  and  lacking  in  tone.  There  is  no  atrophy,  but 
there  are  no  deep  reflexes  to  be  obtained.  The  muscles  react  to  strong 
electrical  currents.  The  chief  symptom  is  the  lack  of  voluntary  move- 
ment ;  the  infants  lie  inert  and  lielpless,  though  slight  motions  are 
made  in  response  to  painful  impressions.  The  children  are  intelligent, 
'the  cranial  nerves  are  normal,  sensation  and  the  action  of  the  sphincters 
are  normal.  Slowly,  after  many  months,  improvement  begins,  the 
muscles  develop  and  power  is  gained.  In  the  fatal  cases  no  lesion  of 
the  nervous  system  has  been  found,  but  there  is  an  atony  of  the  muscles, 
which  appear  pale  and  poorly  developed.  As  congenital  poliomyelitis 
has  not  been  observed,  this  disease  need  not  be  confounded  with  in- 
fantile paralysis.  It  has  a  relation  to  myasthenia  gravis  and  is  prob- 
ably due  to  some  congenital  disease  of  the  thymus  gland.  (Chapter 
XLIV.) 

A  localized  injury  of  the  brachial  plexus  (Erb's  paralysis),  causing 
paralysis  of  the  deltoid,  biceps,  coracobrachialis  and  supinator  longus 
is  not  uncommon  in  infants,  and  might  be  mistaken  for  infantile  palsy. 
The  history  of  trauma  during  delivery  and  the  local  anaesthesia  in  the 
distribution  of  the  circumflex  nerve  should,  however,  correct  the  mistake 
(see  page  180). 

These  possibilities  being  excluded,  and  the  diagnosis  of  a  poliomye- 
litis being  reached  from  a  study  of  the  symptoms,  it  is  now  possible  by 
examination  of  the  spinal  fluid  obtained  by  lumbar  puncture  to  make 
such  a  diagnosis  positive.  But  further  it  is  also  possible  by  such  an 
examination  to  reach  a  diagnosis  on  the  second  or  third  day  of  the  dis- 
ease when  the  child  shows  merely  an  acute  fever  without  any  paralysis. 
At  this  time  the  blood  examination  shows  only  a  moderate  increase  in 
leucocytes  such  as  occurs  in  any  infection.  But  Flexner  has  proven 
that  even  in  this  early  stage  the  amount  of  cerebrospinal  fluid  is  in- 
creased so  that  on  puncture  it  flows  more  freely  than  normal.  It  is 
slightly  opalescent  and  on  centrifugalization  the  sediment' contains  a 
considerable  number  of  small  and  large  lymphocytes  and  a  few  poly- 
nuclear  leucocytes.  The  fluid  also  gives  a  protein  reaction  to  the* 
butyric  acid  test.  Such  tests  can  of  course  be  made  only  by  those  who 
have  laboratory  facilities. 

Prognosis. —  The  prognosis  in  anterior  poliomyelitis  is  always  grave. 
Patients  do  not  often  die  of  the  affection,  but  they  rarely  escape  a  per- 
manent paralysis  in  some  part  of  the  body.  It  is  true  that  in  the 
majority  of  cases  the  original  paralysis  subsides,  so  that  there  is  an 
apparent  improvement  of  a  considerable  degree.  Thus  a  patient  who 
has  originally  been  paralyzed  in  both  legs  may  recover  the  power  in 
one  leg  entirely,  and  may  be  left  with  a  condition  of  paralysis  in  the 
peronei  or  in  the  anterior  tibial  group  of  the  other  leg,  so  that  the 
terminal  condition  is  very  much  less  severe   than  that  at  the  onset. 

^  Zur  DifEerentialdiagnose  der  poliomyelitis  anterior  acuta,  myatonia  congenita  und 
polyneuritis.     Berlin,  S.  Karger,  1908. 


TMAtmenT.  279 

As  a  rule,  the  limb  that  is  affected  never  entirely  regains  its  power, 
and  usually  shows  some  atrophy  and  shortening ;  for  the  growth  of 
the  limbs  i^  hampered  by  the  existence  of  the  disease,  and  hence  in  a 
growing  child  the  unaffected  limb  outgrows  the  other.  It  is  thought 
that  an  electrical  examination  may  afford  some  ground  for  a  prognosis. 
It  is  believed  that  the  muscles  which  respond  to  the  faradic  current 
three  weeks  after  the  onset  of  the  disease  will  eventually  recover, 
while  those  that  fail  to  respond  to  this  current  at  that  time  will  always 
be  somewhat  impaired  in  power.  The  loss  of  faradic  reaction,  how- 
ever, is  not  an  indication  that  these  muscles  will  be  totally  paralyzed, 
since-  the  faradic  reaction  has  been  known  to  return  in  a  muscle  a  year 
after  it  has  been  lost,  yet  such  a  muscle  never  recovers  completely  its 
size  or  power.  The  prognosis  is  much  better  in  the  cases  which  begin 
with  fever  than  in  those  which  do  not,  and  in  the  epidemic  cases  after 
the  second  week  of  the  disease  than  in  the  sporadic  cases.  In  epi- 
demics, as  already  stated,  from  7  to  10  per  cent,  are  fatal  on  the 
sixth  day. 

Treatment.  —  The  treatment  of  infantile  spinal  paralysis  in  the 
acute  stage  consists  in  isolating  and  keeping  the  child  quiet  in  bed  and 
applying  a  mild  form  of  counter-irritation  along  the  spine,  which  is 
best  done  by  a  paste  of  mustard  1  part  and  flour  3  parts,  applied  in  a 
poultice  along  the  back  and  removed  as  soon  as  the  skin  is  reddened, 
and  then  renewed  after  three  hours,  so  that  for  at  least  a  week  there 
shall  be  continued  counter-irritation  without  the  discomfort  of  a  blister. 
The  frequent  application  of  dry  cups  along  the  spine  may  be  used  to 
produce  the  same  effect.  Repeated  sponging  with  alcohol  and  cold 
water  is  indicated  in  the  cases  in  which  the  temperature  is  above  101  °  F., 
but  phenacetin  or  antipyrine  is  not  to  be  used  unless  the  temperature 
reaches  103°  F.  Dr.  Harvey  Gushing  has  recently  observed  that  the 
administration  of  urotropin  results  in  the  presence  of  formaldehyde  in 
the  cerebrospinal  fluid.  As  the  virus  of  the  disease  exists  in  the 
cerebrospinal  fluid,  it  is  well  to  give  a  child  of  three  years  one  grain, 
of  urotropin  every  six  hours  during  the  first  ten  days.  Iodide  of  potas- 
sium may  be  given  in  1 -grain  dose  in  the  early  stage,  and  moderate 
doses  of  salicylate  of  strontium  (2  gr.)  or  of  quinine  (^  gr.)  may  be 
used  for  a  child  of  two  years.  If  the  child  is  in  much  pain  or  has  con- 
vulsions, bromide  of  sodium  (5  gr.)  with  or  without  codeine  ( J^  gr.), 
may  be  employed  as  a  symptomatic  remedy.  The  general  treatment  of 
febrile  conditions,  a  light  diet  and  laxatives,  is  not  to  be  neglected.  In 
the  infectious  cases  some  gastro-intestinal  poison  may  be  present,  hence 
free  purgation  has  been  urged.  The  best  laxative  is  castor  oil  Sss, 
glycerme  5ij,  cinnamon-water  TTLx,  given  with  an  equal  amount  of 
lemon  juice  sweetened.  Rest  in  a  prone  position  in  bed  is  better  than 
constant  lying  upon  the  back. 

The  isolation  of  the  patient  should  be  as  complete  as  in  scarlet  fever. 
The  nose  and  throat  sliould  be  sprayed  with  Dobell's  solution  every 
few  hours  to  lessen  the  chance  of  contagion  to  others,  and  this  should 
be  continued  for  several  week§  after  the  fever  has  subsided. 


280  POLIOMYELITIS. 

When  the  acute  stage  is  passed  there  is  little  to  be  done  during  the 
second  week  excepting  to  nourish  the  child  well  and  to  keep  the  para- 
lyzed limb  warm. 

"When  the  paralysis  begius  to  subside  spontaneously  it  is  well  to 
administer  strychnine  in  full  dose,  -^  gr.,  three  times  a  day  for  a  child 
of  three  years  of  age.  This  remedy  is  best  given  at  intervals,  and  not 
continuously,  and  it  is  my  rule  to  use  it  for  one  week,  and  then  to 
intermit  for  three  days.  The  condition  of  mechanical  irritability  in 
unparalyzed  muscles,  as  determined  by  percussion  with  a  hammer,  is  a 
good  indication  of  the  degree  of  effect  being  produced  by  the  strych- 
nine, and  the  drug  may  be  increased  until  it  causes  a  distinct  iucrease 
in  this  irritability.  It  is  to  be  remembered,  however,  that  twitcliing 
of  the  limbs  or  stiffness  of  the  back,  usually  indicative  of  an  effect  of 
strychnine,  is  not  to  be  relied  upon  in  infantile  paralysis  when  the 
muscles  are  paralyzed.  Whether  general  tonics,  such  as  cod-liver  oil, 
hypophosphites,  or  arsenic,  have  any  effect  of  a  favorable  kind  may 
be  left  to  the  judgment  of  the  physician  in  each  individual  case. 

The  most  important  indication  during  the  stage  of  regression  is  to 
preserve  the  nutrition  and  function  of  the  paralyzed  muscles,  and  this 
is  to  be  attained  by  skilful  massage,  by  hydrotherapy,  or  by  the  use 
of  electricity.  Massage  is  of  the  utmost  importance  in  these  cases,  and 
should  be  given  once  or  twice  a  day  with  care,  combined  with  such 
attempts  at  active  movement  as  the  child  is  able  to  make.  Among 
the  poorer  classes  it  is  well  to  instruct  the  mother  how  to  give  this,  so 
that  it  may  be  given  with  persistence.  The  massage  should  not  be  of 
the  hardest  kind,  and  yet  should  be  sufficient  to  stimulate  the  circu- 
lation in  the  limbs  and  to  promote  the  lymphatic  and  venous  flow. 
Next  to  massage  mechanical  devices  which  induce  the  child  to  make 
use  of  the  weakened  limb  are  to  be  employed.  A  household  gym- 
nasium adapted  to  each  individual  case  can  easily  be  devised  by  the 
physician,  and  if  such  exercises  are  made  of  the  nature  of  play  to  the 
child's  imagination,  much  good  will  be  derived  from  its  own. efforts. 

Hydrotherapy  is  also  an  important  aid  in  treatment.  The  general 
circulation  in  the  cold  and  flabby  limb  may  be  aided  by  warm  baths, 
and  it  is  my  rule  to  have  these  children  play  in  warm  water,  tempera- 
ture 99°  F.,  for  half  an  hour  twice  daily.  This  warm  bath  may  be 
followed  by  a  cooler  sponging  and  brisk  rubbing,  but  cold  water 
should  not  be  employed  in  the  bathing  of  these  children,  as  the  tem- 
perature of  the  paralyzed  limb  is  always  below  that  of  health,  and 
the  vasomotor  paralysis  prevents  the  quick  reaction  which  is  so  bene- 
ficial in  other  conditions.  Proper  protection  of  these  limbs  by  extra 
flannel  clothing  is  always  advisable. 

Electricity  is  a  valuable  agent  in  the  treatment  of  infantile  paralysis, 
but  a  clear  statement  of  its  use  should  be  made  by  the  physician  to 
the  family.  Electricity  has  no  influence  whatever  upon  the  course  of 
the  disease.  It  does  not  affect  the  lesion  in  the  spinal  cord,  either  to 
decrease  the  hypersemia  or  to  increase  the  nutrition  of  the  nerve  cen- 
tres.   Applications,  therefore,  of  galvanism  to  the  spine  are  absolutely 


TBEATMENT.  281 

useless.  But  applications  to  the  muscles  may  be  of  distinct  service  in 
two  diifer^t  ways  —  first,  by  causing  their  contraction,  and  thus  exer- 
cising them  when  voluntary  exercise  is  impossible,  and,  secondly, 
by  promoting  the  chemical  changes  in  the  muscle  that  are  essential  to 
growth  and  nutrition. 

Examination  in  any  case  will  show  a  certain  number  of  muscles  in 
the  paralyzed  limb  that  respond  to  faradism.  These  muscles  will 
eventually  recover  entirely,  yet  the  tone  of  the  muscle  and  its  strength 
can  be  kept  up  during  the  period  of  improvement  by  means  of  exer- 
cise with  either  the  faradic  or  galvanic  current.  It  is  quite  well 
proven  that  just  as  exercise  of  a  healthy  arm  will  markedly  increase 
the  size  of  the  biceps  muscle,  so  applications  of  faradism  regularly  to 
a  muscle  that  it  will  contract  will  increase  the  size  of  this  muscle ; 
hence  to  the  weakened  muscles  which  still  respond  to  faradism  an  ap- 
plication of  the  faradic  current  for  about  ten  minutes  once  or  twice  a 
day  will  be  of  service.  The  majority  of  the  paralyzed  muscles  do  not 
respond,  however,  to  faradism,  and  it  is  time  wasted  to  apply  the 
faradic  current  to  these  muscles.  They  do  respond,  as  a  rule,  to  gal- 
vanic interrupted  currents,  the  positive  pole  being  placed  over  the 
muscle  and  the  negative  upon  the  limb  at  a  short  distance  above.  The 
interruptions  should  be  made  by  an  electrode  held  in  the  hand  and 
provided  with  a  finger-key,  and  each  muscle  should  be  treated  for 
about  three  minutes  daily,  fifty  to  sixty  interruptions  being  made  per 
minute  by  the  finger.  The  strength  used  should  be  the  least  which 
will  secure  contraction  in  the  muscle.  When  interruptions  of  the  cur- 
rent do  not  produce  a  prompt  response  alternation  of  the  current  may 
be  employed  by  reversing  the  current  rapidly  by  means  of  the  pole- 
changer  on  the  battery.  It  is  to  be  remembered  that  in  this  disease 
the  application  of  electricity  is  more  painful  than  in  health.  It  is 
also  to  be  remembered  in  applying  electricity  to  children  that  their 
confidence  must  be  gained,  and  that,  if  they  are  frightened  at  the  first 
application,  subsequent  treatment  will  result  in  a  continual  struggle. 
It  is  my  custom,  therefore,  to  begin  a  course  of  electrical  treatment  to 
a  child  by  several  applications  of  the  sponges  and  electrodes  while  no 
current  is  passing,  thus  accustoming  the  child  to  the  apparatus  and 
gaining  its  confidence.  After  two  or  three  such  applications  it  will  be 
possible  to  use  a  weak  current,  and  then  day  by  day  to  increase  its 
strength  until  by  the  end  of  ten  days  the  necessary  strength  is  being 
used.  In  this  way  a  daily  struggle,  with  the  result  of  unsatisfactory 
and  probably  useless  applications,  can  be  avoided ;  and  the  parents' 
consent  obtained  to  a  course  of  treatment  which  they  would  eventually 
object  to  if  every  application  resulted  in  a  struggle.  Any  intelligent 
mother  or  nurse  can  be  taught  to  give  the  galvanism  or  faradism  to  a 
child  in  this  manner,  and  it  is  best  to  interest  the  attendant  in  the 
treatment  from  the  beginning,  and  to  instruct  her  carefully,  so  that 
within  a  week  the  treatment  can  be  left  entirely  in  her  hands.  Such 
an  application  of  electricity  is  to  be  made  daily  or  twice  a  day  for  two 
or  three  years.     Spontaneous  recovery  will  have  been  reached  at  the 


2S2  POLIOMYELITIS. 

end  of  the  first  year,  but  even  after  this  time  these  muscles  may  be 
brought  into  a  condition  of  hypertrophy  by  means  of  continued  exer- 
cise. When,  however,  a  child  is  quite  able  to  move  voluntarily  with 
some  force  any  paralyzed  muscle,  it  is  far  better  to  rely  upon  voluntary 
exercises  than  upon  electrical  applications.  If  no  eifect  is  obtained 
from  massage,  bathing,  and  electricity  in  a  muscle  at  the  end  of  a  year 
there  is  no  use  in  continuing  the  treatment  of  that  muscle,  as  it  will 
never  recover,  its  nerve  cells  being  entirely  destroyed. 

The  use  of  braces  plays  a  great  part  in  the  treatment  of  infantile 
paralysis  in  the  chronic  stage.  It  is  necessary  to  prevent  stretching 
of  all  muscles,  those  which  are  slightly  or  not  at  all  paralyzed  as  well 
as  those  which  are  paralyzed.  Hence  an  eifort  must  be  made  to  hold 
the  joints  in  their  normal  positions  from  the  earliest  stage  of  the 
disease.  It  is  to  be  remembered  that  many  weak  muscles  can  do 
their  work  only  when  the  limb  is  placed  in  an  advantageous  position 
or  when  they  are  assisted  in  their  action.  Many  of  the  muscles 
have,  as  part  of  their  function,  to  keep  the  joints  in  place,  and  this 
part  can  be  supplied  by  properly  adjusted  braces ;  hence  an  appa- 
ratus may  enable  the  child  to  use  a  muscle  or  to  move  a  joint  that 
it  could  not  do  if  the  joint  were  unsupported.  Again,  the  result  of 
paralysis  of  one  group  of  muscles  is  to  allow  the  joint  to  be  bent 
by  its  opponent  or  to  yield  to  the  influence  of  gravitation,  and  hence 
the  paralysis  is  often  followed  by  deformity  if  a  brace  is  not  applied 
early  to  correct  this  tendency.  There  is  no  disease  in  which  ortho- 
pedic apparatus  is  of  more  service  than  in  infantile  paralysis,  and  it 
cannot  be  applied  too  early,  as  it  may  prevent  the  development  of  con- 
tractures and  of  deformities.  There  is  no  stage  in  which  it  is  too  late 
to  fit  a  brace,  for  even  if  these  deformities  have  occurred  tenotomy 
may  be  employed  to  straighten  and  adjust  a  joint,  and  then  the  limb 
can  be  fixed  by  the  brace  in  a  proper  position.  But  every  case  has  to 
be  treated  skilfully  in  accordance  with  its  own  condition,  and  the 
ready-made  braces  of  the  shops  are  often  worse  than  none.  Hence 
for  each  case  a  special  apparatus  must  be  fitted  under  the  direction  of 
an  orthopedic  surgeon,  and  it  is  to  be  remembered  that  in  a  growing 
child  such  apparatus  must  be  constantly  readjusted,  its  length  and  size 
being  changed  from  month  to  month  in  accordance  with  the  develop- 
ment of  the  limb. 

In  many  cases  of  deformity  where  there  is  a  strong  contracture  of  a 
fairly  healthy  muscle  overcoming  the  weak  paralyzed  muscle  the  ques- 
tion of  tenotomy  will  arise.  Such  tenotomy  will  of  course  result'  in  a 
temporary  replacement  of  the  deformed  joint  to  its  natural  position, 
but  unless  the  joint  can  be  held  by  a  brace  in  this  position,  tenotomy 
alone  will  be  of  no  permanent  service.  Hence  tenotomy  is  only  to  be 
regarded  as  a  preliminary  in  some  cases  to  the  proper  application  of 
apparatus.  Apparatus  has  also  been  devised  (especially  in  the  treat- 
ment of  infantile  paralysis  of  the  hands)  by  means  of  which  weakened 
muscles  may  be  reinforced  by  elastic  bands  so  applied  as  to  take  the 
place  of  the  paralyzed  muscle.     Thus  a  dropped-wrist  or  a  paralysis 


TBEATMENT.  ■  283 

of  the  extensors  of  one  side  of  the  wrist  can  be  somewhat  relieved  by 
a  series  ofi"  elastic  bands  attached  to  finger  tips  or  to  rings  and  to  the 
elbow  and  running  through  a  bracelet  at  the  wrist.  Dropped-foot  may 
also  be  similarly  remedied.  Such  devices,  however,  are  usually  dis- 
carded after  a  time,  as  they  are  more  cumbersome  than  useful.  Ap- 
paratus is  especially  applicable  to  spinal  curvature  of  the  paralytic 
type,  and  in  any  case  in  which  the  body  or  back  muscles  are  involved 
at  the  onset  it  is  well  for  the  child  to  wear  a  corset  in  order  to  pre- 
vent the  development  of  some  form  of  curvature.  A  thick  cork  sole 
will  prevent  the  curvature  due  to  a  short  leg. 

It  has  been  proposed  to  divide  longitudinally  the  tendon  of  certain 
healthy  muscles  and  attach  one-half  to  the  severed  tendon  of  a  par- 
alyzed muscle  about  the  knee,  ankle,  wrist,  and  elbow,  in  order  that 
the  healthy  muscle  may  be  made  to  do  the  work  of  the  muscle  which  is 
paralyzed,  and  many  successful  attempts  in  this  direction  have  been 
reported.  I  have  seen  permanent  benefit  in  a  remarkable  degree,  in  a 
case  of  paralysis  of  the  peronei,  by  this  method  of  treatment,  in  which 
a  part  of  the  posterior  tibial  tendon  was  attached  to  the  cut  peroneus 
longus  tendon.  I  have  also  seen  paralysis  of  the  anterior  tibial  muscle 
relieved  by  attaching  its  tendon  to  the  tendon  of  the  long  peroneal 
muscle.  I  have  seen  paralysis  of  the  soleus  improved  by  attaching  its 
tendon  to  the  deep  muscles  beneath  it,  and  I  have  seen  several  cases 
of  great  improvement  in  movements  of  the  fingers  and  wrists  by  at- 
taching the  tendons  of  paralyzed  muscles  to  those  which  were  healthy. 
This  is  a  method  of  relief  which  is  rational  and  deserves  wide  adoption. 
Periosteal  implantation  rather  than  tendon  joining  is  preferred  by  some 
surgeons. 

It  has  also  been  proposed  to  divide  the  nerve  going  to  a  paralyzed 
muscle  and  to  graft  the  peripheral  end  on  to  a  normal  nerve  trunk 
adjacent  to  it,  in  the  hope  that  the  nerve  may  regenerate  and  that  the 
muscle  will  receive  impulses  and  nutrition  through  the  new  nerve. 
This  method  is  still  under  observation,  though  some  success  has  been 
reported.  It  deserves  a  trial  in  cases  where  tendon  grafting  is  impos- 
sible. The  effect  of  the  division  of  the  healthy  nerve  is  always  to  be 
borne  in  mind.  And  if  the  manifest  result  of  the  possible  failure  of 
the  operation  will  be  to  render  the  patient  much  more  helpless  than 
before  it,  this  operation  is  not  to  be  advised.  Lovett  has  collected 
twenty  cases,  sixteen  of  which  were  successful. 


CHAPTER  XV. 

CHEONIC  ATEOPHIC  PAEALYSIS. 

Progressive  Muscular  Atrophy.     Amyotrophic  Lateral  Sclerosis. 

History.  —  A  condition  of  progressive  paralysis  with  atrophy  was 
first  described  by  Sir  Charles  Bell  in  1836  ;  it  was  not  referred  to  a 
lesion  of  the  spinal  cord,  however,  until  1850,  when  Aran^  studied  it 
carefully.  His  statements  were  added  to  by  Duchenne  in  1853.  But 
the  form  of  progressive  atrophic  paralysis,  termed  progressive  muscular 
atrophy  of  Aran-Duchenne,  was  soon  found  to  be  but  one  of  several 
types  of  the  disease.  Duchenne  in  1853*  described  another  type, 
which  he  named  paralysie  g6nSrale  anUrieure  subdigue  ascendante,  a 
disease  beginning  with  paralysis  and  atrophy  in  the  legs,  and  gradually 
invading  the  trunk  and  the  arms,  and  finally  causing  death  from  re- 
spiratory paralysis.  For  many  years  all  cases  of  chronic  atrophic 
paralysis  were  referred  to  these  two  classes,  and  were  supposed  to  have 
as  a  basis  an  affection  of  the  anterior  gray  matter  of  the  cord. 

But  between  1860  and  1870  numerous  cases  of  atrophic  paralysis 
were  reported  in  which  no  spinal  lesion  was  to  be  found.  And  grad- 
ually it  became  evident  that  some  forms  of  this  disease  were  dependent 
entirely  upon  changes  in  the  muscles.  To  Friederich  ^  and  the  German 
school  must  be  given  the  credit  of  separating  the  muscular  dystrophies 
from  the  forms  of  spinal  paralysis. 

In  1872  Charcot^  and  the  French  school  discovered  amyotrophic 
lateral  sclerosis  and  showed  the  difference  between  it  and  progressive 
muscular  atrophy.  And  finally  in  1882  Dejerine  and  others  brought 
forward  many  facts  to  prove  that  numerous  cases  formerly  supposed 
to  be  due  to  spinal  lesions  were  really  due  to  multiple  neuritis  (see 
page  212). 

A  chronic  atrophic  paralysis  characterized  by  a  slowly  progressing 
weakness,  and  atrophy  beginning  in  one  part  of  the  body  and  advanc- 
ing to  another  part,  may  occur  under  the  following  conditions : 

First :  Chronic  anterior  poliomyelitis. 

Second  :  Amyotrophic  lateral  sclerosis,-  (a)  beginning  in  the  spinal 

'  Tubby.     Lancet,  March  28,  1903.     Assoc,  franc,  de  Chirurgie,  1907,  p.  380-574. 

^Lovett  and  Lucas.     Jour.  Amer.  Med.  Assoc,  1908,  Nov.  14. 

*Arch.  gen.  de  Mdd.,  xxiv.,  42. 

*Traite  de  I'Electrotherapie  localis^e. 

*  Ueber  progressive  Muskelatrophie.     Berlin,  1873. 

^Lepons  sur  les  maladies  du  systemenerveux.     Paris,  1880,  ii.,  192. 

284 


CEBONIC   POLIOMYELITIS.  285 

cord  and  ascending  to  the  medulla  and  pons ;  (6)  beginning  in  the 
medulla  and  pons  as  bulbar  paralysis,  and  descending  to  the  spinal 
cord. 

Third  :  Multiple  neuritis. 

Fourth  :  Muscular  dystrophy. 

In  the  present  chapter  attention  will  be  directed  to  chronic  anterior 
poliomyelitis,  and  to  amyotrophic  lateral  sclerosis,  other  conditions 
causing  atrophic  paralysis  being  considered  elsewhere. 

CHRONIC  POLIOMYELITIS. 

Pathology.  —  The  pathological  changes  present  in  this  disease  con- 
sist of  a  slowly  advancing  atrophy  in  the  primary  motor  neurones  of 
the  cord,  cell  bodies,  dendrites,  and  axones  degenerating  together. 
These  cells,  as  already  stated  (p.  52),  lie  in  groups  in  the  anterior 
horns  and  in  the  central  gray  matter,  and  the  lesion  affects  these  groups 
in  different  degrees. 

We  have  several  clinical  types  of  chronic  anterior  poliomyelitis, 
due  to  the  fact  that  the  lesion  may  begin  in  different  parts  of  the  spinal 
cord. 

In  the  first  type  the  atrophy  begins  in  the  lower  groups  of  cells  of 
the  lumbosacral  region,  and  extends  to  all  the  groups  in  the  lumbar 
enlargement.  The  paralysis  begins  in  the  peronei  and  anterior  tibial 
groups  of  muscles,  then  advances  to  the  adductors  of  the  thigh  and 
glutei,  and  finally  invades  all  the  muscles  of  the  legs,  but  does  not  ex- 
tend to  the  arms. 

In  the  second  type  of  the  disease  (Duchenne's  subacute  ascending 
paralysis)  there  is  a  progressive  atrophy  of  the  cells,  beginning  in  the 
lumbosacral  region  and  advancing  gradually  up  the  cord  until  all  the 
motor  cells  are  involved.  The  cells  in  the  sacral  region,  in  which  the 
lesion  begins,  show  a  more  advanced  and  complete  atrophy  than  those 
in  the  cervical  region,  though  at  the  end  of  the  disease,  in  case  the 
patient  is  not  carried  off  by  some  intercurrent  affection,  a  large  majority 
of  the  cells  of  the  anterior  horns  have  disappeared. 

In  the  third  type  (the  progressive  muscular  atrophy  of  spinal  origin 
of  Aran-Duchenne)  the  atrophic  process  is  limited  to  certain  groups 
of  cells  in  the  cervical  region  of  the  cord,  especially  in  the  first  dorsal, 
eighth  and  seventh  cervical  segments  which  govern  the  hands.  After 
it  has  progressed  to  a  considerable  extent  in  these  segments,  the  groups 
of  cells  in  the  fifth  segment,  which  control  the  deltoid  muscle,  and  the 
adjacent  groups  controlling  the  biceps,  coracobrachialis,  and  supinator 
longus  muscles,  become  affected,  and  then  finally  all  the  groups  of  the 
cervical  enlargement  are  attacked  by  the  disease.  Later  the  same 
lesions  appear  in  the  lumbar  region  in  the  various  groups  of  cells,  and 
tlie  paralysis  extends  to  the  legs. 

The  lesion  in  all  these  types  is  similar.  The  difference  in  the  clini- 
cal symptoms  is  wholly  due  to  the  difference  of  location,  different 


286  CEEONIC    ATBOPEIC    PABALYSIS. 

groups  of  cells  being  first  attacked  or  progressively  invaded  in  the 
three  types  of  case.  Tlie  difference  between  the  subacute  and  chronic 
cases  is  only  the  difference  of  rapidity  in  progress,  and  no  hard-and- 
fast  line  can  be  drawn  between  the  two. 

The  exact  lesions  in  these  affections  consist  in  changes  which  are 
only  visible  to  the  microscope.  There  is  no  deformity  or  atrophy  of 
the  spinal  cord  on  inspection,  and  there  is  no  change  in  the  mem- 
branes of  the  cord.  The  anterior  nerve  roots  may  appear  to  be 
slightly  atrophied,  and  are  liable  to  tear  more  easily  in  the  process  of 
extraction  of  the  cord.  On  microscopic  examination  there  is  not  to  be 
seen  any  congestion  of  the  cord  or  any  exudation  of  cells  within  the 
substance,  but  a  simple  progressive  atrophy  in  various  stages  in  the 
cells  of  the  anterior  horns  and  of  the  central  gray  matter.  There  is 
no  breaking  of  the  dendrites,  no  swelling  of  the  axoues,  no  intracel- 
lular vacuolization,  no  hernia  of  the  nucleus,  and  no  diffuse  chroma- 
tolysis.     There  is  no  trace  of  capillary  hemorrhages. 

The  cell  remains  with  all  its  characteristics,  but  is  simply  shrunken. 
In  the  early  stage  the  body  of  tbe  cell  is  small  and  its  nucleus  is 
diminished  in  volume,  but  the  cell  retains  its  polygonal  form,  merely 
becoming  too  small  for  its  lymphatic  space.  Its  chromatophile  granules 
appear  small,  almost  like  a  mass  of  dust  within  the  cell,  and  may  be 
thicker  about  the  nucleus  or  in  the  periphery.  Collections  of  pigment 
in  the  cell  appear  in  large  quantity.  Little  by  little  the  cell  diminishes 
until  the  only  thing  left  is  a  round  cellular  nucleus  with  a  small  nucle- 
olus or  a  mass  of  pigment  granules.  (See  Plate  III.,  F,  G.)  The  lesion 
attacks  not  only  the  large  and  small  cells  which  give  origin  to  the  ante- 
rior nerve-root  fibres,  but  also  the  large  and  small  cells  which  give  rise 
to  the  association  fibres  passing  into  the  antero-lateral  column  of  the 
spinal  cord.     The  cells  of  the  column  of  Clarke  are  not  affected. 

As  a  consequence  of  this  atrophy  of  the  cells  there  is  a  correspond- 
ing atrophy  and  disappearance  of  their  dendrites  and  axones.  As  the 
dendrites  disappear  the  fine  plexus  of  fibres  in  the  gray  matter  be- 
comes less  noticeable.  As  the  axones  atrophy  there  is  a  shrinkage  in 
the  antero-lateral  tracts  of  the  cord  due  to  a  disappearance  of  the 
anterior  motor  root  fibres,  and  also  of  the  association  fibres  that  pass 
to  other  levels  of  the  cord.  The  atrophy  of  the  fibres  within  this 
column  is  diffuse,  fibres '  here  and  there  through  the  entire  antero- 
lateral column  being  found  atrophied  ;  hence,  although  no  lesion  is  ap- 
parent by  the  Weigert  stain,  the  Marchi  method  reveals  this  diffuse 
degeneration.  A  picrocarmine  stain  demonstrates  very  slight  sclerosis 
of  the  antero-lateral  column  adjacent  to  the  anterior  horn  of.  the  cord 
on  all  its  sides.  The  other  tracts  are  normal.  There  is  marked  de- 
geueration  and  atrophy  in  the  anterior  nerve-root  fibres  and  in  the 
nerves  to  their  termination  in  the  muscles.  There  is  a  single  atrophy 
of  the  muscular  fibres  in  the  muscles  which  are  paralyzed.  This 
atrophy  consists  of  a  granular  disintegration  or  a  disintegration  of  the 
;nuscles,  with  fine  fatty  deposits  and  disappearance  of  striation  and  ^ 


CEEONIC   POLIOMYELITIS. 


287 


simple  atrophy  of  the  muscular  fibres.     Occasionally  a  single  hyper- 
trophied  muscular  fibre  may  be  fi^und.^ 

Symptoms.  —  The  symptoms  of  chronic  anterior  poliomyelitis  differ 
in  the  different  types  of  cases.  First,  in  chronic  ascending  paralysis 
we  have  a  slowly  advancing  paralysis  beginning  in  the  peronei 
muscles,  first  in  one  leg,  but  within  two  or  three  months  of  the  onset 

Fig.  116. 


The  anterior  horn  in  the  lumbar  region  in  a  case  of  chronic  anterior  poliomyelitis.    Total  disappear- 
ance of  the  cells  and  thinning  of  the  network  of  fibres  within  the  horn.     (Oppenheim.) 


appearing  also  in  the  other  leg  and  causing  a  sensation  of  weakness 
in  walking,  with  a  tendency  to  dropping  of  the  feet.  The  paralysis 
corresponds  exactly  with  the  degree  of  atrophy  present.  After  some 
months   the  weakness  appears  in  the  anterior  tibial  group  of  muscles 

^  Recent  cases  with  careful  pathological  observations  in  this  disease  have  been  re- 
ported by  Phillipe  and  Ceston  before  the  Neurological  Section  of  the  International 
Medical  Congress,  Paris,  1900  ;  also  Vjy  Raymond  and  Rickling  at  the  same  Congress  ; 
by  J.  B.  Charcot,  Tliese  de  Paris,  1892,  and  by  T.  Aoyama,  Deut.  Zeltschr,  f.  JSferven* 
heilk.,  xxvL,  375,  1904. 


288  CHBONIC    ATBOPHIC    PABALYSIS. 

and  then  a  fully  developed  drop-foot  appears.  The  patient  walks  with 
difficulty  and  steps  high  in  order  to  avoid  stumbling  over  his  toes. 
Any  dorsal  flexion  of  the  foot  is  impossible  when  the  patient  is  lying 
down,  and  while  standing  he  cannot  lift  the  toes  from  the  floor.  The 
paralysis  and  atrophy  go  on  little  by  little,  measurements  showing  a 
reduction  of  perhaps  one-quarter  of  an  inch  every  month  in  the  calf. 
The  mechanical  excitability  of  the  muscles  is  diminished,  but  not  lost, 
and  there  is  very  often  a  fine  fibrillary  twitching  in  the  muscles,  caus- 
ing a  wave-like  movement  of  the  skin,  especially  when  the  muscles 
are  exposed  to  cold  or  are  percussed.  Patients  may  perceive  fatigue 
on  slight  exertion  and  may  have  slight  muscular  pains  in  the  leg,  but 
they  have  no .  sharp  pains  and  absolutely  no  sensory  disturbances. 
After  several  months  the  paralysis  advances  to  the  adductors  of  the 
thigh  and  to  the  glutei  muscles.  Then  walking  becomes  even  more 
difficult,  going  up  stairs  becomes  impossible,  and  it  is  with  difficulty 
that  the  patient  rises  from  the  chair.  In  one  case  still  under  my 
observation  two  years  elapsed  before  this  stage  was  reached.  Then 
about  simultaneously  the  posterior  tibial  muscles  of  the  leg  and  the 
anterior  muscles  of  the  thigh  are  invaded,  and  also  the  psoas  and  iliacus 
muscles,  and  when  the  paralysis  in  these  muscles  is  advanced  to  a 
moderate  degree  the  patients  are  no  longer  able  to  walk.  The  measure- 
ments of  the  legs  are  by  this  time  reduced  several  inches,  and  not 
uncommonly  the  atrophy  becomes  extreme.  The  legs  feel  rather  cold 
to  the  touch,  but  there  is  rarely  any  oedema,  and  there  is  no  tendency 
to  bed-sores.  The  disease  may  not  go  beyond  this  point,  and  for  years 
the  patient  may  live  in  a  state  of  paraplegia.  There  is  no  tendency 
to  any  afi'ection  of  the  bladder  or  rectum,  and  the  muscles  of  the  back 
may  escape. 

In  the  second  type  —  Duchenne's  ascending  paralysis  —  there  is  a 
tendency  to  progression  upward  of  the  affection.  The  muscles  of  the 
back  and  trunk  become  involved,  the  patient  can  no  longer  sit  upright 
in  bed  or  on  a  chair,  and  usually  the  muscles  of  the  shoulders  and  of 
the  hands  become  invaded  simultaneously,  and  inability  to  use  the 
upper  extremities  gradually  develops.  The  course  of  the  atrophy  of 
these  cases  may  be  quite  similar  to  progressive  muscular  atrophy  of 
the  Aran-Ducheune  type,  and  finally  bulbar  paralysis  may  ensue. 
These  patients  commonly  die  of  some  intercurrent  disease,  pneumonia 
being  the  most  common  cause  of  death,  especially  if  the  respiratory 
muscles  become  affected. 

The  third  type  of  chronic  anterior  poliomyelitis  is  the  type  described 
by  Aran  and  Duchenne.  The  patient  first  notices  an  inability  to  move 
the  muscles  of  the  thumb  of  one  hand,  adduction  being  imperfect. 
Almost  simultaneously  there  is  a  paralysis  of  the  abductor  indicis,  and 
consequently  the  patient  cannot  separate  the  forefinger  from  the  middle 
finger.  The  paralysis  of  these  small  muscles  is  noticed  in  the  finer 
movements  of  writing,  playing  the  piano,  buttoning  the  clothes,  or 
picking  up  small  objects^  and  this  disability  is  the  first  thing  which 


CHRONIC   POLIOMYELITIS. 


289 


attracts  the  patient's  attention.  As  the  disease  goes  on  the  paralysis 
extends  to^the  other  fine  muscles  of  the  hand,  the  interossei,  lumbri- 
cales,  and  the  muscles  moving  the  little  finger.  And  parallel  with  the 
paralysis  there  is  an  atrophy  in  all  these  muscles,  causing  a  flattening 
of  the  thenar  and  hypothenar  eminences  and  a  distinct  thinning  of  the 
hand,  producing  a  sulcus  upon  its  outer  side.  The  thumb  can  no 
longer  be  touched  to  the  tip  of  the  fingers,  and  as  the  interossei  are 
flexors  of  the  first  phalanx,  flexion  of  the  fingers  is  confined  to  the 
second  and  third  phalanges ;    the  hand,  therefore,  cannot  be  closed 

Fig.  117. 


Hand  and  forearm  in  chronic  spinal  muscular  atrophy,  showing  especially  wasting  of  tl 
hypothenar  eminences  and  of  the  abductor  indicis.     (Dercum. ) 


thenar  and 


firmly.  A  characteristic  abnormal  position  is  soon  assumed  by  the 
hand  as  a  result  of  this  paralysis  of  the  interossei.  They  no  longer 
aflbrd  any  opposition  to  the  long  flexor  and  extensor  muscles.  The 
long  flexors  flex  the  second  and  third  phalanges,  the  long  extensors  ex- 
tend the  first  phalanx,  hence  the  hand  is  thrown  into  a  position  called 
main  en  griff e  or  claw-hand.  (See  Fig.  118.)  And  as  the  interossei 
are  much  atrophied  the  tendons  of  the  long  muscles  stand  out 
upon  the  hand  forming  distinct  cords  which  may  be  felt  and  seen. 
At  the  same  time  a  paralysis  of  the  thenar  muscles  results  in  a  hyper- 
extension  of  the  first  phalanx  of  the  thumb,  with  some  rotation  out- 
ward, so  that  the  ball  of  the  thumb  is  in  a  plane  parallel  with  that  of 
the  palm. 

As  a  rule,  one  hand  is  invaded  some  months  before  the  other,  and  in 
several  cases  I  have  seen  the  disease  come  to  a  standstill  when  only 
one  hand  was  afiected ;  but,  as  a  rule,  there  is  a  tendency  to  a  gradual 
progress  of  the  paralysis  and  atrophy,  until  both  hands  are  almost  use- 
less. In  one  case  three  years  elapsed  before  this  condition  was  reached. 
But  before  this  stage  is  reached  a  progressive  atrophy  appears  in  the 
deltoids,  first  in  one,  and  then  in  the  other  shoulder.  In  consequence 
of  this  paralysis  abduction  of  the  arms  above  the  horizontal  line  is 
impossible,  and  when  the  patient  is  stripped  it  is  evident  that  almost 
all  abduction  of  the  arm  is  produced  by  rotation  of  the  shoulder-blade 
by  the  serratus  magnus  muscle. 

The  paralysis  tlicn  extends  from  the  deltoid  to  the  biceps,  brachialis 


19 


290 


CHBONIC    ATBOPHIC    PABALTSIS. 


anticus,  and  supinator  longus  muscles.  The  supraspinatus  and  infra- 
spinatus are  also  aifected,  producing  a  marked  atrophy  about  the 
shoulder-blade,  and  later  the  teres  and  subscapularis  become  affected. 
In  this  condition  the  patient  is  completely  paralyzed  in  the  arms, 
which  hang  like  flails  at  the  side,  and  are  of  very  little  use,  flexion  at 
the  elbow  being  impossible  by  voluntary  effort. 

As  the  disease  progresses  beyond  this  point  the  atrophy  attacks  the 
muscles  of  the  body  about  the  shoulders.  The  serratus  magnus  is 
paralyzed,  and  then  the  shoulder-blade  sticks  out  from  the  side  of  the 
body  like  a  wing.  The  rhomboids,  the  levator  anguli  scapulae,  and 
the  lower  half  of  the  trapezius  become  paralyzed,  the  shoulder-blade 
cannot  be  moved,  and  the  muscles  of  the  back  become  so  atrophied 
that  all  the  bones  are  visible.  Even  in  the  extreme  cases,  however, 
certain  muscles  escape  for  some  unknown  reason — namely,  the  upper 
part  of  the  trapezius  between  the  occipital  bone  and  the  clavicle,  the 
triceps,  latissimus  dorsi,  and  the  lower  half  of  the  pectoralis  major. 

Fig.  118. 


Atrophy  and  paralysis  with  main  en  griffe  in  chronic  anterior  poliomyelitis. 

As  these  muscles  stand  out  in  contrast  to  the  atrophied  muscles  about 
them  and  contract  because  of  the  lack  of  opposition,  deformity  of  the 
shoulders  and  of  the  neck  is  quite  evident. 

As  the  disease  extends  the  muscles  of  the  neck  are  next  invaded, 
and,  as  these  hold  the  head  in  an  erect  posture,  gravitation  leads  to  a 
falling  forward  of  the  head,  so  that  the  chin  rests  upon  the  chest. 
This  falling  forward  of  the  head  is  usually  intensified  by  the  contrac- 
tion of  the  sterno-cleido-mastoid  muscles,  which  are  not  affected  by 
the  disease. 

In  the  last  stage  of  the  case  the  paralysis  and  atrophy  extend  to  the 
intercostal  muscles  and  to  the  muscles  of  respiration,  so  that  all  respi- 
ration becomes  diaphragmatic.  As  a  rule,  patients  die  from  some  in- 
tercurrent disease  when  the  atrophy  and  paralysis  have  reached  this 
stage  ;  but  in  case  they  live  the  paralysis  may  extend  to  the  legs,  cans- 


CEBONIC   POLIOMYELITIS.  291 

ing  a  condition  of  paraplegia  of  progressive  type  such  as  has  been 
already  described  in  the  first  class  of  cases.  In  a  number  of  cases  of 
both  types  there  has  been  an  extension  of  the  paralysis  and  atrophy  to 
the  muscles  of  the  face,  of  the  tongue,  and  of  the  throat,  producing  a 
gradual  difficulty  in  articulation,  in  swallowing,  and  in  all  movements 
of  the  face,  and  death  by  suffocation  or  choking. 

The  symptoms  of  this  complication  are  more  fully  described  under 
the  head  of  bulbar  palsy. 

In  all  these  forms  of  paralysis  with  atrophy  there  are  frequent  fibril- 
lary contractions  in  the  paralyzed  muscle,  which  are  both  spontaneous, 
and  can  be  caused  by  percussion  or  by  exposure  of  the  limb  to  cold. 
There  are  also  progressive  electrical  changes  in  the  muscles.  A  partial 
reaction  of  degeneration  is  the  type  of  change  most  commonly  found. 
The  nerves  react  to  both  faradic  and  galvanic  currents,  but  there  is  a 
progressive  diminution  in  the  degree  of  contraction,  and  hence  very 
strong  currents  are  necessary  in  order  to  produce  any  effect.  The 
muscles  respond  to  the  galvanic  current  very  sluggishly,  with  a  so- 
called  "vermiform  contraction,"  and  usually  react  better  to  the  posi- 
tive than  to  the  negative  pole.  Finally,  all  faradic  reaction  is  lost,  a 
complete  reaction  of  degeneration  develops,  and  as  the  muscle  becomes 
completely  atrophied  all  reaction  to  galvanism  is  lost.  The  knee-jerk 
is  not  affected  in  the  disease  until  the  rectus  femoris  becomes  atrophied, 
when  it  becomes  gradually  diminished,  and  finally  is  lost.  In  a  few 
cases  the  reflex  has  been  increased.  As  already  stated,  sensation  is 
normal  from  beginning  to  end,  and  there  is  no  affection  of  the 
sphincters. 

The  duration  of  the  disease  varies  very  much  in  different  cases. 
The  most  rapid  case  that  I  have  seen  was  two  years  in  its  duration. 
But  an  arrest  of  the  atrophy  may  occur  at  any  time  in  the  course  of 
the  case,  and  hence  the  prognosis  must  be  very  guarded,  as  it  is  unjust 
to  condemn  these  patients  when  there  is  still  hope. 

While  the  majority  of  the  cases  correspond  to  one  of  the  three  types 
described,  it  is  not  to  be  forgotten  that  a  progressive  muscular  atrophy 
may  begin  in  any  muscle  of  the  body  and  advance  to  any  other  set  of 
muscles  with  a  most  irregular  course.  Thus  Werdnig^  has  reported 
a  case  in  which  the  paralysis  began  in  the  muscles  of  the  back  and 
glutei,  then  advanced  to  the  neck  and  throat,  and  finally  to  the  extremi- 
ties. Striimpell  ^  has  recorded  a  case  in  which  the  progress  was  from 
the  hands  to  the  forearms  and  then  to  the  shoulder,  in  distinction  from 
the  ordinary  progress  from  the  hands  to  the  shoulders.  Others  have 
described  cases  in  which  both  hands  and  legs  have  been  invaded 
together. 

Diagnosis.  —  As  already  stated,  chronic  atrophic  paralysis  may 
occur  in  several  diseases.  The  differentiation  of  chronic  anterior 
poliomyelitis  from  amyotrophic  lateral  sclerosis  is  as  follows : 

In  amyotrophic  lateral  sclerosis  there  is  an  increase  in  the  knee-jerks 

1  Arch.  f.  Psych.,,  xxvi.,  706. 

^Deut.  Zeitschr.  f'iir  Nervenheilk.,  iii.,  6. 


292 


CEBONIC    ATBOPHIC    PABALYSIS. 


early  in  the  disease,  an  early  development  of  Babinski's  reflex  —  i.  e., 
retraction  of  the  great  toe  and  flexion  of  the  other  toes  on  tickling  the 
sole  —  and  increased  mechanical  excitability  in  the  muscles  that  are 
paralyzed ;  a  tendency  to  rigidity  in  the  movement  of  the  legs,  and 
some  spastic  rigidity  of  the  arms;  also,  an  increase  in  the  tendon  re- 
flex at  the  elbow  and  wrist.  In  other  words,  the  symptoms  of  lateral 
sclerosis  are  added  to  the  symptoms  of  progressive  muscular  atrophy, 
and  it  is  by  a  discovery  of  these  additional  symptoms  that  the  diagnosis 
is  made.  The  progress  of  the  case  is  usually  more  rapid  in  amyo- 
trophic lateral  sclerosis  than  in  chronic  anterior  poliomyelitis ;  and 


Fig.  119. 


Atrophy  of  the  muscles  about  the  shoulder-blades  and  arms  in  a  case  of  chronic  anterior 
poliomyelitis.     The  triceps  and  lalissimus  dorsi  have  escaped. 

bulbar  symptoms  either  appear  early  in  the  aiFection  or  precede  the 
paralysis  in  the  limbs. 

A  differential  diagnosis  from  muscular  dystrophy  is  to  be  made  by 
a  consideration  of  the  progress  of  the  case  and  the  succession  of  im- 
plication of  the  various  muscles,  which  is  quite  characteristic  in  the 
different  types  of  muscular  dystrophy,  and  differs  from  that  already 
described.  (See  Muscular  Dystrophy,  Chapter  XVI.)  In  muscular 
dystrophy  there  is  never  any  fibrillary  contraction  in  the  paralyzed 
muscles.  The  atrophy  of  the  muscles  is  often  attended  by  a  deposit 
of  fat,  so  that  there  is  an  appearance  of  hypertrophy  in  the  muscles  that 
are  really  atrophied.  The  tendon  reflexes  in  muscular  dystrophy  are 
progressively  diminished  as  the  muscles  become  atrophied.  The  his- 
tory of  the  case,  its  onset  in  childhood,  its  development  in  a  child  of  a 
family  in  which  other  members  ha-ve  been  known  to  be  affected  are 


CBMNIC  POLIOMYELITIS.  293 

important  facts  in  connection  with  the  differentiation  from  progressive 
muscular  atrophy.  There  is  no  electrical  change  in  the  muscles  affected 
in  dystrophy. 

In  the  muscular  atrophies  that  are  consequent  upon  neuritis  there  are 
usually  sensory  symptoms  and  tenderness  along  the  nerve  trunks,  and 
there  is  a  history  of  one  of  the  well-known  causes.     (See  Chap.  VIII.) 

In  cases  of  paralysis  of  the  ulnar  nerve  the  distribution  of  the  pa- 
ralysis and  atrophy  to  the  muscles  in  the  hand  may  simulate  progres- 
sive muscular  atrophy,  but  it  is  to  be  remembered  that  in  ulnar  paral- 
ysis the  first  and  second  interossei  escape  and  there  is  usually  some 
affection  of  sensibility  in  the  little  finger  (see  page  185).  J.  R.  Hunt^ 
has  described  an  atrophy  of  the  hands  due  to  a  neuritis  of  the  deep 
palmar  branch  of  the  the  ulnar  nerves  occurring  in  certain  workmen, 
who  use  the  fingers,  handling  files ;  and  he  considers  that  this  neuritis 
is  the  cause  of  an  atrophic  paralysis  of  the  hands  described  by  Gessler 
as  an  occupation  atrophy. 

The  symptoms  of  progressive  muscular  atrophy  are  sometimes  the 
first  symptoms  to  develop  in  a  case  of  syringomyelia,  but  the  subse- 
quent appearance  of  dissociated  anaesthesia  (a  loss  of  sensibility  to  pain 
and  temperature,  while  that  to  touch  is  present)  and  of  trophic  symp- 
toms, ulcerations  of  the  skin,  etc.,  together  with  the  usual  increase  of  the 
knee-jerks,  will  enable  the  diagnosis  to  be  made.   (See  Chapter  XVII.) 

A  differentiation  between  progressive  muscular  atrophy  and  the 
Charcot-Marie  ^-Tooth  ^  type  of  atrophic  paralysis  is  somewhat  difficult. 
That  disease  appears  in  early  youth.  It  produces  a  progressive  mus- 
cular atrophy  of  the  peroneal  type  in  the  legs  and  of  the  Aran-Du- 
chenne  type  in  the  hands,  about  simultaneously  or  in  rapid  succession ; 
but  the  paralysis  does  not  extend,  as  a  rule,  above  the  knees  or  elbows, 
though  occasionally  the  muscles  around  the  thigh  and  glutei  are  in- 
vaded. The  contrast  between  the  atrophied  lower  parts  of  the  limbs 
and  the  well-developed  upper  portions  of  the  limbs  is  very  marked. 
Fibrillary  tremor  and  reaction  of  degeneration  are  present  in  both  dis- 
eases, but  in  the  Charcot-Marie-Tooth  type  sensation  is  usually  dimin- 
ished in  the  legs  and  feet  and  on  the  hands.  In  this  disease  a  few 
autopsies  (Hoffman,  Muhlenburg,  Dubreihl  and  Marinesco)  have  dem- 
onstrated a  peripheral  neuritis  with  some  posterior  sclerosis,  but  no 
affection  of  the  anterior  horns.      (See  Chapter  XVI.) 

Prognosis.  —  The  prognosis  in  chronic  anterior  poliomyelitis  is  a 
serious  one,  as  the  tendency  of  the  disease  is  to  go  on  to  extreme 
atrophy  and  death  from  respiratory  or  bulbar  paralysis.  It  must  be 
remembered,  however,  that  an  arrest  at  any  time  in  the  course  of  the 
case  is  possible.  Gowers  maintains  that  the  cases  in  which  the  disease 
begins  on  V)oth  sides  simultaneously  are  more  liable  to  a  spontaneous 
arrest.  When  the  disease  has  once  been  arrested  there  is  no  return  of 
power  and  no  increase  in  size  in  the  affected  muscles,  which  are  per- 

^See  J.  E.  Hunt.     Occupation  Neuritis.     Jour.  Nerv.  and  Ment.  Dis.,  Nov.,  1908. 

''Rev.  de  MM.,  February,  1880,  p.  97. 

» Tooth.     Dissertation,  London,  1886,     Neurol.  Centralbl.,  1887,  vi.,  16. 


294  CEBONIC    ATBOPHIC    PABALYSIS. 

manently  paralyzed.  If  the  disease  starts  up  a  second  time  after  an 
arrest  of  its  progress  the  prognosis  is  most  unfavorable,  as  a  rapid 
course  is  probable.  Death  occurs  from  paralysis  of  the  respiratory 
muscles,  or  from  bronchitis  or  pneumonia  consequent  upon  the  dis- 
turbance of  respiration,  or  from  bulbar  palsy 

Treatment.  —  The  treatment  of  chronic  anterior  poliomyelitis  is 
the  same  as  that  of  acute  anterior  poliomyelitis  in  the  chronic  stage 
—  namely,  generri  hygienic  measures ;  good  food,  especially  of  fatty 
kind  ;  fresh  air,  and  every  means  of  keeping  up  the  general  nutrition  of 
the  patient.  Exercise,  while  not  prohibited,  should  be  advised  in  great 
moderation,  and,  if  the  legs  are  affected,  walking  should  be  avoided, 
so  as  not  to  put  any  strain  upon  the  paralyzed  muscles.  Any  effort 
which  causes  fatigue  is  sure  to  be  followed  by  an  increase  in  the  weak- 
ness and  a  more  rapid  atrophy.  Any  exhausting  disease,  such  as  an 
intercurrent  attack  of  the  grippe,  pneumonia,  or  any  infectious  fever, 
or  a  severe  gastro-enteritis  is  very  likely  to  increase  the  paralytic 
symptoms.  Thus  in  one  of  my  patients  who  had  been  in  a  stationary 
condition  for  a  year  an  attack  of  the  grippe  was  followed  by  a  rapid 
increase  in  his  paralysis.  There  is  no  treatment  that  will  arrest  the 
progress  of  the  paralysis.  Massage  and  rubbing  are  usually  prescribed 
and  may  possibly  aid  in  keeping  up  the  general  strength  by  increasing 
the  nutrition  of  the  atrophied  muscles.  I  have  never  seen  any  benefit 
from  the  continued  use  of  electricity,  though  it  is  usually  prescribed. 
If  it  is  used  it  should  be  with  a  current  only  sufficient  to  produce 
contraction  in  the  muscles,  and  very  strong  or  very  many  long-con- 
tinued applications  are  to  be  avoided  on  the  same  principle  that  exer- 
cise is  to  be  avoided.  The  muscles  should  not  be  overworked  or 
fatigued.  In  one  case  the  use  of  mechanical  massage  and  vibratory 
treatment  at  the  Zander  Institute  caused  marked  improvement  and  was 
followed  by  an  arrest  in  the  progress  of  the  disease  for  a  year. 

General  tonics  are  of  distinct  service,  and  I  have  seen  temporary 
improvement  from  the  employment  of  strychnine,  -^-^  grain,  three  times 
a  day  for  four  days  in  the  week,  alternating  with  arsenic,  gJ^  grain 
three  times  a  day  for  the  other  three  days  of  the  week.  Some  authors 
recommend  the  hypodermic  use  of  strychnine,  but  in  a  long-continued 
chronic  disease  of  this  kind  very  little  advantage  is  to  be  obtained,  and 
the  dangers  attendant  upon  septic  infection  are  many  Apparatus  may 
prevent  deformities  and  assist  weakened  joints  to  bear  the  weight  of 
the  body  after  walking  becomes  impossible. 

AMYOTROPHIC  LATERAL  SCLEROSIS. 

This  is  a  chronic  progressive  form  of  spinal  paralysis  characterized 
by  the  symptoms  of  progressive  muscular  atrophy  in  the  arms  and  of 
lateral  sclerosis  or  spastic  paraplegia  in  the  legs.  It  was  first  described 
by  Charcot  in  1872  and  was  carefully  studied  by  his  pupils,  Gombault 
in  1877  and  Debove  in  1879.  It  has  been  called  Charcot's  disease. 
But  while  Charcot  believed  that  it  was  a  disease  of  the  spinal  cord 


AMYOTBOPHIC   LATERAL    SCLEROSIS.  295 

only,  it  is  now  known  that  all  the  motor  elements  of  the  nervous  system, 
from  the  cortex  of  the  brain  to  the  termination  of  the  nerves  in  the 
feet,  are  inVolved,  both  the  cortico-spinal  and  spinomuscular  elements 
being  simultaneously  affected. 

Pathology.  —  The  pathological  condition  consists  of,  first,  a  gradual 
progressive  atrophy  in  the  motor  neurones  lying  in  the  anterior  horns 
of  the  spinal  cord,  similar  to  that  described  as  occurring  in  chronic 
anterior  poliomyelitis.  This  is  sometimes  limited  to  the  cervical  region, 
but  in  long-continued  cases  the  lumbar  region  is  affected  and  in  some 
cases  the  entire  cord  is  involved.  These  changes  in  the  cells  are  also 
present  in  the  motor  nuclei  of  the  cranial  nerves  in  the  medulla  and 
pons.  Secondly,  in  addition  to  these  changes  in  the  spinomuscular 
element,  there  are  atrophy  and  degeneration  in  the  cortico-spinal  ele- 
ment which  lies  in  the  lateral  pyramidal,  and  anterior  median  columns 
of  the  spinal  cord.  These  changes  have  been  followed  upward  through 
the  medulla,  pons,  crus,  and  internal  capsule  to  the  motor  cells  of  the 
brain.  And  recent  investigation  has  shown  that  in  the  majority  of  cases 
of  advanced  amyotrophic  lateral  sclerosis  there  is  a  degeneration,  with 
atrophy  of  the  larger  cells  of  the  second  and  third  layers  of  the  cortex 
about  the  fissure  of  Rolando,  which  are  the  neurone  bodies  of  the 
cortico-spinal  elements  of  the  motor  system.  This  degeneration  of  the 
cortico-spinal  elements  begins  in  the  ends  of  the  axones  in  the  spinal 
cord  and  advances  upward  until  the  entire  neurone,  of  whatever  length, 
becomes  affected  and  atrophied.  The  sclerosis  is  a  replacement  hyper- 
plasia in  the  spinal  cord,  the  increased  connective  tissue  taking  the 
place  of  the  atrophied  nervous  elements.  The  sclerosis  is  not  wholly 
limited,  however,  to  the  motor  tracts  in  the  cord,  but  is  present  through- 
out the  antero-lateral  columns  in  the  vicinity  of  the  gray  horns,  as  in 
chronic  anterior  poliomyelitis.  (See  Fig.  120.)  It  is  evident,  there- 
fore, that  the  association  tracts  belonging  to  the  motor  system  of  the 
cord  are  involved  as  well  as  the  longer  voluntary  tracts ;  hence  this 
disease  is  one  of  the  most  widespread  of  all  the  so-called  "  system 
diseases  "  of  the  spinal  cord. 

The  point  of  beginning  of  the  pathological  process  varies  in  different 
cases.  In  some  cases  it  is  evident  that  the  lesion  begins  in  the  cervical 
region  of  the  spinal  cord  in  the  gray  matter,  and  hence  the  symptoms 
first  appear  in  the  muscular  system  of  the  arms.  This  has  been  the 
course  in  about  60  per  cent,  of  my  cases.  It  was  the  course  in  39  out 
of  81  cases  analyzed  by  Collins.^  In  other  cases  the  lesion  commences 
in  the  lateral  columns  of  the  cord,  and  then  the  spastic  paralysis  of 
the  legs  is  the  first  evidence  of  the  disease.  This  is  the  second  form 
and  has  been  the  history  in  30  per  cent,  of  my  cases  and  in  14  of 
Collins'  cases.  In  either  case  in  the  course  of  the  disease  both  sets  of 
symptoms  appear.  In  the  cases  that  begin  in  the  spinal  cord  the  ten- 
dency of  the  disease  is  to  extend  to  the  medulla  and  pons,  so  that 
symptoms  of  bulbar  paralysis  ensue  and  terminate  the  case.     In  other 

*J.  Collins,  American  Journal  Medical  Sciences,  June,  1903.  See  also  Haeuel' 
Arch.  f.  Psych.,  Bd.  xxxvii.,  Th.  1,  1903. 


296  CHBONIC    ATBOPHIC    PABAL7SIS. 

cases,  however,  bulbar  paralysis  is  the  first  disease  to  appear,  and  later 
on,  after  its  symptoms  are  well  marked,  the  symptoms  of  spastic  para- 
plegia or  of  progressive  muscular  atrophy  in  the  hands  indicates  that 
the  original  disease  was  not  simply  bulbar  palsy,  but  amyotrophic 
lateral  sclerosis.  This  has  been  the  course  in  10  per  cent,  of  my 
cases.     It  was  the  course  in  25  per  cent,  of  Collins'  cases.     The  lesion 

Fig.  120. 


The  lesions  in  amyotrophic  lateral  sclerosis,  lumbar  region.  A,  anterior  horn,  atrophied  and 
sclerotic,  no  cells  remaining ;  B,  posterior  horn  ;  C,  posterior  nerve  root ;  D,  posterior  septum ;  E, 
posterior  commissure  ;  F,  anterior  commissure  ;  G,  anterior  fissure  ;  JI,  antero-lateral  column,  slightly 
sclerotic ;  /,  posterior  column  ;  J,  lateral  pyramidal  tract,  sclerotic.     (Blocq.) 

is  a  slowly  progressive  one,  occasionally  comes  to  a  standstill  for  a 
time,  but  never  has  any  tendency  to  recover,  regeneration  not  appear- 
ing in  the  degenerated  neurones. 

Etiology.  —  Very  little  is  known  about  the  causation  of  this  disease. 
It  develops  in  persons  between  the  ages  of  thirty-five  and  fifty  years. 
Cold,  overexertion,  various  forms  of  poisoning,  either  by  alcohol, 
arsenic,  lead,  mercury,  or  by  toxins  of  infectious  diseases,  or  from  auto- 
intoxication through  disturbance  of  the  gastro-intestinal  tract,  rheuma- 
tism, gout,  and  diabetes  have  all  been  mentioned  as  causes  without  any 
absolute  proof.  There  is  no  proof  that  the  affection  develops  subse- 
quently to  syphilis.  Gowers  has  seen  it  develop  after  severe  injuries. 
A  probable  theory  of  the  affection  is  that  in  certain  families  a  con- 
genital weakness  of  the  motor  elements  of  the  central  nervous  system 
is  present,  a  fact  which  receives  some  support  from  the  occurrence  of 
the  disease  occasionally  in  several  members  of  the  same  family.     lu 


AMYOTBOPHIC  LATERAL   SCLEROSIS. 


297 


some  cases  an  extensive  endarteritis  has  been  found  in  the  spinal 
bloodvessels,  and  this  has  been  supposed  to  be  the  cause  in  these  cases. 
Symptoirfs.  —  Amyotrophic  lateral  sclerosis  usually  begins  with 
stiffness  in  the  muscles  and  increased  reflex  excitability,  soon  followed 
by  atrophy  and  paralysis  in  one  or  both  hands,  the  small  muscles  of 
the  hands  being  the  parts  first  affected,  as  in  progressive  muscular 
atrophy  of  the  Aran-Duchenne  type.  The  paralysis  then  advances  to 
the  forearms,  though  occasionally  it  appears  in  the  muscles  about  the 
shoulders  before  those  of  the  arms  are  invaded.     Paralysis  and  atrophy 

Fig.  121. 


'W^M 


Sclerosis  of  the  spinal  cord.  The  specimen  is  taken  from  the  border  of  the  lateral  pyramidal  tract 
in  a  case  of  amyotrophic  lateral  sclerosis.  The  upper  part  of  the  specimen  is  normal :  the  lower  part 
is  sclerotic,  and  in  this  part  few  or  no  fibres  remain. 


go  hand-in-hand,  and  the  weakness  is  proportionate  to  the  size  of  the 
muscle,  as  in  progressive  muscular  atrophy.  Fibrillary  contractions 
are  present  in  the  muscles  from  the  beginning  of  the  disease,  and  may 
be  elicited  by  exposure  to  cold  or  by  percussion.  The  order  in  which 
the  various  muscles  are  invaded  corresponds  quite  closely  to  that  de- 
scribed in  progressive  muscular  atrophy  of  the  Aran-Duchenne  type. 
When  all  the  symptoms  are  well-developed  in  the  upper  extremities 
the  arms  are  adducted  to  the  body,  the  forearms  are  pronated,  and  the 
hands  flexed  or  in  the  position  of  majin  en  grife.  There  is  some 
resistance  offered  to  passive  movements,  as  the  muscles  are  rigid  and 
spastic  even  when  quite  weak. 

The  symptoms  appearing  in  the  legs  may  follow  or  precede  those  in 
the  arms  ;  l)ut,  as  a  rule,  a  considerable  length  of  time  (several  months) 
intervenes  between  the  two.  When  the  disease  begins  in  the  legs  the 
early  diagnosis  is  lateral  sclerosis.     The  legs  become  stiff,  and  the 


298 


CEBONIC    ATEOPHIC    PAEALYSIS. 


muscles  rigid,  so  that  the  patient  moves  them  with  some  difficulty ;  the 
knee-jerks  are  increased,  and  ankle  clonus  and  the  Babinski  reflex  can 
be  elicited.  The  gait  becomes  a  true  spastic  gait,  with  short  steps, 
scraping  of  the  foot  upon  the  ground,  tendency  to  overlapping  of  the 
knees  and  feet,  and  great  rigidity  in  bending  of  all  the  joints.  The 
muscles  of  the  legs  do  not  atrophy  in  the  early  stage  and  are  not  the 
subject  of  fibrillary  twitches.  The  symptoms  may  not  begin  in  both 
legs  at  the  same  time ;  in  fact,  one  leg  may  be  quite  rigid  for  several 
months  before  the  other  is  attacked.  The  rigidity  gives  rise  to  some 
aching  and  discomfort  in  the  muscles,  but  there  are  no  sharp  pains, 
and  there  are  no  sensory  disturbances  either  of  the  nature  of  anses- 

FlG.  122. 


Extreme  atrophy  of  the  thenar,  hypothenar,  and  interossei' muscles  of  the  hands  in  amyotrophic 
lateral  sclerosis.     (Dejerine.) 

thesia  or  parsesthesia.  The  bladder  and  rectum  are  not  in  any  way 
affected. 

In  this  condition  of  partial  paralysis  the  patient  may  remain  for 
several  months,  the  disease  coming  to  a  standstill,  or  the  disease  may 
go  on  progressively.  One  patient  in  my  clinic  remained  in  a  station- 
ary state  for  four  years  after  all  these  symptoms  had  developed.  If 
it  progresses  the  spastic  rigidity  of  the  legs  becomes  so  intense  that 
walking  is  impossible,  and  the  patient  is  confined  to  the  bed.  Then 
the  muscles  which  have  hitherto  been  rigid  begin  to  atrophy,  and  to 
show  fibrillary  twitchings,  and  the  spastic  paraplegia  is  succeeded  by 
a  flaccid  paralysis  quite  similar  to  that  which  has  appeared  from  the 
outset  in  the  hands  and  arms. 

Sometimes  the  paralysis  extends  from  the  upper  extremities  to  the 
neck,  the  head  falls  forward,  the  chin  resting  on  the  sternum,  and  the 
head  cannot  be  raised  or  turned. 


AMYOTltOPHiC   LATEEAL    SCLEROSIS.  299 

Some  months  before  complete  paralysis  has  developed  in  the  arms 
it  is  common  to  observe  some  symptoms  of  bulbar  palsy.  A  few  cases 
begin  with  these  symptoms,  and  only  develop  paralysis  of  the  arms 
and  legs  later.  The  first  symptom  noticed  is  a  disturbance  of  speech 
due  to  a  paralysis  of  the  muscles  of  the  tongue,  lips,  mouth  and  palate. 
The  speech  is  slower,  pronunciation  being  difficult ;  the  voice  becomes 
monotonous,  possibly  nasal  and  indistinct,  and  finally  pronunciation 
of  both  Unguals  and  labials  is  so  imperfect  that  it  is  with  difficulty 
that  the  patient  is  understood.  As  this  paralysis  goes  on  the  tongue 
becomes  thin,  is  thrown  into  folds,  and  shows  fibrillary  twitchings,  and 
cannot  be  freely  moved  and  protruded  from  the  mouth.  The  palate 
also  becomes  paralyzed,  the  uvula  is  no  longer  properly  elevated,  and 
hence  swallowing  is  imperfect,  fluids  return  through  the  nose,  and 
attacks  of  choking  are  frequent.  The  face  also  gradually  becomes 
paralyzed,  especially  in  its  lower  portions,  it  is  flattened  so  that  there 
is  a  mask-like  expression,  and  gradually  all  expression  is  obliterated. 
Whistling,  blowing,  or  kissing  is  impossible.  The  mouth  is  partly 
open,  the  saliva  runs  from  the  corners,  and  the  act  of  chewing  is  in- 
terfered with  on  account  of  the  paralysis  of  the  cheeks  and  on  account 
of  the  weakness  of  the  muscles  of  mastication.  The  muscles  of  the 
face  may  show  some  fibrillary  twitching  for  some  time  before  they  be- 
come paralyzed.  Finally  the  upper  branches  of  the  facial  nerve  are 
affected,  patients  cannot  close  the  eyes,  but  the  ocular  muscles  do  not 
often  share  in  the  paralysis.  One  or  two  cases  have  been  reported  in 
which  the  Argyll-Robertson  pupil  has  been  present.^  The  inability 
to  swallow  may  cause  death  from  choking  or  from  respiratory  paralysis, 
or  a  pneumonia  may  develop  from  irritation  of  the  lung  by  particles 
of  food  which  are  inhaled. 

One  of  the  characteristic  symptoms  of  the  disease  is  the  great  in- 
crease in  the  muscular  irritability  in  all  the  muscles  that  are  affected. 
Percussion,  either  on  the  muscle  or  on  its  tendon,  causes  a  quick, 
unusually  sharp  contraction,  and  this  exaggeration  of  reflex  activity 
throughout  the  entire  body,  both  in  the  muscles  that  are  atrophic  and 
in  those  that  are  spastic,  is  characteristic  of  the  disease,  and  is  not 
present  in  progressive  muscular  atrophy.  Percussion  of  almost  any 
point  upon  the  limbs  —  on  the  muscle,  tendons,  or  the  periosteum  — 
produces  sudden  contraction  of  groups  of  muscles,  and  sudden  exten- 
sion of  any  tendon  may  be  followed  by  a  clonus.  Percussion  of  the 
facial  muscles,  especially  of  the  masseter,  and  of  the  jaw  produces  sud- 
den contractions  and  even  the  chin  reflex  may  be  so  exaggerated  as  to 
give  rise  to  a  clonus. 

The  atrophic  paralysis  is  usually  attended  by  cramps  and  also  by 
deformities  [main  en  (/rife  or  drop-wrist)  such  as  appear  in  progressive 
muscular  atrophy.  If  deformities  occur  in  the  leg  talipes  may  develop 
from  contracture  of  the  posterior  tibial  tendons. 

In  the  later  stages  of  the  disease  the  muscles  of  the  trunk  and 

'  Schlesinger.  Obersteiner's  Arbeiten,  1900,  vol.  vii.,  p.  154.  Ziir  Kenntniss  atyp- 
ische  Formen  der  Amyotrophischen  Lateralsclerose. 


S<^<^  CHUOmC  ATBOPSIC  pa:baltsis. 

shoulders  and  neck  may  become  atrophied,  so  that  sitting  up  or  lifting 
the  head  is  impossible. 

The  electrical  examination  shows  a  diminution  of  excitability  both 
to  faradism  and  galvanism,  the  muscles  that  are  atrophied  requiring  a 
stronger  current  for  the  production  of  contraction.  A  partial  reaction 
of  degeneration  may  also  be  found. 

Tachycardia  occasionally  develops  in  the  later  stage  of  the  disease, 
as  in  bulbar  palsy,  and  usually  is  an  exceedingly  serious  symptom,  as  it 
may  cause  death.  The  brain  is  not  in  any  way  aifected,  excepting  in 
its  motor  region.  Intelligence  is  preserved,  consciousness  is  not 
affected,  memory  is  not  impaired,  and  epileptic  attacks  do  not  occur. 
Occasionally,  however,  as  in  bulbar  palsy,  an  unusual  emotional  ex- 
citability develops,  and  causeless  crying  or  laughing  indicate  a  weaken- 
ing of  the  patient's  power  of  self-control. 

The  duration  of  the  disease  varies  between  two  and  ten  years,  de- 
pending entirely  upon  the  time  of  onset  of  the  bulbar  symptoms,  which 
are  the  immediate  cause  of  death.  If  these  symptoms  appear  early  or 
are  the  first  to  occur  the  patient  rarely  lives  more  than  two  years.  If 
the  symptoms  first  appearing  are  those  of  lateral  sclerosis  the  case  may 
last  for  many  years.  The  immediate  cause  of  death  is  usually  dis- 
turbance of  respiration  or  suffocation  from  food  getting  into  the  larynx, 
or  pneumonia  from  food  being  received  in  the  lungs,  or  from  heart 
failure. 

The  course  of  the  disease  when  it  begins  with  bulbar  palsy  is  illus- 
trated by  the  following  case  :  I.  D.,  aged  forty-two  years,  had  been 
much  exposed  to  wet,  to  extreme  heat,  and  to  cold  in  his  occupation, 
and  had  suffered  from  muscular  rheumatism  for  years.  In  February, 
1889,  he  noticed  double  vision,  due  to  a  weakness  of  the  left  external 
rectus  muscle,  and  ptosis  which  first  affected  the  left  eye  and  then  the 
right  eye.  The  ptosis  gradually  subsided,  but  the  strabismus  re- 
mained. In  March,  1889,  his  speech  became  thick,  and  in  April  he 
began  to  have  difficulty  in  swallowing,  fluid  food  coming  out  of  his 
nose.  In  May  he  noticed  difficulty  in  chewing,  and  his  friends  saw  a 
change  in  his  facial  expression  due  to  a  weakness  of  all  the  facial 
muscles.  During  all  this  time  he  felt  an  increasing  weakness  in  all 
his  movements.  His  neck  had  become  stiff  and  his  head  tended  to  fall 
forward  and  was  held  with  chin  projecting  beyond  the  line  of  the 
body.  On  several  occasions  after  March,  1889,  he  had  fainting  attacks 
in  which  he  became  pulseless  and  pale.  In  June,  when  I  saw  him,  he 
was  thin  and  pale,  clear  in  his  mind,  but  feeble  in  all  movements. 
His  eyes  were  both  turned  in  a  little  and  his  left  eye  looked  up.  He 
had  corresponding  double  images,  but  no  nystagmus  and  his  pupils 
were  normal.  His  optic  nerves  were  normal.  There  was  anosmia  on 
the  left  side.  His  sensation  in  the  face  was  normal,  but  the  muscles 
of  mastication  were  atrophied,  did  not  react  to  faradism,  and  were  so 
weak  that  chewing  was  impossible.  He  moved  his  jaw  with  his  hand 
and  gave  it  constant  support.  His  face  was  expressionless ;  he  could 
not  whistle ;  food  collected  in  his  cheeks,  but  all  his  facial  muscles 


AMYOTBOPHIC   LATERAL    SCLEROSIS.  301 

could  be  slightly  moved,  and  reacted  to  faradism.  His  palate  was 
paralyzed,  ^nd  it  was  to  this  cause  that  his  defect  of  speech  was  due, 
as  the  tongue  was  not  paralyzed  or  atrophied.  His  neck  muscles  were 
weak.  There  was  an  atrophic  condition  of  the  thenar  muscles  and 
interossei  in  the  hands,  and  marked  weakness  in  all  the  muscles  of  the 
forearm.  The  deltoid  and  muscles  of  the  arms  were  in  good  condi- 
tion. His  gait  was  slow  and  feeble,  but  not  spastic ;  but  his  knee- 
jerks  were  exaggerated  and  anklg  clonus  was  obtained.  He  con- 
trolled his  sphincters  well.  During  the  following  year  his  paralysis 
increased  in  his  arms  and  legs,  his  inability  to  talk  became  more  ap- 
parent, and  in  August,  1890,  he  choked  to  death. 

In  the  following  case  the  symptoms  began  in  the  leg  on  one  side : 
A  woman,  aged  forty-five  years,  began  to  suffer  from  weakness,  fibril- 
lary twitchings,  and  atrophy  in  the  right  leg,  with  reaction  of  degenera- 
tion in  the  muscles  supplied  by  the  peroneal  nerve,  in  February,  1890. 
This  was  supposed  to  be  due  to  traumatism  of  the  nerve,  as  she  had 
had  an  injury.  But  in  April,  1890,  an  atrophy  had  begun  in  the 
thenar  and  hypothenar  eminences  of  the  right  hand.  These  symptoms 
increased,  and  in  October,  1890,  she  was  almost  completely  paralyzed 
in  the  entire  right  side  and  partially  in  the  left  side,  there  being  every- 
where an  atrophic  state  of  the  muscles  with  fibrillary  contractions  and 
reaction  of  degeneration.  There  was  no  pain,  no  sensory  disturbance, 
no  bladder  or  rectal  symptoms.  The  tendon  reflexes  were  increased. 
During  the  winter  of  1890-1891  the  paralysis  extended,  and  she  was 
finally  confined  to  the  bed.  In  April,  1891,  the  muscles  of  her  throat 
and  face  were  affected,  and  in  July  she  died  of  respiratory  paralysis. 

Diagnosis.  —  The  diagnosis  of  this  affection  does  not  present  any 
difficulties.  The  muscular  dystrophies  are  not  attended  by  fibrillary 
twitchings  or  by  increased  tendon  reflexes  or  by  bulbar  symptoms. 
Bulbar  paralysis  is  not,  as  a  rule,  attended  by  any  spinal  symptoms  or 
paralysis,  or  increased  rigidity,  or  increased  knee-jerks.  If  such  symp- 
toms develop  in  the  course  of  bulbar  paralysis  it  is  evident  that  the 
disease  is  a  true  amyotrophic  lateral  sclerosis  in  which  the  bulbar  symp- 
toms have  been  the  first  to  appear.  Compression  of  the  cervical  por- 
tion of  the  spinal  cord  by  tumor  or  by  pachymeningitis  is  usually 
attended  by  pain  in  the  shoulders,  neck,  and  arms,  by  stiffness  of  the 
cervical  vertebrae,  and  pain  from  pressure.  Localized  compression  of 
the  spinal  cord,  such  as  occurs  in  these  diseases,  if  attended  by  spastic 
paraplegia  is  usually  attended  also  by  sensory  disturbances  and  by  an 
affection  of  the  bladder  and  rectum.  The  diagnosis  from  syringomyelia 
is  made  by  the  absence  of  sensory  disturbances  or  trophic  affections, 
and,  as  is  well  known,  in  this  disease  bulbar  symptoms,  while  occa- 
sionally developing,  are  unilateral  and  do  not  present  the  typical  fea- 
tures of  a  bulbar  palsy.  Tumors  of  the  spinal  cord  of  the  nature  of 
gliomatosis  give  rise  to  sepsory  symptoms  as  well  as  to  spastic  para- 
plegia and  progressive  muscular  atrophy.  The  same  is  true  of  chronic 
myelitis.  In  any  case  of  ])rimary  lateral  sclerosis  it  must  be  remem- 
bered   that   spastic    paraplegia   often   goes   on   to   atrophic  paralysis. 


302  CEBONIC   ATEOPHIC    PABALYSI8. 

Hence  many  cases  of  true  amyotrophic  lateral  sclerosis  beginning  with 
symptoms  of  spastic  paralysis  in  the  legs  are  supposed  to  be  primary 
lateral  sclerosis.  Time  alone  can  decide  between  the  two  affections. 
In  a  large  number  of  cases  diagnosticated  as  lateral  sclerosis  the  lesions 
of  amyotrophic  lateral  scleposis  have  been  found. 

Treatment.  —  The  only  treatment  is  general  tonic  treatment.  It  is 
well  to  prevent  all  strains  and  exertion  and  to  build  up  the  general 
health  in  every  way  possible.  Massage  is  to  be  used  and  electrical 
applications,  but  with  the  same  caution  against  over-fatigue  of  the 
muscles  by  these  measures  as  has  been  mentioned  in  connection  with 
the  treatment  of  chronic  anterior  poliomyelitis.  Long-continued  warm 
baths  and  douches  without  very  great  alternations  of  temperatures  are 
of  considerable  service  in  maintaining  the  nutrition.  When  the  bulbar 
symptoms  appear  the  patient  should  be  instructed  in  the  art  of  passing 
a  tube  into  the  stomach,  so  that  as  soon  as  difficulty  of  swallowing 
appears  nourishment  may  be  carried  on  in  this  manner,  and  thus  the 
danger  of  suffocation  may  be  avoided.  The  use  of  strychnine  does  not 
appear  to  retard  the  progress  of  the  disease,  and,  in  fact,  is  objection- 
able on  account  of  the  tendency  to  spastic  paraplegia. 


CHAPTER  XVI.      - 

MUSCULAR   DYSTROPHIES. 

The  Classification  of  Various  Forms.  Etiology.  Pathology.  Pseudomuscular  Hyper- 
trophy. Erb's  Juvenile  Form  of  Dystrophy.  Landouzy-Dejerine  Form  of  Dys- 
trophy. Muscular  Atrophy  of  the  Peroneal  Type.  Myotonia  Congenita,  Thomsen's 
Disease. 

There  are  certain  forms  of  paralysis  appearing,  as  a  rule,  in  early- 
life  that  are  due  to  muscular  and  not  to  nervous  disease.  These  have 
been  named  the  muscular  dystrophies.  The  muscles  appear  to  be 
affected  primarily  by  a  progressive  atrophy.  This  atrophy  involves 
the  fine  muscular  filaments  and  proceeds  slowly  until  in  the  end  even 
the  largest  muscles  may  be  reduced  to  a  small  band  of  connective 
tissue.  In  some  cases  there  occurs  a  deposit  of  fat  around  the  atro- 
phied muscle  fibres,  filling  up  the  empty  spaces  within  the  muscle  so 
that  no  apparent  atrophy  occurs.  In  some  cases  this  deposit  of  fat  is 
abnormally  great,  increasing  the  size  of  the  muscle  until  it  may  be  two 
or  three  times  its  normal  diameter,  and  producing  the  appearance  of  a 
large  muscle  where  there  is  really  an  atrophied  muscle.  This  form  is 
termed  pseudomuscular  hypertrophy.  In  other  cases  there  is  a  true 
hypertrophy  of  the  muscle  fibres.  Inasmuch  as  any  form  of  muscular 
dystrophy  produces  at  first  paresis  and  then  paralysis,  it  is  very 
natural  that  these  muscular  dystrophies  should  be  mistaken  for  spinal 
paralyses,  though  a  brief  study  of  their  characteristic  features  will 
make  it  clear  that  no  such  mistake  is  warranted. 

The  muscular  dystrophies  have  been  classified  by  Erb,^  who  has 
gathered  and  analyzed  all  the  facts  in  regard  to  this  affection,  more 
completely  than  any  other  author.  Erb  distinguished  first  between 
cases  occurring  in  infants  and  those  developing  in  adult  life,  though 
this  distinction  appears  to  be  of  little  moment,  inasmuch  as  an  analysis 
of  a  large  number  of  cases  demonstrates  that  the  disease  may  develop 
at  any  age.  It  must  be  admitted,  however,  that  a  very  large  propor- 
tion of  the  cases  appear  in  infancy  or  childhood,  and  that  when  adults 
are  affected  the  disease  usually  begins  before  the  twentieth  year.  Erb 
further  classified  these  cases,  first  into  those  in  which  there  is  an  hyper- 
trophy of  muscular  tissue,  either  a  pseudohypertrophy  or  a  true  hyper- 
trophy of  the  muscle  fibres,  and,  secondly,  those  in  which  there  is  a 
progressive  atrophy  without  any  deposit  of  fat.  This  classification, 
while  of  value  clinically,  appears  to  rest  upon  no  essential  pathological 
factor,  for  all  grades  in  the  process  are  possible.  In  some  cases  the 
deposit  of  fat  is  excessive,  causing  pseudohypertrophy  ;  in  other  cases 

iDeut.  Zoitschr.  f.  Nervenhk.,  i.,  13  and  173, 
303 


304  MUSCULAR   DYSTROPHIES. 

there  is  a  deposit  of  fat  taking  the  place  of  atrophied  muscle,  but 
without  producing  any  apparent  change  in  the  size  of  the  muscle,  and 
in  many  cases  of  extreme  atrophy  of  the  muscles  fatty  deposits  are 
found  between  the  muscle  fibres ;  hence  this  classification  does  not 
rest  upon  a  pathological  basis.  And  we  may  find  all  these  conditions 
in  different  muscles  in  the  same  patient. 

A  further  classification  has  been  based  upon  the  distribution  of  the 
affection  in  different  muscles.  Here,  unfortunately,  the  different  types 
have  been  named  after  the  observers  who  happen  to  have  first  carefully 
described  them.  Thus  we  have  the  Duchenne  type  of  pseudohyper- 
trophy, and  we  have  the  Erb  type  of  juvenile  dystrophy,  in  which  the 
muscles  about  the  shoulder-blades  and  arms  are  chiefly  affected.  We 
have  the  Sachs-Hoffman  type,  in  which  the  affection  begins  in  the 
peroneal  muscles  of  the  legs.  We  have  the  Landouzy-Dejerine  type, 
in  which  the  muscles  of  the  face  and  arms  are  affected,  and  we  have 
the  Charcot-Marie-Tooth  type,  in  which  legs  and  forearms  and  back 
are  affected,  thighs  and  arms  and  face  escaping.  It  is  true  that  the 
various  cases  observed  conform  in  many  particulars  to  one  of  these 
different  types,  but  there  is  no  essential  difference  between  them  in  the 
causation  or  in  the  pathology.  And  intermediate  types  have  been 
observed  not  conforming  to  any  of  these.  Furthermore,  it  is  found 
that  one  type  may  progress  and  gradually  merge  into  another.  It  is 
evident,  therefore,  that  these  attempts  at  classification  in  the  discus- 
sion of  muscular  dystrophies  are  not  wholly  satisfactory. 

Etiology.  —  The  causation  of  muscular  dystrophy  is  a  matter  of 
considerable  interest.  The  disease  is  undoubtedly  a  disease  of  develop- 
ment, and  from  its  marked  tendency  to  appear  in  very  early  life,  to 
attain  a  certain  status,  and  then  to  be  arrested  without  any  tendency 
to  repair,  or  else  to  progress  steadily  until  it  causes  an  entire  destruc- 
tion of  the  muscular  system,  it  seems  probable  that  it  must  be  traced 
to  an  inherent  defect  of  development  in  the  muscles  affected.  It 
seems  as  if  these  muscles  were  not  endowed  with  the  proper  vitality, 
and,  after  a  short  life,  wither  and  die. 

We  have  evidence  in  other  organs  of  the  body  of  this  difference 
between  the  life-period  of  a  special  structure  and  that  of  the  entire 
body.  Thus  the  life-period  of  the  first  and  even  of  the  second  teeth 
is  a  comparatively  short  one  as  related  to  the  life-period  of  the  indi- 
vidual. The  life-period  of  the  hair,  of  the  reproductive  system,  of 
the  eye  and  ear,  and  of  the  brain  may  be  less  than  that  of  the  entire 
individual.  We  see  in  the  study  of  optic  atrophy  and  of  auditory 
atrophy  that  there  are  some  families  in  which  these  diseases  develop, 
even  in  early  life,  without  apparent  cause,  the  only  explanation  being 
that  these  nerves  have  not  the  power  of  survival  which  other  nerves 
have.  We  recognize  the  existence  of  senile  atrophy  of  the  brain  in 
old  age,  but  we  must  admit  that  the  term  "  old  age  "  cannot  be  fixed 
within  definite  limitations,  for  some  individuals  become  old  at  the  age 
of  sixty  years,  while  others  are  young  and  vigorous  at  the  age  of 
eighty  years.     I  once  had  under  my  observation  a  lady  of  the  age  of 


PATHOLOGY.  305 

ninety-nine  years,  all  of  whose  visceral  functions  were  as  vigorous 
and  active  as  they  were  at  the  age  of  seventy,  but  whose  brain  for 
ten  years  had  been  practically  dead,  powers  of  perception  in  eye  and 
ear  being  suspended,  powers  of  memory  being  almost  obliterated,  and 
powers  of  reasoning  being  reduced  to  the  level  of  a  child  of  three 
years.  Thus,  it  is  evident  that  the  organism  as  a  whole  may  outlive 
any  one  of  several  of  its  organs,  provided  these  organs  are  not  essential 
to  life.  And  the  duration  of  vitality  of  any  organ  varies  greatly  in 
different  individuals.  This  fact  is  particularly  evident  in  regard  to 
the  reproductive  system  both  in  males  and  females,  and  it  is  equally 
apparent  in  regard  to  the  muscular  system.  For  it  is  a  matter  of 
comment  that  the  muscles  of  old  people  wither  and  become  feeble  long 
before  their  death.^ 

It  is  quite  evident  from  the  consideration  of  these  facts  that  the 
explanation  of  muscular  dystrophies  is  to  be  found  in  an  inherent 
inability  of  survival  of  certain  muscles  in  certain  individuals.  In 
other  words,  that  the  disease  is  one  of  congenital  defective  power  of 
evolution.  This  explains  to  a  certain  extent  the  frequency  with  which 
we  find  that  several  members  of  a  family  are  affected  by  this  type  of 
disease.  It  is  true  that  isolated  cases  are  not  uncommonly  met  with 
where  careful  investigation  fails  to  reveal  any  member  of  the  family  or 
any  member  of  any  collateral  branch  similarly  aflTected.  But  in  the 
majority  of  cases  it  is  found  that  if  the  patient  has  no  brother  or  sister 
similarly  diseased,  or  has  not  inherited  it  directly  from  either  parent, 
he  will  have  several  cousins  who  have  suffered,  possibly  several  second 
cousins  who  have  suffered,  and  it  is  not  uncommon  to  be  able  to  trace 
the  disease  through  four  or  even  five  generations. 

Whether  extraneous  causes,  such  as  undue  muscular  effort,  exposure 
to  cold,  the  unfavorable  effects  of  the  occurrence  of  infectious  diseases, 
falls,  and  blows,  have  anything  to  do  with  setting  up  the  disease  in 
those  who  have  an  inherent  tendency  toward  weakness  of  the  muscles 
is  a  matter  which  may  be  open  to  doubt,  though  these  factors  have 
been  mentioned  as  exerting  a  causative  agency.  ,   _ 

Pathology.  —  The  changes  found  in  the  muscles  in  this  disease  are 
those  of  a  simple  progressive  atrophy.  It  will  be  remembered  that 
the  muscle  fibre,  which  may  be  5  cm.  long  and  from  15  to  55  mm. 
broad,  has  a  striated  appearance  due  to  the  existence  of  two  types  of 
structure  within  its  substance,  one  of  which  interrupts  the  light  more 
completely  than  the  other.  Each  muscle  fibre  is  surrounded  by  a 
sheath  of  connective  tissue  (the  sarcolemma)  within  which  lie  nuclei 
or  long  oval  cells  from  which  the  muscle  fibre  itself  has  developed. 
These  muscle  fibres,  surrounded  by  the  sarcolemma,  are  really  made 
up  of  filjrils  of  very  fine  structure,  each  fibril  originating  from  a  single 
cell,  and  the  fibrils  are  separated  from  one  another  by  a  structure 
termed  the  sarcoplasma ;  hence,  under  the  microscope  a  muscle  fibre 
has  a  double  striation,  a  transverse  striation,  and  the  longitudinal 
fibrillar  structure.     These  fibres  are  gathered  into  greater  or  lesser 

20  ^  See  Gowers,  Abiotrophy,     London,  1903, 


306 


MUSCULAR    DYSTROPHIES. 


bundles,  forming  the  muscle,  and  they  are  supplied  by  nerves  whose 
terminal  filaments  spread  out  upon  the  surface  of  the  fibre  in  the  so- 
called  motor  plate.  Within  the  muscle  and  within  the  fibres  lie  oval 
bodies  called  the  muscle  spindles  which  contain  the  sensory  filaments 
through  which  the  muscular  sense  is  received. 

When  a  muscle  undergoes  atrophy  we  may  find,  first,  a  simple  reduc- 
tion in  the  calibre  of  the  individual  fibres.      (Fig.  123.)     There  is  a 


Fig.  123. 


Fig.  124. 


fi?m-rf^ 


mjmmmmii 


W^M'ji 


i^mV'^fg:'::.::'^/:^ 


^mmm^mm 


W':^ 


Atrophy  of  a  muscle,  with  increase  of  nuclei. 
X250.     (Schmaus.) 


Atrophy  of  muscle,  with  deposit  of  fat  about  the 
atrophied  fibres.     X  250.     (Schmaus.) 


thinning  in  the  entire  fibre,  which  becomes  narrower  and  shorter,  pre- 
serving until  the  end  its  striated  appearance,  though  it  becomes  pale 
and  sometimes  a  little  pigmented  by  the  deposit  of  coloring  matter. 

Secondly,  in  other  cases  there  is  a  degeneration  of  the  muscle,  ^con- 
sisting of  a  swelling  or  oedema,  followed  by  an  albuminoid  or  fatty  de- 
generation of  the  muscle  fibre,  resulting  in  the  deposit  of  small  fatty 
globules  and  granular  masses  within  the  sarcolemma.  As  the  muscle 
becomes  degenerated  the  individual  fibre  contracts,  obliterating  the 
striated  appearance,  leaving  the  muscle  with  a  homogeneous,  smooth, 
or  yellow  tint.  As  the  process  goes  on  all  trace  of  the  muscular  sub- 
stance may  vanish,  leaving  the  sarcolemma  containing  only  fatty 
globules.  Sometimes  waxy  deposits  may  occur  within  the  muscle, 
with  compression  and  obliteration  of  the  fibres,  but  this  is  not  com- 
mon in  the  dystrophies.    Occasionally  as  the  muscle  undergoes  progres- 


SYMPTOMS.  307 

sive  atrophy  its  sheath  is  filled  up  by  an  oedematous  exudation.  But 
usually  there  is  merely  an  increase  in  the  cells  of  the  sarcolemma. 
Under  some  circumstances  there  is  not  only  within  these  empty  sarco- 
lemma sheaths  a  fatty  remainder  of  the  degenerated  muscle,  but  be- 
tween the  sheaths  there  is  an  actual  deposit  of  fat  of  new  formation. 
(See  Fig.  124.)  Under  these  circumstances  not  only  is  the  space 
formerly  occupied  by  the  normal  muscle  filled  out  by  fat,  but  the  fatty 
deposit  may  increase  the  actual  diameter  of  the  muscle ;  and  if  this 
goes  on  to  any  extent  pseudohypertrophy  of  the  muscle  will  be  pre- 
sented. Along  with  the  fatty  deposit  there  is  usually  an  hypertrophy 
of  the  connective  tissue  in  the  muscle,  so  that  when  the  process  be- 
comes extreme  the  muscle  is  turned  into  a  mass  of  fat  separated  by 
trabecule  of  connective  tissue.  If,  after  the  course  of  time,  the  fat  is 
reduced  in  volume  and  is  finally  absorbed  the  muscle  will  have  as  its 
only  relic  this  connective-tissue  strand,  and  as  this  contracts  longi- 
tudinally it  will  cause  a  shortening  of  the  atrophied  muscles. 

Thirdly,  along  with  the  atrophy  we  find  occasionally  a  true  hyper- 
trophy of  individual  muscle  fibres.  The  fibrils  are  increased  by  true 
formation  of  muscle  tissue  from  their  nuclei  within  the  sarcolemma, 
and  not  only  increased  in  number,  but  also  increased  in  lateral  diam- 
eter, so  that  giant  muscle  cells  and  giant  muscle  fibres  quite  similar  in 
structure  to  a  normal  fibre  are  evident.  Any  of  these  processes  may  go 
on  alone  or  may  be  combined  with  others  in  the  process  of  muscular 
dystrophy.  The  connective-tissue  changes  which  were  for  a  time 
thought  to  be  primary  have  long  been  known  to  be  of  a  secondary 
nature,  a  hyperplasia  of  connective-tissue  within  the  muscle  occurring 
just  as  a  hyperplasia  of  neuroglia  occurs  in  the  spinal  cord  to  take  the 
place  of  atrophied  substance.  In  the  connective-tissue  near  to  the  ends 
of  the  muscle  new  formations  of  thick  tendons  may  be  seen. 

These  changes  in  the  muscles  are  found  in  all  forms  of  muscular 
atrophy  both  spinal  and  peripheral.  It  is  thought  that  in  the  dys- 
trophies the  development  of  hypertrophy  of  the  muscle  fibre  in  con- 
nection with  the  atrophy  is  a  peculiar  feature  of  the  disease.  It  is  not 
often  that  deposits  of  fat  are  found  within  the  muscles  in  the  spinal 
and  peripheral  types  of  atrophy. 

Symptoms.  —  From  the  clinical  standpoint  we  recognize  the  follow- 
ing types  of  the  disease,  the  symptoms  of  which  difier  distinctly  from 
one  another : 

1.  Pseudomuscular  hypertrophy  of  Duchenne. 

2.  Erb's  juvenile  dystrophy. 

3.  Landouzy-Dejerine  type  of  dystrophy. 

1.  Pseudomuscular  Hypertrophy. — This  disease  begins  in  children 
between  the  second  and  the  seventh  year,  and  has  often  made  consid- 
erable progress  before  it  is  brought  to  the  attention  of  the  physician. 
It  begins  by  a  gradual  increase  in  the  size  of  the  calves  of  the  legs, 
which  arc  at  first  subjects  of  pride  to  the  mother,  but  soon  awaken  her 
apprehension  because  of  being  out  of  proportion  to  the  growth  of  the 
rest  of  the  limbs  and  because  they  appear  to  be  attended  by  a  certain 


308  MUSCULAR   DYSTROPHIES. 

clumsiness  of  gait.  The  child,  in  learning  to  walk  or  in  walking,  be- 
gins to  stumble,  to  go  up  stairs  with  some  difficulty,  to  get  tired  too 
soon  after  walking  or  running,  and  to  walk  with  a  peculiar  gait. 
This  gait  is  characterized  by  a  dragging  of  the  legs,  which  appear  to 
be  raised  from  the  ground  with  difficulty,  and  in  order  to  drag  the  leg 
the  child  throws  its  body  from  side  to  side  and  raises  its  pelvis  in  the 

Fig.  125. 


Pseudohypertrophic  muscular  dystrophy.  Four  brothers,  aged  twelve,  eleven,  eight,  and  seven 
years.  The  calves  and  the  anterior  surface  of  the  thighs  are  hypertrophied.  The  muscles  of  the  back 
are  atrophied.  The  eldest  has  so  much  weakness  of  the  muscles  of  the  neck  that  he  cannot  hold  up  his 
head.     (Curschmann,  Klin.  Abbildungen.) 

act  of  walking.  As  the  disease  progresses  great  difficulty  is  experi- 
enced in  rising  from  a  chair  or  in  getting  up  from  the  floor,  and  these 
children  soon  learn  to  aid  themselves  by  the  use  of  their  hands,  which 
give  support  where  the  muscles  of  the  legs  are  weak.  When  the  dis- 
ease is  well  advanced  these  children  rise  from  a  lying  to  a  standing 
posture  in  a  peculiar  manner  that  is  characteristic  of  weakness  in  the 
muscles  of  the  calves,  quadriceps  femoris,  and  glutei  muscles  and 
muscles  of  the  back  (Figs.  126  to  130).  This  typical  method  of  getting 
on  the  feet,  together  with  the  peculiar  waddling  gait,  is  sufficient  to 
make  a  diagnosis  of  the  affection.  When  the  disease  is  fully  devel- 
oped inspection  shows  an  hypertrophy  of  the  muscles  of  the  calves 
(Figs.  131  and  132),  an  hypertrophy  of  the  anterior  muscles  of  the 
thighs  (Fig.  125),  and  an  atrophy  of  the  glutei  muscles.  An  atrophy 
of  the  muscles  of  the  back  produces  a  condition  of  forward  curvature 


P8EUD0M UBCULAR    H YFEllTKOFHY. 


309 


of  the  spine  and  causes  the  child  to  throw  his  shoulders  back  in  order 
to  preserve  iiis  centre  of  gravity.  Even  in  the  early  stage  of  the  dis- 
ease, before  the  muscles  about  the  scapulae  are  affected,  it  is  found  that 


Fig.  126. 


Pseudohypertrophic  paralysis.    The  act  of  rising.    The  child  rolls  over  on  the  face. 

if  a  plumb-line  be  dropped  from  the  scapula  it  will  clear  the  buttocks. 
This  is  not  possible  in  a  state  of  health. 

As  the  disease  advances  upward  the  arms  become  affected,  the  iu- 
fraspinati  are  the  first  to  be  hypertrophied,  so  that  the  child  appears  to 

Fig.  127. 


Pseudohypertrophic  paralysis.    The  act  of  rising.    The  child  raises  the  trunk  on  hands  and  knees 
This  position  shows  the  weakness  of  the  muscles  of  the  neck  and  the  atrophy  of  the  arms. 

have  a  very  prominent  pad  on  the  back  of  the  shoulder-blades.  The 
supraspinatus  and  deltoid  may  become  hypertrophied  also,  and  some- 
times the  triceps  and  biceps  as  well,  but  in  the  majority  of  cases 
these    muscles   atrophy   without   any   deposit   of   fat.     While   these 


810 


MVSOVLAR  DrsTROPStES. 


muscles  hypertrophy  otlier  muscles  about  the  shoulder  become  atro- 
phied; thus  the  rhomboids  and  levator  anguli  scapulse,  and  also  the 
serrati  become  atrophied  and  paralyzed,  and  hence  the  shoulder-blades 


Fig.  128. 


Pseudohypertrophic  paralysis.    The  act  of  rising.    The  child  raises  the  trunk  by  bringing  the  foct 
forward  and  extending  the  legs  at  the  knee. 


Fig.  129. 


Pseudohypertrophic  paralysis.    The  act  of  rising.    The  child  raises  the  shoulders  by  supporting  the 
weight  on  the  hand  pressed  upon  the  knee. 


PHEVDOMVHCULAR    UYPERTROPEY. 


311 


stick  out  from  the  back  like  a  pair  of  wings  (Fig.  131).  Biceps  and 
triceps  ma/tlien  in  turn  atrophy,  rendering  all  motions  at  the  shoulder- 
joint  imperfect  and  making  flexion  and  extension  of  the  elbow  very- 
weak.  As  a  rule,  the  muscles  of  the  forearms  and  of  the  hands  are 
not  affected. 

After  the  muscles   are  considerably  atrophied,  with  or  without  the 
production  of  pseudohypertrophy,  a  contraction  occurs,  causing  de- 

FiG.  130. 


Pseudohypertrophic  paralysis.    The  act  of  rising. 

formities  in  the  joints.  Thus  talipes  equinus  is  first  produced,  then  a 
permanent  flexion  of  the  legs  upon  the  thighs  and  the  thighs  upon  the 
pelvis.  Curvature  of  the  spine,  which  can  no  longer  be  corrected  by 
suspension,  develops  and  often  leads  to  lateral  curvature  by  the  un- 
equal contraction  of  the  muscles  of  the  back  upon  the  two  sides. 
Contractions  may  occur  also  in  the  arms,  producing  a  flexion  of  the 
elbow  and  interfering  with  passive  movements  of  the  shoulder-joint. 

The  electrical  contractility  of  the  muscles  is  gradually  reduced  as  the 
muscles  atrophy,  but  there  are  no  changes  of  the  nature  of  reaction  of 
degeneration.  There  are  no  fibrillary  contractions  in  the  muscles. 
Sensation  is  not  at  all  impaired.  The  reflexes  remain  normal  until  the 
muscle  becomes  too  much  atrophied  to  respond.  These  facts  enable  a 
differential  diagnosis  from  anterior  poliomyelitis  of  the  acute  or 
chronic  typo  to  be  easily  reached. 

The  course  of  the  disease  is  slow  and  progressive  through  four  to  ten 


312 


MUSCULAR    DYSTROPHIES. 


years,  by  the  end  of  which  time  the  child  is  rendered  helpless,  cannot 
stand  or  sit  up  in  bed,  or  feed  itself.  The  disease  is  not  a  fatal  one, 
but  in  the  enfeebled  invalid  state  intercurrent  diseases,  especially  of  the 
respiratory  organs,  are  the  usual  cause  of  death. 

2.  Erb's  Juvenile  Form.  —  This  type  begins,  as  a  rule,  between  the 
ages  of  twelve  and  sixteen ;  but  cases  have  been  observed  which  did 
not  develop  until  after  the  age  of  twenty.     In  this  form  of  muscular 


Fig 


Fig.  132. 


^r-^ 


Pseudohypertrophic  paralysis.  The  calves  are 
large ;  the  back  is  weak  and  curved  forward. 
Deltoids  and  triceps  are  atrophied.  Serrati  are 
weak,  hence  the  scapulae  protrude. 


Pseudohypertrophic  paralysis.  The  lordosis 
is  well  marked,  and  the  attempt  to  preserve 
the  balance  by  throwing  the  shoulders  back  is 
demonstrated. 


dystrophy  the  muscles  of  the  shoulder  are  first  affected,  the  pectorals, 
trapezii,  latissimus  dorsi,  rhomboids  and  deltoid  muscles  become  grad- 
ually hypertrophied,  but  at  the  same  time  are  weakened.  Peculiar 
deformities  of  the  chest  often  develop,  the  shoulders  being  thrown  for- 
ward and  the  scapulae  protruding.  As  the  disease  progresses  the  biceps 
and  triceps  become  atrophied,  but  the  muscles  of  the  forearms  and  hands 


ERB'S    JUVENILE    DYSTROPHY. 


313 


remain  in  their  normal  condition.  Later  on,  if  the  disease  does  not 
come  to  a  standstill,  the  muscles  of  the  back  become  atrophied,  lordosis 
appears,  with  deformities  of  the  spine  and  difficulty  in  walking.  Later 
the  glutei  and  anterior  muscles  of  the  thigh  are  affected,  either  atro- 
phied or  hypertrophied,  and  finally  the  calf  muscles  and  peronei  become 
implicated,  causing  total  paralysis  with  talipes  equinus.  In  the  majority 
of  cases  hypertrophy  is  more  marked  in  the  deltoid,  infraspinatus,  sar- 


FiG.  133. 


Pseudohypertrophic  paralysis  ;  five  years  after  the  onset.    Muscles  of  arms  and  legs  greatly  hyper- 
trophied.   Both  feet  contractured  and  in  a  position  of  talipes.     (Curschmann,  Klin.  Abbildungen. ) 

torius,  and  gastrocnemii.  It  will  be  seen  that  in  the  later  stage  of  the 
disease  the  terminal  condition  is  similar  to  that  occurring  in  pseudo- 
hypertrophy, a  fact  which  makes  it  evident  that  the  only  difference 
between  these  two  types  of  dystrophy  is  in  the  direction  of  progress  in 
the  muscles  involved. 

3.  Landouzy-Dejerine  Tsrpe.  —  In  this  type,  which  develops  usually 


314 


MUSCULAR    DYSTROPniES. 


Fig.  134 


/ 


\ 


in  early  childhood,  but  occasionally  in  adults,  the  feature  is  the  appear- 
ance of  the  muscular  atrophy  in  the  face.  It  is  true  that  both  in 
pseudomuscular  hypertrophy  and  in  Erb's  juvenile  dystrophy  the  dis- 
ease may  eventually  extend  to  the  face, 
producing  all  the  symptoms  seen  in  the 
Landouzy-Dejerine  type.  The  atrophy 
begins  in  the  orbicularis  oris  and  ex- 
tends to  the  risorii,  to  the  levator  menti, 
and  to  the  finer  muscles  of  the  face 
about  the  mouth.  The  result  is  that  the 
lips  become  weak,  cannot  be  firmly 
closed,  and  the  mouth  is  habitually 
open  and  the  lips  are  slightly  everted, 
producing  a  peculiar  facial  appearance 
that  has  been  termed  the  "tapir  mouth." 
This  form  of  paralysis  aifects  the  act 
of  speech.  Unguals  and  labials  being 
defectively  pronounced,  the  ability  to 
whistle,  or  to  purse  the  lips,  or  to  apply 
the  lips  firmly  to  a  glass  in  drinking 
being  lost.  Frequently  saliva  runs 
from  the  patient's  mouth,  as  in  facial 
palsy.  The  affection  is  bilateral.  The 
muscles  about  the  eyes,  as  a  rule,  escape, 
so  that  winking  is  not  interfered  with, 
and  the  muscles  of  mastication  and  of 
deglutition  are  not  involved.  As  time 
goes  on  in  these  cases  the  muscles  of  the 
shoulders  and  body,  and  finally  of  the 
legs,  become  involved,  and  the  terminal 
stage  of  the  disease  is  not  unlike  that  in 
the  two  types  already  described.     Fibril- 

Erb's  juvenile  dystrophy.    The  disease    ,  ,      • ,    i  •  .  i         i 

began  at  the  age  of  sixteen ;  photograph   lary  twitchmgs  are  not  present,  the  elec- 
made  at  age  of  thirty.  Supra-  and  infra-  trical  reactions  are  normal,  but  are  pro- 

spinati,  and  gastrocnemii  hypertrophied.     grCSSivcly  lost.        There  is  UO  disturbance 
rrapczn, deltoids,  biceps,  triceps, latissimi     o  .1  .i.  -n/r      1         •      i  •      1  •!• 

dorsi,  serrati  magni,  erectores  spina,  all    01     SCnSlblllty.       McchaniCal    CXCltabuity 

muscles  of  thighs,  and  glutei  are  atro-  of  the  musclcs  is  gradually  lost  as  the 

phied.      (By    permission    of   Dr.    G.    L.  i  1         1 

Walton.)  muscles  atrophy. 

While  these  three  types  of  muscular 
dystrophy  are  sufficiently  different  from  one  another  to  be  easily  recog- 
nized, it  must  be  admitted  that  many  patients  suffering  from  the  dis- 
ease present  symptoms  common  to  two  or  more  types.  Even  Erb 
admits  the  occurrence  of  transitional  forms  of  dystrophy,  and  shows 
that  the  course  of  the  disease  may  present  variations  in  various 
members  of  the  same  family  who  are  affected.  In  the  terminal  stage 
it  is  not  always  easy  to  ascertain  to  which  type  a  patient  is  to  be 
assigned. 

The  diagnosis  of  this  affection  from  bulbar  palsy  is  not  difficult, 


K. 


LANDOtlZY-DEJEniyE    DYSTROPHY. 


Sli 


because  in  dystrophy  the  tongue  and  muscles  of  swallowing  are  not 
involved.  ■/■ 

Prognosis.  —  The  prognosis  in  muscular  dystrophy  is  unfavorable. 
In  all  forms  of  the  disease  there  is  a  tendency  to  slow  progress,  to  an 


Fig.  135. 


Progressive  muscular  dystrophy,  atrophic  type  of  Landouzy-Dejerine.  Age,  nine  years ;  duration, 
seven  years.  Face,  arms,  and  legs  extremely  atrophied.  Face  expressionless;  pectorals  and  deltoids 
wholly  atrophic  ;  thighs  atrophied.    The  forearms  and  the  legs  are  normal.     (Curschmann.) 

extension  of  the  aifection  from  muscle  to  muscle  until  finally  a  state 
of  helpless  paralysis  with  extreme  emaciation  is  reached.  This,  how- 
ever, is  not  an  invariable  occurrence,  as  I  have  seen  several  patients  in 
whom  the  disease  has  come  to  a  standstill  and  who  have  remained  in  a 
stationary  condition  for  six  or  eight  years  without  being  incapacitated 
from  walking.  The  prognosis  as  far  as  life  is  concerned  is  good,  pro- 
vided complicating  diseases,  especially  of  the  respiratory  organs,  are 


316  MUSCULAR    DYSTFOPHIES. 

prevented.  These  patients  are  peculiarly  liable  to  develop  tuberculosis 
of  the  lungs  or  pneumonia  or  bronchitis  from  disability  in  respiratory 
movements.  Occasionally  the  diaphragm  is  involved  and  the  patients 
die  of  respiratory  paralysis. 

Treatment.  —  Treatment  of  muscular  dystrophy  must  be  by  general 
measures  to  support  the  health  and  nutrition  of  the  individual.  A 
country  life  in  the  open  air,  with  good  food  and  moderate  exercise,  is 
advisable,  and  the  systematic  use  of  massage  and  gymnastic  exercises, 
not  carried  to  the  point  of  fatigue,  will  often  tend  to  reduce  the  rate 
of  progress  in  the  muscular  dystrophy  and  in  some  cases  to  cause  an 
arrest  of  its  progress.  Thus  the  patients  alluded  to  in  whom  the  disease 
has  come  to  a  standstill  have  had  most  skilful  massage  for  years,  daily 
for  about  one  hour.  Electricity  has  been  used  for  the  purpose  of  exer- 
cising the  atrophied  muscles,  and  when  massage  is  impossible  it  may 
give  good  results.  When  the  contractures  have  occurred  the  question 
may  arise  of  the  propriety  of  tenotomy,  but  in  several  patients  in  whom 
I  have  had  it  done  it  has  not  afforded  any  permanent  relief,  but,  in 
fact,  has  been  attended  by  an  increase  of  the  disability.  The  appli- 
cation of  corsets  or  plaster-of-Paris  jackets  to  correct  the  deformity  in 
the  spine  is  not  to  be  advised,  because  the  lordosis  assists  the  indi- 
vidual in  preserving  his  equilibrium,  and  if  the  spine  is  held  erect  in 
its  natural  posture  the  child  falls  forward  and  is  unable  to  walk.  In- 
dividuals suffering  from  muscular  dystrophies  are  usually  such  chronic 
invalids  as  not  to  consider  the  question  of  marriage,  but  should  this 
question  arise  it  should  be  strongly  advised  against,  inasmuch  as  this 
disease  is  so  peculiarly  hereditary. 

MUSCULAR  ATROPHY  OF  THE  PERONEAL  TYPE. 

Char  cot-Marie -Tooth  Form  of  Progressive  Muscular  Atrophy. 
Progressive  Neural  Muscular  Atrophy  of  Hoffman. 

In  1886  Charcot  and  Marie  ^  in  Paris,  and  Tooth  ^  in  Cambridge, 
England,  described  independently  a  form  of  progressive  muscular 
atrophy  beginning  in  the  muscles  supplied  by  the  peroneal  nerves  and 
advancing  upward  as  high  as  the  knees  in  the  legs,  and  subsequently 
attacking  the  muscles  of  the  hands  and  forearms.  This  particular 
type  of  muscular  atrophy  has  been  recognized  by  many  authors,  espe- 
cially by  J.  Hoffman  ^  and  B.  Sachs.*  Although  it  is  an  extremely  rare 
form  of  disease,  a  sufficient  number  of  cases  can  now  be  collected  to 
establish  the  affection  as  independent  both  of  the  muscular  dystrophies 
on  the  one  hand  and  of  anterior  poliomyelitis  on  the  other. 

Etiology.  —  In  some  cases  there  appears  to  be  an  hereditary  dispo- 
sition to  the  disease.  Several  members  of  a  family  may  be  affected, 
and  in  a  few  families  it  has  been  traceable  through  two  or  three  genera- 

»  Rev.  deMdd.,  1886.  .^ 

*  Brain,  vol.  x.,  p.  243. 

»Arch.  f.  Psych.,  Bd.  xx.,  S.  560,  and  Deut.  Zeitschr.  f.  Nerven.,  Bd.  i.,  S.  95, 

*  New  York  Medical  Journal,  December,  1888.     Brain,  1890. 


MUSCULAR   ATROPHY    OF    TEE    PERONEAL    TYPE. 


317 


tions.  In  many  patients,  however,  no  history  of  inheritance  can  be 
obtained.  ^  No  etiological  factors  have  been  established,  and  syphilis 
does  not  appear  to  be  a  cause.  In  a  few  cases  it  has  been  preceded 
by  some  form  of  infectious  disease.  The  disease  uniformly  appears  in 
young  persons  before  the  age  of  twenty  years. 

Pathology.  —  The  cases  first  recorded  were  supposed  to  be  of  the 
nature  of  muscular  dystrophy,  a  progressive  atrophy  of  the  muscles 
of  the  legs  and  forearms  and  of  the  feet  and  hands  being  the  only 

Fig.  136. 


Charcot-Marie-Tooth  disease.    Atrophy  of  the  legs  below  the  knees  and  of  the  arms  below  the 

elbows. 

lesion  noticed.  But  in  1889  Hoffman  discovered  an  atrophic  neuritis 
in  the  peroneal  nerves  in  addition  to  the  muscular  atrophy,  and  from 
that  date  the  disease  has  been  ascribed  to  a  degenerative  neuritis. 
Bernhardt/  in  1893,  recorded  a  case  in  which  in  addition  to  the 
atrophy  of  the  peroneal  nerves  there  were  found  some  changes  of  a 
sclerotic  character  in  the  posterior  columns  of  the  spinal  cord ;  and 

'Virchow's  Archiv,  Bd,  cxxxiii,,  S.  259. 


818 


MUSCULAR    DYSTROPHIES. 


Fjg.  137. 


Siemerling/  in  1899,  after  a  study  of  all  the  cases  upon  record,  reached 
the  conclusion  that  the  lesion  of  the  disease  consists  in  a  degeneration 
both  of  the  muscles,  nerves,  and  posterior  columns  of  the  spinal  cord, 
a  lesion  which  involves  the  entire  sensory  element  both  in  its  periph- 
eral and  central  prolongations  as  well  as  the  motor  fibres  of  the  nerves. 
He  has  found  the  posterior  spinal  ganglion  aifected,  and  he  has  also 
found  a  sclerosis  in  a  portion  of  the  lateral  columns  of  the  cord  not 

involving  the  pyramidal  tracts. 
Siemerling  is  inclined  to  abandon 
the  view  that  the  disease  begins  in 
the  peripheral  nerves,  and  is  inclined 
to  ascribe  it  to  a  central  lesion  in 
the  posterior  horns  of  the  spinal 
cord,  but  the  exact  pathology  is  still 
a  matter  of  uncertainty. 

Symptoms.  —  The  disease  begins 
with  a  gradually  increasing  atrophy 
and  weakness  of  the  intrinsic  mus- 
cles of  the  feet  and  of  the  long 
peroneal  muscles  on  the  outer  side 
of  the  legs.  The  weakness  and 
atrophy  then  appear  in  the  anterior 
tibial  muscles  and  extensor  com- 
munis digitorum  and  in  the  poste- 
rior tibial  muscles,  so  that  after  a 
slow  increase  during  two  or  three 
years  the  patient  is  almost  inca- 
pacitated from  walking.  The  foot 
falls  in  walking,  and  hence  the 
gait  resembles  the  stepping  gait  of 
multiple  neuritis.  Occasionally  the 
vastus  iuternus  in  the  thigh  is  at- 
rophied and  adduction  of  the  knee 
becomes  somewhat  difficult.  The 
affection  of  this  muscle  somewhat 
impairs  the  gait,  allowing  the  knee 
to  fall  outward ;  hence  the  patients 
place  the  feet  somewhat  too  far 
from  one  another  in  walking.  (See 
Fig.  136.)  The  muscles  which  are 
Charcot-Marie-Tooth  disease.   Atrophy  of  the  affccted  show  fibrillary  Contractions, 

legs  and  drop-feet,  and  atrophy  of  the  hands.        thcV    lose    their    reflcX    activity    and 

mechanical  excitability,  and  there  is 
a  progressive  diminution  of  the  electrical  excitability  for  both  currents. 
In  many  cases  a  complete  reaction  of  degeneration  appears  in  an  early 
stage.  As  a  rule  the  weakness  and  atrophy  do  not  extend  above  the 
knees,  and  hence  the  contrast  in  the  appearance  of  the  thigh  and  leg 

lArch.  I  Psjch.,  Bd.  xxxi.,  S.  105, 


MUSCULAR    ATROPHY    OF    THE    PERONEAL    TYPE.  319 

is  very  marked  in  these  patients.  In  all  cases  club-feet  finally  de- 
velop, and^bilateral  club-feet  should  always  suggest  this  disease.  The 
symptoms  appear  in  the  arms  after  the  legs  have  been  affected  for  some 
months  or  years.  Occasionally,  however,  the  hands  and  arms  are 
affected  from  the  beginning.  There  is  a  gradual  atrophy  and  weakness 
of  the  intrinsic  muscles  of  the  hands,  of  the  thenar  and  hypothenar 
groups,  and  also  of  the  forearms,  and  as  these  become  weaker  ab- 
normal positions  of  the  fingers  are  assumed,  giving  rise  to  deformities 
of  the  nature  of  claw-hand.  The  muscles  both  on  the  front  and  back 
of  the  forearm  are  affected,  and  fibrillary  contractures  and  diminution 
of  electrical  contractility  with  reaction  of  degeneration  are  found. 
The  disease  may  come  to  a  standstill  at  this  point,  as  in  the  patient 
whose  photograph  is  shown  in  Fig.  136,  and  there  may  appear  to  be  no 
progressive  increase  in  the  symptoms  for  a  long  time.  In  other  cases, 
however,  the  disease  makes  more  rapid  progress,  the  atrophy  and 
paralysis  involving  the  thighs  and  the  arms,  the  muscles  of  the  body 
and  neck,  and  even  the  face,  and  the  patient  is  gradually  reduced  to  a 
skeleton,  and  dies  from  exhaustion. 

There  are  a  few  sensory  disturbances.  In  some  cases  a  slight  numb- 
ness with  diminution  in  the  pain  sense  along  the  outer  side  of  the  legs 
is  noticed,  and  in  other  cases  complete  anaesthesia  in  the  distribution  of 
the  peroneal  nerves  has  been  found.  The  paralyzed  limbs  are  usually 
cold  and  have  a  tendency  to  cyanosis  especially  in  cold  weather. 

The  duration  of  the  disease  is  uncertain,  for  many  cases  appear  to  be 
arrested,  and  the  patients  live  their  lives  with  a  condition  of  atrophy 
in  arms  and  legs  and  do  not  die  of  the  affection.  In  other  cases  where 
the  progress  of  the  atrophy  is  more  rapid  they  die  of  some  intercurrent 
disease. 

Prognosis  for  recovery  is  always  unfavorable. 

Treatment,  —  A  general  course  of  tonic  treatment  with  baths,  mas- 
sage, and  electricity  to  the  atrophied  muscles  appears  to  have  some 
effect  in  lessening  the  rapidity  of  the  atrophy,  but  does  not  appear  to 
arrest  the  progress  of  the  affection. 


CHAPTER  XVII. 

SYRINGOMYELIA   OR  GLIOSIS  SPINALIS. 

History.  —  Syringomyelia  {aupcyq,  tube,  hollow ;  //'J£/oc,  marrow) 
or  gliosis  spinalis  is  a  disease  of  the  spinal  cord  characterized  by  the 
production  of  a  cavity  within  the  cord  of  varying  length.  The  name 
was  given  by  Ollivier  in  1824,  but  the  condition  was  first  described 
by  Etienne  in  1546  and  is  mentioned  by  numerous  writers  on  anatomy 
from  that  time  onward.  Portal  (1804)  was  probably  the  first  to  ascribe 
a  form  of  spinal  paralysis  to  this  lesion  on  the  basis  of  four  cases  which 
he  observed.  From  his  time,  however,  until  1860  the  condition,  though 
occasionally  noted  by  pathologists,  excited  no  interest.  Then  with  the 
beginning  of  pathological  study  of  the  nervous  system  various  hypo- 
theses were  proposed  to  explain  the  existence  of  cavities  within  the 
cord,  and  Lockhart  Clarke,  Vulpian,  Hallopeau,  Charcot  and  JoflFroy, 
Leyden,  Schultze,^  and  Kahler^  made  important  contributions  to  the 
subject.  In  the  monographs  of  Roth,  Wichmann,  and  Anna  Baumler 
(1889)  over  100  cases  with  autopsies  were  collected  and  analyzed. 
The  study  of  these  cases  from  a  pathological  standpoint  was  soon  fol- 
lowed by  their  analysis  from  the  clinical  side,  and  in  1887  Schultze^ 
and  Kahler*  established  the  possibility  of  diagnosticating  this  condition 
during  life.  Their  statements  have  been  confirmed  by  clinical  observers 
all  over  the  world.  In  a  monograph  by  Schlesinger^  526  references 
to  published  cases  or  discussions  of  the  subject  are  given.  Dimitrofi"® 
has  recently  added  to  this  literature. 

Pathology.  —  The  post-mortem  appearances  in  a  case  of  syringo- 
myelia are  very  characteristic.  The  spinal  meninges  are  normal.  The 
contour  of  the  cord  is  sometimes  irregular,  owing  to  a  bulging  at  some 
places  or  a  retraction  at  other  places,  or  it  appears  at  places  flattened ; 
sometimes  it  is  not  altered.  Fluctuation  may  be  detected  by  palpation. 
Usually  a  rupture  occurs  in  the  process  of  removal  of  the  cord,  and 
the  fluid,  a  clear  serum,  runs  out,  leaving  the  cord  partly  collapsed. 
It  is  then  evident  that  there  is  a  long  cavity  within  the  cord,  usually 
near  the  central  canal,  but  sometimes  so  extensive  as  in  a  cross-section 
to  leave  merely  a  thin  ring  or  wall  of  cord  tissue.  Sections  of  the  cord 
at  various  levels  will  demonstrate  that  this  cavity  extends  for  some  dis- 
tance through  the  cord,  and  that  it  varies  in  size  and  shape  at  different 

*  Schultze,  Virchow's  Archiv,  Bd.  Ixxxvii.  and  civ. 

^Kahler,  Vierteljahi-schrift  fur  Pract.  Heilk.,  1879.     Arch.  f.  Psych.,  Bd.  x, 

3 Schultze,  Zeitschr.  fiir  klin.  Med.,  Bd.  xiii.,  1887. 

*Kahler,  Prager  med.  Woch.,  1888,  Nos.  6  and  7. 

^  Schlesinger,  Die  Syringomyelic,  1895,  Leipzig. 

«Arcb,  f.  Psych.,  Bd.  xxxv.,  S.  42. 

320 


PATHOLOGY. 


321 


levels.  The  usual  situation  of  the  cavity  is  in  the  lower  cervical  and 
dorsal  regies.  In  some  cases  it  is  short,  not  involving  more  than  five 
or  six  segments ;  in  others  it  is  long,  extending  through  the  entire 
length  of  the  cord  and  upward  into  the  medulla  and  pons.  Sometimes 
two  or  three  separate  cavities  have  been  found  at  different  levels.  All 
possible  variations  have  been  observed  in  different  cases.  In  some 
cases  a  tumor  has  been  found  on  one  side  or  within  the  wall  of  the 
cavity. 

When  the  cord  is  hardened,  cut,  stained,  and  examined  microscopi- 
cally it  presents  certain  characteristic  appearances.     The  cavity  may 


Fig.  138. 


Syringomyelia.  Cavity  in  the  central  gray  matter  of  the  left  half,  with  numerous  diyerticula 
Walls  of  cavity  .formed  hy  gliomatous  tissue  infiltrating  the  spinal  cord.  Weigert  stain.  (W.  A. 
Turner.) 

be  of  auy  size  or  of  any  shape,  but  lies  chiefly  near  the  central  canal, 
behind  the  anterior  commissure,  or  in  the  posterior  central  gray  matter, 
or  in  a  posterior  horn,  or  in  both  horns  of  the  cord.  In  some  cases  it 
invades  the  central  gray  matter  and  the  anterior  horn  or  horns,  but  it 
is  rarely  symmetrical  in  its  invasion  of  the  cord  tissue  on  the  two  sides. 
In  some  cases  the  gray  matter  is  entirely  replaced  by  the  cavity.  lu 
other  cases  the  cavity  has  invaded  the  white  columns  of  one  or  both 
sides.  The  posterior  columns  are  more  frequently  invaded  than  the 
lateral  or  anterior.  In  the  most  extreme  cases  it  appears  as  if  all  the 
cord  tissue  had  been  destroyed.  The  cavity  is  then  surrounded  by  a 
thin  wall  forming  its  sac,  and  no  trace  of  gray  or  white  substance 
remains.  The  wall  of  the  cavity  is  smooth,  but  here  and  ihere  papil- 
lary projections  occur  upon  it. 
21 


322  SYRINGOMYELIA    OR    GLIO^If?    SPINALIS. 

The  cavity  is  usually  surrounded  by  a  zone  of  thick  neuroglia  tissue 
which  stains  deeply  with  carmine  and  hematoxylin,  is  unstained  in  the 
Weigert  hematoxylin  stain,  is  deeply  stained  by  the  Weigert  neuroglia 
stain,  and  by  the  Golgi  stains.  The  thickness  of  this  neuroglia  wall 
varies  in  different  cases.  Its  structure  is  most  dense  near  the  cavity ; 
but  it  is  thinner  in  the  adjacent  parts  and  fades  away  into  tlie  normal 
cord,  not  having  as  a  rule  a  sharp  boundary.  (See  Fig.  138.)  Under 
a  high  power  of  the  microscope  it  is  seen  to  be  made  up  of  fine  fibres, 
of  nuclei,  and  of  small  and  large  neuroglia  cells,  a  few  of  which  are 
seen  in  some  cases  to  be  in  a  state  of  vacuolization  and  progressive 

Fig.  139. 


Syringomyelia.    The  cavity  has  taken  the  place  of  the  central  gray  matter,  and  has  invaded  both 
posterior  columns. 

liquefaction  and  to  be  breaking  down.  A  fine  filamentous  network, 
containing  spider  cells  and  spindle-shaped  cells  with  long  processes 
and  many  nuclei,  extends  outward  into  the  nerve  tissue  —  the  appear- 
ance being  that  of  a  partial  infiltration  of  the  normal  cord  by  glioma- 
tous  elements,  the  degree  of  which  is  greatest  near  to  the  wall  of  the 
cavity.  Such  an  infiltration  of  the  cord  with  cells  is  also  found  in  the 
segments  above  and  below  the  limits  of  the  cavity,  especially  about 
the  central  canal.  The  cavity  frequently  occupies  the  place  usually 
taken  by  the  central  canal.  Sometimes  it  is  seen  to  communicate  with 
the  remains  of  the  central  canal,  and  then  some  epithelial  elements  may 
be  found  in  its  wall.  In  other  cases  the  canal  is  pushed  to  one  side 
and  lies  in  the  wall  of  the  cavity.  In  a  few  sections  there  may  appear 
to  be  two  cavities  side  by  side ;  but  a  careful  examination  of  sections 
above  or  below  will  show  that  one  of  these  is  really  a  diverticulum  from 


PATHOLOGY.  323 

the  main  cavity.  The  cavity  itself  is  never  entirely  lined  by  cylin- 
drical epiUielium ;  but  in  a  few  cases  one  side  of  it  may  be  so  lined, 
and  in  these  there  is  a  manifest  absorption  of  the  original  central  canal 
into  the  new  cavity,  with  more  or  less  proliferation  of  the  lining  epithe- 
lium. A  few  cases  have  been  described  in  which  a  true  glioma  or 
sarcoma  filled  the  cavity,  being  an  evident  outgrowth  from  its  wall.^ 
Changes  in  the  bloodvessels  of  the  cord  are  sometimes  observed.  There 
are  very  few  capillaries  to  be  seen  in  the  wall  of  the  cavity ;  but  out- 
side of  it,  in  the  adjacent  parts  of  the  cord  where  the  infiltration  of 
small  cells  and  nuclei  is  seen  vessels  are  more  numerous  than  normal, 
their  calibre  is  larger,  and  they  are  more  tortuous  than  usual.  In  some 
cases  distinct  thickening  of  their  walls  has  been  noticed.  Capillary 
hemorrhages  are  often  found  within  the  gliomatous  structure. 

Various  hypotheses  have  been  proposed  to  explain  the  conditions 
described.     They  may  be  briefly  summarized  as  follows  : 

1.  It  has  been  supposed  that  syringomyelia  always  originates  in  a 
congenital  defect  in  the  development  of  the  spinal  cord.  It  has  been 
thought  that  the  central  canal  of  the  cord  may  be  unduly  distended 
during  foetal  life  and  early  infancy  by  fluid,  thus  remaining  as  an  un- 
usually large  cavity  within  the  cord,  around  which  cavity  there  subse- 
quently develops  a  proliferation  of  the  embryonal  epiblastic  elements 
or  a  thickening  of  the  normal  glia  tissue,  which  is  known  to  be  more 
abundant  in  this  situation  than  elsewhere  in  the  cord.  Such  a  cavity 
is  lined  by  epithelium  and  is  described  by  many  pathologists  under  the 
name  "  hydromyelus."  But  some  believe  that  hydromyelus  may  pass 
into  syringomyelia  by  the  proliferation  of  glia  cells,  the  invasion  of 
the  normal  tissue,  and  its  subsequent  breaking  down.  They  thus  ex- 
plain the  admitted  fact  that  the  degree  in  which  the  cavity  in  syrin- 
gomyelia is  lined  by  cylindrical  epithelium  varies.  Others  hold  that  in 
the  closure  of  the  central  canal  during  embryonal  development  a 
portion  of  it  is  shut  oif  from  the  main  canal  so  as  to  leave  a  subsidiary 
canal  in  the  posterior  septum  of  the  cord,  lined  with  epithelium  and 
surrounded,  as  is  the  normal  central  canal,  by  epiblastic  tissue  which 
subsequently  proliferates,  constituting  a  neuroglia  hyperplasia,  and  then 
breaking  down.  Those  who  hold  this  opinion,  therefore,  ascribe  all 
cases  of  syringomyelia  to  a  congenital  malformation  of  the  cord  and 
believe  that  the  cavity  of  syringomyelia  has  a  necessary  relation  to  the 
normal  central  canal  and  usually  communicates  with  it. 

2.  Another  view  of  the  disease  is  that  the  normal  glia  structure  of 
the  spinal  cord,  which  is  thickest  about  the  central  canal,  undergoes 
a  proliferation  from  some  unknown  cause  —  possibly  an  irritant  poison 
in  the  fluid  of  the  central  canal  ;^  that  this  gliomatous  new  structure 
extends  outward  into  the  adjacent  tissue,  both  into  the  gray  and  white 
matter,  and  subsequently  breaks  down  in  its  center,  the  cells  becoming 
liquefied  and  disintegrated ;  thus  a  cavity  originates  within  a  gliomatous 
mass.     This  cavity  at  its  origin  has  no  necessary  relation  to  the  central 

'  Van  Gieson,  Journal  of  Nervous  and  Mental  Disease,  July,  1889. 
^lijtbea  and  Manicatide,  Archives  des  Sci.  Med.  de  Bucharest,  May,  1896, 


824 


SYRINGOMYELIA    OR    GLIOSIS    SPINALIS. 
Fig.  140. 


A/lhTlor  llullL- 

A. 


Ceniru'l  Canal 


Cenlral  cavilies  CQiised  dy 
breaking  (Itm''n  ofUssice. 


Softened  area- 


Sufi  en  ed   cord 


Stem  of  Cliomatosis 


Glioma  of  the  spiual  cord,  with  formation  of  cavities  within  it.  AR,  anterior  nerve  roots- 
T,  tumor;  PR,  posterior  nerve  roots;  EC,  epithelial  lined  cavities  within  the  tumor.  (Hudson, 
American  Journal  of  the  Medical  Sciences,  cxvii.,  648.) 


PATHOLOGY.  825 

canal ;  but  inasmuch  as  the  gliomatous  proliferation  begins,  as  a  rule, 
near  to  th«r  canal,  the  cavity  usually  breaks  into  the  central  canal  and 
thus  makes  a  communication  with  it.  The  wall  of  the  cavity  is  de- 
scribed as  consisting  of  hyperplastic  neuroglia  with  larger  and  smaller 
branching  neuroglia  cells,  and  small  spheroidal  cells,  and  oval  cells 
lying  in  a  network  of  fibres,  at  places  closely  packed  together,  at 
places  loosely  arranged,  so  that  it  has  a  porous  meshwork  structure. 
This  forms  a  sort  of  limiting  membrane  for  the  cavity,  but  beyond  it 
within  the  nervous  tissue  there  is  an  infiltration  of  glia  cells.  These 
cells  are  seen  to  be  in  a  state  of  liquefactive  degeneration  both  in  the 
wall  of  the  cavity  and  elsewhere.  In  some  cells  the  nucleus  is  attached 
to  or  surrounded  by  the  homogeneous  remains  of  the  cell  body. 
Some  cells  are  converted  into  sacs  of  fluid.  Thus  there  is  a  manifest 
tendency  in  the  glia  cells  to  break  down,  and  the  cavity  is  the  result 
of  such  disintegration.  Those  who  hold  this  opinion  have  named  the 
disease  spinal  gliosis,  believing  the  glia  proliferation  to  be  the  essential 
factor  in  the  pathology.  Some  consider  this  an  inflammatory  process,^ 
others^  deny  anything  more  than  a  simple  hyperplasia. 

Investigations  of  Weigert^  upon  the  structure  of  neuroglia  seem  to 
establish  that  new  formations  of  glia  may  be  either  cellular  in  structure 
or  fibrous  in  structure.  If  cellular,  the  new  formation  is  a  true 
glioma,  such  as  is  found  in  tumors  of  the  brain  or  spinal  cord,  and  in 
such  a  glioma  fibres  are  few.  Weigert  calls  attention  to  the  fact  that 
this  is  not  the  structure  of  the  glia  tissue  about  the  cavity  of  syrin- 
gomyelia, but  that  the  neuroglia  found  around  this  cavity  consists 
almost  exclusively  of  glia  fibres  with  few  cells ;  and  that  these  fibres, 
though  extending  in  all  directions,  are  chiefly  vertical  in  their  course. 
Miura  also  has  shown  the  sharp  contrast  between  ordinary  glioma, 
even  glioma  containing  a  cavity,  and  the  gliomatous  condition  of  the 
cord  in  syringomyelia.  Weigert  holds  that  the  neuroglia  is  merely  a 
substance  produced  by  nature  to  take  the  place  of  nerve  tissue  which 
has  been  destroyed,  and  that  its  proliferation  is  always  a  sign  that  the 
nerve  tissue  has  primarily  disintegrated.  Such  destruction  of  nerve 
tissue  would,  therefore,  according  to  his  view,  precede  the  formation  of 
gliomatous  tissue ;  hence  he  wholly  discards  the  hypothesis  of  syrin- 
gomyelia to  which  the  name  spinal  gliosis  has  been  applied.  Weigert 
says:  "Many  authors  believe  that  the  essential  lesion  in  syringomyelia 
is  the  formation  of  a  tumor  followed  by  softening  and  the  formation  of 
a  cavity.  It  is  admitted  that  there  is  a  growth  of  neuroglia  of  the 
typical  fibre  type  about  the  cavity.  But  this  fibre  mass,  devoid  of 
cells,  does  not  resemble  a  glioma  and  there  is  no  reason  to  believe 
from  the  mere  presence  of  neuroglia  that  the  cavity  is  not  a  congenital 
or  acquired  abnormality  of  the  central  canal.  There  is  a  thick  cluster 
of  neuroglia  fibres  normally  about  the  canal.  By  the  pressure  in  this 
enlarged  canal   the  nervous  tissue  may  be  destroyed,   and   hence  a 

'Miura,  Ziegler's  Beitrage  zur  path.  Anat.,  xi.,  91. 

^Hchnhze,  Zeitschr.  fiir  klin.  Med.,  xiii.  _ 

^Weigert,  Beitrage  zur  Kenntniss  der  normalen  menschlichen  Neuroglia.  Frank- 
fort, 181J5. 


326  SYRINGOMYELIA    OR    GLIOSIS   SPINALIS. 

growth  of  neuroglia  fostered.  If  the  pressure  increases  the  neuroglia 
may  also  be  destroyed,  and  in  its  place  about  the  cavity  a  hyaline  form- 
less mass  may  remain.  The  gliosis  is  not  the  essential  feature,  it  is  only 
a  secondary  result."  Turner,'  however,  has  described  a  case  in  which 
there  is  a  gradual  and  direction  transition  between  a  true  glioma  and  a 
gliomatous  infiltration  of  the  cord  with  the  production  of  a  cavity. 

3.  Many  authors  have  observed,  subsequently  to  disease  of  the  spinal 
arteries,  the  formation  of  cavities  in  the  cord  independent  in  their  situ- 
ation of  the  central  canal.  Thus  Miiller  and  Medin  have  seen  a  cavity 
in  the  gray  matter  of  the  cord  with  walls  of  normal  nerve  tissue  with 
no  signs  of  inflammatory  disease  or  of  proliferation  in  the  glia,  the 
size  of  the  cavity  corresponding  in  situation  to  the  degree  of  endarteritis 
in  the  spinal  vessels  and  having  no  definite  relation  to  the  central 
canal.  Wieting  has  described  a  cord  containing  numerous  cavities 
due  entirely  to  the  low  nutrition  of  the  nerve  tissues,  and  consequent 
necrosis  from  disease  of  the  spinal  arteries  in  connection  with  men- 
ingo-myelitis.  It  has  been  thought  by  Kronthal  that  lymph  stasis 
within  the  cord,  and  consequent  necrosis,  produced  by  transverse  com- 
pression of  the  cord,  may  lead  to  the  formation  of  cavities  which  may 
or  may  not  communicate  with  the  central  canal ;  but  this  surmise  is 
doubtful,  since  compression  by  tumors  or  after  Pott's  disease  is  not 
found  to  cause  cavities.  The  supposition  that  a  cavity  in  the  cord 
may  be  due  to  a  diseased  condition  of  the  bloodvessels  seems  proven 
in  some  cases ;  but  such  cavities  do  not  resemble  those  of  syringomyelia, 
and  the  suggestion  finds  little  support  in  Weigert's  hypothesis  regard- 
ing the  function  of  neuroglia.  For  if  this  hypothesis  be  correct  a 
neuroglia  growth  would  occur  to  replace  the  disintegrated  nerve  tissue, 
and  in  the  attempt  of  nature  to  fill  up  the  empty  space  would  be  thick- 
est about  the  cavity.  Necrotic  cavities,  however,  rarely  have  a  well- 
marked  wall. 

4.  Van  Gieson  has  recently  described  a  condition  which  he  calls 
hemato-myelo-porus,  of  perforating  hemorrhage  in  the  cord,  with  the 
production  of  a  long,  narrow  cavity.  Such  a  cavity  is  occasionally 
surrounded  by  thickened  glia  tissue.  Van  Gieson  shows  that  some 
cases  which  have  been  described  as  syringomyelia  have  really  been  old 
cases  of  hemorrhage.  Turner  and  Mackintosh  point  out  that  the  pres- 
ence of  a  fibrin-like  material,  which  they  describe  in  some  of  the  cav- 
ities in  several  cases  of  gliomatosis  of  the  cord,  suggests  that  hemor- 
rhage assists  in  the  formation  of  these  spaces.  P.  Bailey^  has  shown 
in  his  studies  of  spinal  injuries  that  a  symptom  complex  exactly  like 
that  of  syringomyelia  may  develop  rather  rapidly  after  internal  spinal 
hemorrhage.  The  hemorrhage  may  be  single  and  destroy  the  central 
gray  matter  of  the  cord  for  some  length,  or  there  may  be  multiple 
small  hemorrhages  at  various  levels.  Begg^  has  recently  reported 
such  a  case. 

^  Turner  and  Mackintosh,  Brain,  1896,  Pt.  Ixxv. 
^Accident  and  Injury.     D.  Appleton  &  Co.,  1906. 
3  Lancet,  July  16,  1904,  p.  145. 


^tMPfOM^.  327 

5.  Lastly,  there  is  not  wanting  an  hypothesis  which  would  trace  it 
to  bacteriajl  infection.  Prus/  from  a  careful  study  of  Morvan's  disease, 
reached  the  conclusion,  which  several  authors  had  already  announced, 
that  Morvan's  disease  and  syringomyelia  are  identical.  He  calls  atten- 
tion, however,  to  the  fact  that  Zambaco  maintains  that  it  is  identical 
with  lepra  ansesthetica,  the  bacillus  of  which  can  be  recognized.  And 
he  appears  to  be  willing  to  admit  that  the  three  diseases  are,  in  fact, 
due  to  the  same  cause,  namely,  an  infection  of  the  nervous  system  by 
a  germ  which,  in  the  lighter  forms,  attacks  the  peripheral  nerves  only 
and  in  the  more  severe  forms  attacks  the  spinal  cord.  In  this  view 
the  neuroglia  formation  is  set  up  by  the  irritation  of  the  bacillus,  and 
the  cavity  is  due  to  the  disintegration  of  the  gliomatous  substance. 
This  hypothesis  is  strongly  combated  by  Babes,  who  has  observed  six 
cases  of  lepra  in  which  the  bacilli  were  found  in  the  cells  of  the  cord, 
but  in  which  there  was  no  lesion  resembling  that  of  syringomyelia. 

It  seems  evident,  therefore,  from  a  review  of  these  various  hypoth- 
eses, that  cavities  may  be  formed  within  the  spinal  cord  under  vary- 
ing circumstances  and  by  various  pathological  processes.  First,  from 
congenital  defects  of  development;  secondly,  by  a  disintegration  sub- 
sequent to  a  neuroglia  proliferation  either  of  inflammatory  origin  or 
of  spontaneous  occurrence ;  thirdly,  as  the  result  of  retrograde  meta- 
morphosis of  tissue,  the  nutrition  of  which  is  impaired  by  obstruc- 
tion to  the  circulation ;  fourthly,  by  actual  destruction  of  the  cord  by 
hemorrhage. 

The  time  has  not  yet  come  to  establish  conclusively  any  one  of  these 
views  of  the  origin  of  syringomyelia.  In  fact,  until  cases  are  observed 
at  the  outset  of  the  disease,  as  well  as  after  a  long  duration,  no  basis 
for  a  conclusion  can  be  established. 

When  the  cavity  has  existed  for  some  time  it  is  not  uncommon  to 
find  evidences  of  ascending  and  descending  degeneration  in  the  columns 
of  the  cord,  which  are  secondary  to  pressure  or  to  the  destruction  of 
tissue  at  its  point  of  maximum  extent.  Degeneration  in  the  motor 
nerves  and  atrophy  of  the  muscle  fibres  are  also  parts  of  the  lesion  in 
this  disease.  The  various  trophic  disturbances  in  the  bones  and  skin 
also  require  mention. 

Symptoms.  —  The  diagnosis  of  syringomyelia  rests  upon  the  pres- 
ence of  three  characteristic  symptoms  which,  in  the  majority  of  cases, 
are  present  together.  The  existence  of  one  of  these  symptoms  alone 
should  excite  suspicion  of  the  possibility  of  the  disease  being  present. 
The  presence  of  any  two  of  them  make  the  diagnosis  very  probable. 
These  symptoms  are,  first,  a  loss  of  the  sensations  of  pain  and  of  tem- 
perature in  any  part  of  the  body,  tactile  sense  being  preserved  in  the 
analgesic  area;  secondly,  trophic  disturbances  in  the  skin,  muscles, 
bones,  or  joints;  thirdly,  progressive  muscular  atrophy  attended  by 
paralysis. 

In  addition  to  these  symptoms  there  may  be  (a)  a  spastic  paraplegia, 
or  (6)  disturbance  of  tactile  sense  with  pain,  or  (c)  the  general  symp- 

»Arch.  f.  Psych.,  Bd.  xxvii.,  S.  771. 


328  SYRINGOMYELIA    OR    GLIOSIS    SPINALIS. 

toms  of  transverse  myelitis  in  case  the  disease  invade  respectively  the 
(«)  lateral,  or  (/9)  posterior  columns  of  the  cord,  or  (j)  its  entire  area. 
Such  an  extension  is  not  uncommon,  and  hence  these  symptoms  must 
be  considered  as  a  frequent  complication. 

The  distribution  of  the  characteristic  symptoms  of  the  disease  will 
depend  entirely  upon  the  extent  of  the  lesion  in  the  cord.  As  this 
lesion  usually  begins  in  the  cervical  segments  the  symptoms  almost 
always  appear  in  the  hands.  If  the  lesion  be  limited  to  one  or  two 
segments  of  the  cord  the  symptoms  will  be  very  limited ;  but  if  it 
extend  throughout  the  entire  length  of  the  cord  and  upward  through 
the  medulla  and  pons  to  the  crus,  the  symptoms  will  be  widespread 
and  will  involve  the  cranial  nerves.  The  course  of  the  disease  is  a 
very  chronic  one,  the  symptoms  coming  on  slowly  at  any  age,  and 
often  reaching  a  certain  point  and  remaining  stationary  for  years,  the 
life  of  the  patient  being  ended,  as  a  rule,  by  some  intercurrent  disease, 
though  occasionally  sudden  death  is  caused  by  the  rupture  of  the 
cavity. 

The  disturbance  of  sensation,  called  by  Charcot  dissociated  anaes- 
thesia, is  the  chief  characteristic  of  the  disease.  It  is  a  symptom 
which  is  frequently  unknown  to  the  patient  until  it  is  demonstrated  by 
the  physician,  although  occasionally  among  the  working  classes,  who  are 
much  exposed  to  injuries,  the  patient  may  have  noticed  that  such 
injuries,  especially  burns,  were  not  attended  by  pain.  It  is  found 
upon  examination  of  these  persons  that  pricking,  or  cutting,  or  burning, 
or  freezing  of  the  aifected  area  is  not  attended  by  sensations  of  pain,  or 
of  heat  or  cold,  though  the  sense  of  touch  is  preserved.  The  sense  of 
heat  may  be  impaired  when  that  of  cold  remains,  or  conversely.  The 
sense  of  pain  is  a  great  protection  to  the  body,  giving  warning  of 
injury  and  assuring  care  and  rest  of  the  part ;  hence  its  absence 
exposes  these  patients  to  the  risks  of  serious  affections  of  the  skin  and 
joints,  the  consequence  of  neglect  of  small  pathological  processes  at 
their  start.  The  sense  of  touch  is  not  often  affected  at  all,  although  in 
cases  where  the  cavity  progresses  to  a  considerable  size  and  invades 
the  posterior  columns  of  the  cord  it  may  become  somewhat  blunted. 
The  muscular  sense  appears  to  be  preserved,  excepting  in  this  last 
class  of  cases.  It  is  from  these  phenomena  of  dissociated  anaesthesia 
that  the  conclusion  has  been  reached  that  the  paths  of  sensations  of 
pain  and  temperature  differ  in  their  location  from  those  of  tactile 
sense,  and  that  they  pass  into  the  central  portion  of  the  gray  matter  of 
the  spinal  cord  soon  after  their  entrance.  It  is  certain  that  their 
entire  course  from  below  upward  is  not  in  the  gray  matter,  otherwise 
a  limited  lesion  of  this  portion  in  the  cervical  segments  would  produce 
a  disturbance  of  these  senses  in  the  entire  body  below  the  lesion ;  but 
it  appears  that  these  sensations  on  their  way  from  the  surface  of  the 
body  to  the  centripetal  white  columns  of  the  cord  (the  antero-lateral 
tracts  —  see  page  74)  traverse  the  gray  matter  at  the  level  at  which 
they  enter ;  hence  the  distribution  of  this  disturbance  of  sensibility 
corresponds  exactly  to  the  position  of  the  lesion  in  the  spinal  cord.    As 


SYMPTOMS.  329 

the  exact  area  of  the  skin  related  to  the  individual  segments  of  the  cord 
has  been  ^determined,  it  is  possible  to  reach  a  diagnosis  of  the  exact 
extent  of  the  spinal  lesion  by  determining  the  exact  extent  of  the  an- 
algesia.^ The  diagram,  Plate  VII.  (page  46)  shows  this  relation  so  far  as 
it  is  at  present  determined.  It  demonstrates  that  the  various  districts 
of  the  skin  can  be  assigned  to  the  various  segments  of  the  cord.  The 
distribution  of  the  analgesia  in  syringomyelia  is  usually  irregular,  rarely 
symmetrical  on  the  two  sides.  Inasmuch  as  the  affection  is  more  com- 
mon in  the  cervical  region  the  condition  of  analgesia  is  more  frequently 
found  in  the  hands  and  arms.  It  is  found  that  small  injuries  to  the 
fingers  are  not  attended  by  pain,  an  abnormality  which  first  directs  the 
patient's  attention  to  the  existence  of  his  disease.  In  the  early  stages 
of  the  disease  there  is  merely  a  decided  blunting  of  the  sensations  of 
pain  and  an  inability  to  distinguish  between  slight  variations  of  tem- 
perature, or  certain  sensations  only  are  not  perceived.  Thus  Dejerine 
records  a  case  in  which  the  thermal  sense  was  lost  for  all  temperatures 
above  68°  F.  The  area  of  analgesia  may  not  coincide  exactly  with 
that  of  loss  of  temperature  sense.  Sensations  of  cold  or  of  burning,  or 
sharp  pains,  sometimes  precede  the  loss  of  sensation.  When  the  dis- 
ease is  fully  established  the  patient  cannot  distinguish  any  difference 
between  iced  water  and  boiling  water,  and  a  deep  incision  may  be  made 
without  the  slightest  pain. 

Trophic  disturbances  are  a  very  frequent  symptom  in  syringomyelia. 
In  the  majority  of  patients  it  is  evident  that  the  origin  of  these  dis- 
turbances is  some  injury,  wound,  or  burn  that  had  not  been  observed 
on  account  of  the  loss  of  pain  sense,  and  that  had  therefore  been  ne- 
glected, had  become  infected,  and  had  gone  on  to  ulceration  or  suppura- 
tion. In  some  cases,  however,  it  is  impossible  to  ascribe  trophic  dis- 
turbances to  this  cause,  and  the  hypothesis  of  the  existence  of  trophic 
centres  in  the  spinal  cord  presiding  over  the  general  nutrition  and  the 
repair  of  the  body  receives  its  chief  support  from  the  facts  observed  in 
this  disease. 

The  skin  is  the  seat  of  the  chief  trophic  disturbances.  These  may 
be  of  various  kinds.  There  may  be  localized  hypersemia  or  anaemia 
of  the  skin.  There  may  be  changes  in  the  perspiration,  the  part  being 
abnormally  covered  with  sweat  or  abnormally  dry ;  and  in  addition  to 
the  acute  inflammations  of  the  skin  already  mentioned  as  produced  by 
injuries,  cases  have  been  observed  of  serous  exudation  with  desquama- 
tion, gangrene  of  the  skin  and  subcutaneous  tissue,  bullae  and  peculiar 
hypertrophies  and  atrophies  of  the  skin. 

Another  trophic  disturbance  which  has  excited  much  interest  is  the 
appearance  of  painless  whitlows  and  small  abscesses  upon  the  fingers. 

Morvan  ^  described  a  disease  occurring  in  a  seaport  of  France  among 
fishermen,  in  which  felons  appeared  upon  the  fingers,  producing  deep 
ulcerations  and  even  necrosis  of  the  terminal  phalanges.  These  were 
associated  with  other  trophic  disturbances  of  the  skin  and  nails,  and 

'Max  Laolir,  Arch.  f.  PHychiatfie,  1896,  xxviii.,  773. 
Hiazetto  hob.  de  Med.  et  de  Chir.,  1883. 


330 


SYRINGOMYELIA    OR    GLlOHlH    HPlNAUS. 


with  analgesia.     This  disease,  which  was  named  after  Morvan,  is  now 
thought  to  be  a  variety  of  syringomyelia,  for  in  all  cases  examined  after 


Fig.  141. 


Trophic  changes  In  the  hands  in  syringomyelia  in  the  form  known  as  Morvan's  disease.  The 
muscles  are  atrophied.  The  hands  are  much  deformed.  The  skin  is  atrophied.  The  nails  have  fallen. 
The  bones  are  hypertrophied  at  some  parts,  atrophied  at  others.  The  tip  of  one  finger  is  eroded. 
(Curschmann,  Klin.  Abbildungen.) 


death  a  cavity  has  been  found  in  the  spinal  cord, 
appearance  of  the  hands  in  Morvan's  disease. 


Fig.  141  shows  the 


SYMPTOMS.  331 

The  growth  of  the  nails  is  commonly  affected  in  the  disease.  They 
are  hypertt-ophied,  ridged,  and  occasionally  stained.  They  become 
particularly  brittle  and  are  irregular  in  their  form. 

Affections  of  the  joints  and  bones  are  very  frequently  observed  in 
syringomyelia.  In  fact,  there  is  no  nervous  disease  in  which  joint 
affections  occur  so  commonly  as  a  complication.  The  shoulder,  elbow, 
and  wrist  are  the  joints  most  commonly  affected.  In  this  respect  the 
disease  offers  a  contrast  to  tabes,  in  which  the  joint  affections  most  fre- 
quently occur  in  the  lower  extremities.  The  character  of  the  joint 
affection,  is,  as  a  rule,  quite  similar  to  that  described  by  Charcot  as 
occurring  in  locomotor  ataxia  —  a  large  effusion  within  the  joint,  with 
great  thickening  of  all  the  tissues,  and  later  an  absorption  of  the  bones 
with  an  atrophy  of  the  joint  surfaces.     Fig.  142  shows  the  appearance 

Fig.  142. 


Arthropathy  of  the  right  shoulder  in  syringomyelia.     (Dercuiu.) 

of  a  patient  suffering  from  an  arthropathy  of  the  right  shoulder,  and 
Fig.  143  shows  the  condition  found  in  the  bone  after  death.  Schles- 
inger  has  collected  sixty-three  cases  of  joint  affection  occurring  in 
the  course  of  the  disease,  and  he  estimates  that  this  complication 
occurs  in  more  than  10  per  cent,  of  the  cases.  Alterations  in  the  con- 
dition of  the  long  bones  are  observed  in  syringomyelia,  and  spontane- 
ous fractures,  due  to  a  spongy  and  brittle  condition  of  the  bones,  have 
been  recorded  by  a  number  of  observers.  Both  the  joint  affections  and 
these  fractures  proceed  without  pain  to  the  patient,  and  hence  are  often 
neglected  for  some  time  after  they  begin. 

In  a  considerable  number  of  cases  a  marked  curvature  of  the  spine, 
either  lateral  or  forward,  and  occasionally  backward,  has  been  observed. 
This  has  been  ascribed  by  some  authors  to  atrophy  and  weakness  of 
the  spinal  muscles  and  by  others  to  actual  changes  in  the  bones.  Both 
conditions  may  occur.  The  spine  is,  as  a  rule,  sensitive  to  pressure. 
Deformity  is  more  likely  to  occur  in  the  upper  portion  of  the  dorsal 
region  than  elsewhere.     It  is  never  very  extensive. 

As  the  records  of  the  disease  have  increased  its  incidental  association 
with  various  diseases — acromegalic,  hysteria,  paralysis  agitans — has 
been  recorded.     Such  associations  have  no  particular  significance. 

Muscular  atrophy  attended  by  paralysis  is  present  in  more  than  one- 


332 


SYRINGOMYELIA    OR    GLIOSIS    SPINALIS. 


half  of  the  cases  of  syringomyelia.  It  usually  begins  as  a  progressive 
muscular  atrophy  invading  the  hands,  especially  the  first  lumbricalis 
muscle,  then  the  thenar  and  hypothenar  eminences,  finally  producing 
claw-hands  [main  en  griffe  of  Duchenne),  and  then  advancing  up  the 
limb  to  the  forearm,  arm,  and  shoulder.  Occasionally  the  shoulder 
muscles  are  the  first  to  be  affected,  and  then  the  atrophy  appears  in 
the  deltoid  and  scapular  muscles,  and  later  invades  the  biceps  and 


Fig.  143. 


Humerus  and  scapula  of  the  right  side.  The  head  of  the  humerus  has  been  destroyed.  The  glenoid 
surface  of  the  scapula  Is  enlarged,  and  its  edges  are  thickened  by  bony  deposits.  The  coracoid  process 
presents  peripheral  accretions.     (Dereum.) 

supinator  longus.  The  muscles  of  the  spine  are  particularly  liable  to 
be  invaded  by  the  atrophy  and  paralysis,  and  as  a  consequence  curva- 
ture of  the  spine  is  a  very  frequent  symptom  in  this  disease.  The  legs 
are  less  frequently  affected  (12  per  cent,  of  the  cases),  but  atrophic 
paralysis  of  the  thighs  and  of  the  legs  below  the  knee  with  consequent 
contractures  has  been  seen. 

The  exact  distribution  of  the  atrophy  and  paralysis  depends  upon 
the  extent  of  the  lesion  in  the  various  segments  of  the  cord.  In  the 
table  already  given  on  page  54  the  relation  between  the  various 
muscles  of  the  body  and  the  various  segments  of  the  cord  is  shown. 
It  is  therefore  evident  that  from  a  knowledge  of  the  muscles  invaded  a 
conclusion  can  be  reached  as  to  the  extent  of  the  lesion.  The  atrophic 
paralysis  of  the  muscles  is  attended   by  fibrillary  contractions  and 


SYMPTOMS.  333 

tremors  and  by  a  gradual  diminution  in  the  mechanical  and  electrical 
contractility  of  the  muscle.  It  is  only  in  the  last  stage  of  the  disease, 
when  the  muscle  is  extremely  atrophied,  that  it  presents  the  reaction 
of  degeneration. 

The  spinal  reflexes  may  be  disturbed  in  this  disease.  When  the 
symptoms  are  located  in  the  arms,  elbow  and  wrist  reflexes  are  lost, 
while  the  patella  reflex  is,  as  a  rule,  increased.  If,  however,  the 
disease  invades  the  lumbar  region  of  the  cord  the  patellar  reflex  may 
be  lost  on  the  side  of  the  lesion.  In  a  certain  number  of  cases  there 
is  an  extension  of  the  disease  into  one  or  both  lateral  columns  of  the 
cord.  When  this  occurs  the  lateral  pyramidal  tracts  are  afiected  and 
the  symptoms  of  spastic  paraplegia  (see  Chapter  XVIII.)  are  added 
to  those  of  the  disease.  An  increase  of  knee  jerks,  the  appearance  of 
ankle  clonus  and  Babinski  reflex  and  the  slow  development  of  a  spastic 
gait  with  incontinence  of  urine  will  be  evidence  of  such  extension. 
They  follow  syringomyelia  of  the  cervical  and  dorsal  regions. 

In  a  few  cases  in  which  the  sacral  region  of  the  cord  has  been 
diseased  a  loss  of  control  of  the  bladder  and  rectum  has  occurred  in 
addition  to  the  other  symptoms. 

The  spinal  centre  of  the  cervical  sympathetic  nerve  lies  in  the  first 
dorsal  segment  of  the  cord,  and  as  this  segment  is  very  frequently 
afiected,  symptoms  of  paralysis  of  the  sympathetic  of  one  or  both  sides 
are  commonly  to  be  detected.  They  are  a  narrowing  of  the  palpebral 
fissure,  a  retraction  of  the  eyeball,  sluggish  pupillary  action,  with  im- 
perfect dilatation,  a  flattening  of  the  side  of  the  face,  and  a  defective 
secretion  of  sweat. 

The  extension  of  the  disease  to  the  medulla  may  cause  symptoms 
referable  to  the  implication  of  the  cranial  nerves.  Atrophy  with  fibril- 
lary tremor  in  the  tongue  and  facial  muscles,  ocular  palsies  with  nys- 
tagmus, and  dissociated  anaesthesia  of  the  face  and  head  have  been 
observed.  In  a  few  cases  paralysis  of  the  vocal  cords,  disturbances 
in  the  act  of  swallowing,  difficulty  of  respiration,  and  irregular  heart 
action  have  indicated  that  the  vagus  centre  has  been  affected.  These 
symptoms  are  most  serious,  as  sudden  death  commonly  ensues.  The 
term  syringo-bulbia  has  been  applied  to  this  group  of  symptoms. 

The  course  of  the  disease  is  a  chronic  one.  It  advances  slowly,  and 
the  symptoms  are,  as  a  rule,  well  established  before  the  disease  is 
recognized.  The  patients  remain  for  months  in  a  stationary  condition 
or  the  paralysis  slowly  increases  until  they  are  disabled.  The  symp- 
toms may  finally  extend  to  the  entire  body,  although  this  is  rare. 
Death  occurs  either  from  heart  failure,  or  from  cystitis,  or  bed-sores, 
or  from  some  intercurrent  affection,  or  rarely  very  suddenly  without 
apparent  cause,  really  from  a  rupture  of  the  cord  allowing  an  escape 
of  fluid  from  the  cavity. 

The  following  history  of  a  case  of  syringomyelia  under  my  obser- 
vation at  the  Yanderbilt  clinic  illustrates  the  usual  symptoms  and 
course  of  the  disease  : 

P.  S.,  of  healthy  parentage,  noticed  in  March,  1896,  when  he  was 


334 


SYRINGOMYELIA    OR    GLIOSIS    SPINALIS. 


Fig.  144. 


sixteen  years  of  age,  that  he  was  becoming  weak  and  clumsy  in  his 
hands,  that  he  was  dropping  things  unintentionally,  and  was  losing 
strength  in  "his  arms.  These  symptoms  were  noticed  in  the  left  arm 
before  they  appeared  in  the  right.  They  were  not  attended  by  pain  or 
any  noticeable  sensory  disturbance.     It  was  noticed  that  his  hands  and 

arms  became  gradually  thinner  as 
they  became  weaker,  and  the  emaci- 
ation soon  extended  to  his  body, 
especially  about  the  muscles  -of  the 
chest,  and  back,  and  scapulae.  He 
was  not  aware  of  any  sensory  dis- 
turbance until  the  time  of  his  first 
examination  at  the  clinic  in  Janu- 
ary, 1897.  He  had  had  no  trouble 
with  his  bladder  or  rectum.  He 
had  had  no  symptoms  in  his  legs, 
excepting  a  slight  weariness  on  any 
exertion,  but  he  had  noticed  that 
his  back  had  gradually  become 
crooked,  the  right  side  of  his  body 
appearing  to  bulge.  It  was  evident 
from  the  history  that  all  his  symp- 
toms had  made  such  gradual  progress 
during  the  year  that  they  had  not 
attracted  much  attention  until  his 
inability  compelled  him  to  quit  work. 
Examination  in  January,  1897, 
showed  a  very  marked  condition  of 
atrophy  with  corresponding  paral- 
ysis in  the  muscles  of  both  upper  ex- 
tremities, chest,  scapulae,  and  back, 
as  shown  in  the  picture.  (Fig. 
144.)  No  muscle  was  entirely  para- 
lyzed, but  all  the  muscles  were  ex- 
tremely weak,  presented  fibrillary 
contractions  on  exposure  to  cold  or 
on  percussion,  but  did  not  show 
any  reaction  of  degeneration.  The 
atrophy  was  most  extreme  about 
the  muscles  of  the  scapulae  and  in 
the  deltoids  and  upper  part  of  the  arms.  The  muscles  of  the  thorax 
and  back  were  markedly  atrophied,  so  that  a  lordosis  was  very  evident, 
causing  peculiar  motions  of  balancing  in  the  act  of  walking.  The 
atrophy  was  about  equal  on  both  sides.  The  biceps  was  less  atrophic 
than  the  other  muscles  of  the  upper  arm.  The  flexors  and  extensors 
of  wrist  and  fingers,  the  thenar  and  hypothenar  muscles,  and  the 
interossei  of  the  hand,  were  very  much  atrophied.  The  lower  portion 
of  the  pectoralis    major  on  both  sides  was  preservedj  but  the  upper 


Case  of  syringomyelia.  Atrophy  of  the 
muscles  of  the  shoulders  and  right  arm.  Cur- 
vature of  the  spine  forward  from  atrophy  of 
muscles  of  the  bacli. 


DIAGNOSIS.  835 

part  was  atrophic.  The  muscles  of  the  abdomen  and  legs  were  not  in 
any  way  a^ected,  but  the  knee-jerks  were  very  much  exaggerated,  and 
there  was  ankle  clonus  on  both  sides.  The  elbow  and  wrist  reflexes 
were  lost.  Face  was  normal.  Sensation  to  touch  was  preserved  in  all 
parts  of  the  extremities,  body,  and  thorax,  but  sensations  of  heat  and 
cold  and  of  pain  could  not  be  perceived  over  the  upper  part  of  the 
thorax  or  in  both  upper  extremities.  The  loss  of  pain  sense  was 
somewhat  less  extensive  than  the  loss  of  sensation  to  heat  and  cold  on 
the  back.  This  patient  was  observed  very  carefully  in  St.  Luke's 
Hospital  for  six  months,  but  there  was  little  or  no  change  in  his 
condition.^ 

Diagnosis.  — When  the  three  characteristic  symptoms  already  men- 
tioned are  present  in  any  case  there  is  no  question  regarding  the 
diagnosis  of  syringomyelia.  In  the  early  stages  of  the  disease,  how- 
ever, before  all  three  symptoms  appear,  the  disease  may  be  mistaken 
for  other  spinal  affections.  Thus  many  cases  are  regarded  for  a  con- 
siderable time  as  cases  of  progressive  muscular  atrophy  or  of  chronic 
anterior  poliomyelitis,  and  it  is  only  on  the  appearance  of  the  peculiar 
sensory  disorder  or  of  the  trophic  symptoms  in  the  skin  or  bones  that 
the  first  diagnosis  becomes  questionable.  In  other  cases  the  early  sus- 
picion may  be  of  tabes,  especially  if  the  disease  be  located  in  the  lower 
part  of  the  cord,  for  then  the  pains,  especially  the  burning  sensations, 
the  disturbances  in  temperature  and  pain  sense,  and  the  paraesthesia, 
with  trophic  disturbances  in  the  joints  and  loss  of  tendon  reflex  at  the 
knee  may  suggest  locomotor  ataxia,  even  though  the  ataxia  be  not 
manifest.  Then  it  is  only  when  atrophies  of  the  muscles  and  paralysis 
occur  that  syringomyelia  is  suspected.  The  diagnosis  from  amyotrophic 
lateral  sclerosis  may  be  made  from  the  fact  that  in  that  disease  there  is 
an  increase  of  mechanical  excitability  in  the  paralyzed  muscles,  an 
increase  of  reflex  action,  the  early  appearance  of  a  spastic  gait,  an 
absence  of  sensory  symptoms,  and  little  tendency  to  trophic  disorders. 

A  general  myelitis  or  a  disseminated  myelitis  may  be  diagnosticated 
in  cases  of  syringomyelia  when  both  motor  and  sensory  and  trophic 
disturbances  are  present ;  but  the  lack  of  symmetry  of  the  sensory  dis- 
orders and  the  peculiar  preservation  of  the  tactile  sense  in  syringo- 
myelia should  enable  the  observer  to  avoid  this  mistake.  It  is,  how- 
ever, to  be  remembered  that  in  some  cases  the  two  diseases  occur 
together.^ 

While  it  is  true  that  syringomyelia  is  often  due  to  a  tumor  of  the 
spinal  cord,  especially  glioma,  it  is  to  be  remembered  that  tumors  of 
the  cord  are  usually  limited  in  extent  to  two  or  three  segments,  that 
they  produce  more  widespread  symptoms  than  syringomyelia,  especially 
in  the  body  below  the  level  of  the  lesion  ;  that  the  symptoms  resemble 
those  of  a  transverse  myelitis  of  rapid  onset,  and  that  pain  of  a  severe 
character  is  a  constant  symptom  in  spinal  tumors.     The  course  of  the 

'For  other  histories  the  reader  is  referred  to  the  American  Journal  of  the  Medical 
Sciences,  May,  1888,*  and  December,  1896. 

'Gowers,  Diseases  of  the  Nervous  System,  vol.  i.,  p.  339, 


336  SYRINGOMYELIA    OB    GLIOSIS    SPINALIS. 

disease,  steadily  progressive  in  tumor,  may  aid  the  diagnosis  when 
symptoms  are  ambiguous.  Pachymeningitis  cervicalis  may  give  rise 
to  somewhat  similar  symptoms  in  the  arms;  but  the  severe  pain  in 
the  neck,  the  rigidity  and  the  fixed  posture,  the  absence  of  dissociated 
anaesthesia,  and  the  lack  of  trophic  disturbances  will  prevent  any  mis- 
take in  diagnosis. 

Syringomyelia  presents  some  of  the  features  of  bulbar  palsy  when 
the  cavity  invades  the  medulla  and  pons ;  but  the  cavity  is  rarely  con- 
fined to  the  medulla  and  pons,  and  hence  in  syringomyelia  the  symp- 
toms are  not  exclusively  bulbar ;  thus  a  point  of  distinction  between 
the  two  diseases  is  afforded. 

Prognosis. — The  prognosis  as  to  recovery  is  unfavorable  ;  but  inas- 
much as  the  disease  rarely  progresses  beyond  a  certain  point  it  cannot 
be  considered  dangerous  to  life. 

Treatment.  —  There  is  no  known  remedy  that  will  arrest  the  patho- 
logical process.  The  exposure  of  the  back  to  the  a;-rays  or  to  radium 
has  been  tried  and  deserves  further  trial,  the  results  being  as  yet  uncer- 
tain. The  symptoms  are  to  be  treated  as  they  arise  —  the  paralysis, 
for  instance,  as  in  anterior  poliomyelitis.  The  trophic  disturbances 
may  often  be  prevented  by  care,  and  if  they  occur  are  to  be  treated  by 
rest,  by  mechanical  appliances,  or  by  surgical  measures.  The  sensory 
loss  cannot  be  remedied  by  faradic  applications.  It  is  to  be  remem- 
bered that  the  disease  often  comes  to  a  spontaneous  standstill ;  hence 
remedies  of  a  constitutional  kind  are  not  to  be  implicitly  trusted,  even 
though  they  appear  to  arrest  it. 


CHAPTER  XVIII. 

LATERAL  SCLEROSIS.     SPASTIC   PARAPLEGIA. 

History. — A  condition  of  stiffness  in  the  legs  that  slowly  advances 
to  a  state  of  paralysis,  with  increased  reflexes  and  rigidity,  but  is  not 
attended  by  sensory  symptoms,  though  recognized  by  Turck  in  1856, 
was  first  described  by  Charcot  in  1865.  It  was  carefully  studied  in 
1873  by  Seguin,^  who  named  it  tetanoid  paraplegia.  In  1875  a  com- 
plete analysis  of  the  clinical  symptoms  was  made  by  Erb,^  who  ascribed 
them  to  an  ascending  sclerosis  of  the  lateral  columns  of  the  spinal  cord 
and  named  the  affection  spasmodic  spinal  paralysis.  In  the  following 
year  Charcot  differentiated  it  more  clearly  from  other  spinal  affections, 
naming  it  tabes  dorsale  spasmodique.  Careful  observation  of  cases  soon 
developed  the  fact  that  as  a  symptomatic  condition  it  appeared  much 
more  commonly  as  a  secondary  affection  than  as  a  primary  disease. 
And,  as  the  lesion  of  lateral  sclerosis  was  ascertained  to  be  in  many 
cases  the  result  of  transverse  lesions  of  the  cord  at  a  high  level,  and 
its  identity  with  secondary  descending  degeneration  in  the  lateral 
columns  was  established,  many  observers  denied  the  existence  of  a 
primary  lateral  sclerosis  and  affirmed  that  every  case,  if  of  sufficient 
duration,  would  prove  to  be  of  a  secondary  nature.  It  was  shown  that 
the  lateral  tracts  in  the  spinal  cord,  the  sclerosis  in  which  caused  the 
symptoms,  are  made  up  of  axones  whose  neurone  bodies  lie  in  the  motor 
area  of  the  cerebral  cortex.  And  it  was  proven  that  any  disease  which 
affected  these  neurones,  whether  in  the  brain  cortex  or  in  the  sub- 
cortical tracts,  in  the  brain  axis,  or  in  the  spinal  cord,  unilateral  or 
bilateral,  is  capable  of  causing  the  symptom  of  spastic  paralysis.  It 
was  evident,  therefore,  that  as  a  symptom  it  might  appear  in  many 
different  diseases  and  be  due  to  many  various  lesions. 

It  has  been  shown  that  lateral  sclerosis  results  from : 

1.  Any  disease  in  the  brain  that  affects  the  motor  tracts,  such  as 
tumors,  softening,  hemorrhagic  destruction,  or  inflammation,  with  its 
degenerative  results.  Thus  it  is  the  lesion  present  in  infantile  cerebral 
palsy  (Little's  disease),  whether  unilateral  or  bilateral,  and  also  in 
hydrocephalus.  It  may  also  occur  in  multiple  sclerosis.  After  brain 
diseases  it  is  usually  unilateral.  It  may  be  bilateral  if  the  brain  axis 
is  destroyed  and  both  motor  tracts  are  affected. 

2.  Any  disease  in  the  spinal  cord  that  involves  the  lateral  columns 
or  cuts  them  off  from  their  nutrient  cells  in  the  cortex  of  the  brain, 
such  as  transverse  myelitis,  hemorrhage  in  the  cord,  syringomyelia, 
combined  or  disseminated  sclerosis,  or  tumors  of  the  cord. 

'  New  York  Medical  Journal,  1873. 

^  Virchow's  Archiv,  vol.  Ixx.,  and  Berliner  klin,  Woch.,  No.  26,  1875, 

22  337 


338  LATERAL    8CLER08I8.     SPASTIC    PARAPLEGIA. 

3.  Any  disease  of  the  spiue  which  compresses  the  spinal  cord,  such 
as  caries,  tumors  of  the  vertebra,  pachymeningitis,  or  aneurism. 

There  are,  however,  certain  cases  in  which  the  symptoms  of  spastic 
paraplegia  develop  that  cannot  be  assigned  to  any  of  these- causes,  since 
no  symptoms  of  these  diseases  appear.  And  in  a  few  such  cases  death 
has  occurred  and  the  autopsy  has  proven  that  the  lesion  was  a  lateral 
sclerosis.^  Oppenheim,  Dejerine  and  Soltas,  Striimpell,  and  others 
have  published  cases.  Therefore  it  is  admitted  that  the  disease  may 
occur  as  a  primary  one,  being  a  degeneration  of  the  motor  neurone, 
whose  body  lies  in  the  brain  cortex  and  whose  axone  lies  in  the  lateral 
pyramidal  tract.     This  is  the  cortico-spinal  element  of  the  motor  tract. 

It  has  already  been  shown  that  the  spinomuscular  element  of  this 
tract  is  subject  to  degeneration  in  chronic  anterior  poliomyelitis  (page 
285).  It  has  been  shown  that  in  amyotrophic  lateral  sclerosis  both 
cortico-spinal  and  spinomuscular  elements  are  degenerated  together 
(page  295).  Hence  the  argument,  from  analogy,  points  to  the  exist- 
ence of  primary  lateral  sclerosis.  Furthermore,  Striimpell  has  shown 
that  in  certain  families  a  tendency  exists  to  an  imperfect  development 
of  the  lateral  columns  of  the  cord,  and  he  has  established  the  existence 
of  a  family  type  of  lateral  sclerosis.  Neumark  ^  has  published  the 
history  of  a  family  in  which  seven  children  out  of  nine  developed 
the  disease.  Therefore  the  existence  of  the  disease,  both  congenital 
and  acquired,  must  be  admitted,  having  been  proven  by  pathological 
observation. 

In  1892  Erb  differentiated  from  primary  lateral  sclerosis  a  second 
clinical  form  due  to  syphilis,  and  named  it  syphilitic  spastic  spinal 
paralysis.  It  usually  develops  within  five  years  of  the  initial  lesion. 
It  differs  from  spastic  paraplegia  in  the  facts  that  disturbance  in  the 
control  of  the  bladder  and  rectum  occur,  and  that  there  may  be  slight 
subjective  and  objective  disturbance  of  sensation.  It  has  a  slow  onset 
and  a  chronic  course.  An  analysis  of  the  cases  made  by  Koch  ^  in 
1893  and  added  to  by  Striimpell^  in  1904  has  shown  that  the  lesion 
is  a  transverse  myelitis  of  the  dorsal  region  of  the  cord,  more  or  less 
incomplete,  with  secondary  degeneration  downward  in  the  lateral 
columns  and  upward  in  the  posterior  columns.  Erb  is  inclined  to 
consider  these  degenerations  as  primary  and  due  to  the  syphilo-toxins, 
but  admits  that  they  may  be  secondary  to  the  transverse  lesion.  It 
will  be  considered  under  chronic  myelitis,  as  I  do  not  regard  it  as  a 
true  primary  lateral  sclerosis. 

Etiology.  —  The  etiology  of  spastic  paraplegia  is  obscure.  In  a  few 
cases  long  marches  and  overexertion  have  been  known  to  precede  the 
development  of  the  disease. 

Trauma  has  been  thought  to  be  a  cause,  for  some  cases  have  devel- 
oped after  falls  or  blows  on  the  back.  It  has  been  thought  to  be  trace- 
able to  infectious  diseases  in  a  few  cases. 

^  See  ten  cases  cited  by  Erb,  Lancet,  October  11,  1902, 

2  Amer.  Jour.  Med.  Sci.,  1893,  p.  432  ;  Deut.  Zeitschr.  Nervenk,  1911,  p.  420, 
»Deut.  Zeitschr.  f.  Nervenheilk.,  1893,  vol.  iii,  ^ 

*Ibid.,  vol.  xxvii.,  p.  291. 


PATHOLOGY.  339 

Syphilis  causes  a  condition  closely  allied  to  lateral  sclerosis,  and  is 
probably  sf  cause  in  some  cases.  But,  as  in  tabes,  the  aifection  is  usu- 
ally a  parasyphilitic  disease  and  does  not  yield  to  mercurials  and 
iodide  of  potassium. 

Persons  in  middle  life  are  most  liable,  the  majority  of  cases  devel- 
oping between  the  ages  of  twenty  and  forty  years.  Even  some  of  the 
family  cases  do  not  develop  until  after  the  age  of  twenty  years.  In 
Neumark's  cases  the  disease  developed  gradually  in  some,  in  others  it 
appeared  rapidly  after  acute  infectious  diseases. 

Pathology.  —  The  pathology  of  the  affection  consists  of  a  degenera- 
tion in  the  cortico-spinal  element  of  the  motor  system,  beginning  in  its 
peripheral  portion,  which  lies  in  the  lower  part  of  the  lateral  pyram- 
idal columns  of  the  spinal  cord.  The  degeneration  appears  to  be  a 
primary  one,  not  of  an  inflammatory  nature.  Little  by  little  the 
axones  atrophy  from  below  upward  and  disappear,  the  myelin  that 
surrounds  them  is  absorbed,  and  a  secondary  hyperplasia  of  neuroglia 
occurs,  resulting  in  a  sclerosis  accurately  limited  to  the  distribution  of 
the  long  tracts  of  motor  function  in  the  spinal  cord.  In  the  cervical 
region  the  anterior  median  columns  of  the  cord  have  been  found 
sclerosed.  The  association  tracts  of  the  cord  appear  to  escape,  and 
there  is  no  affection  of  the  anterior  horns  or  of  the  spinomuscular 
element  of  the  nervous  system.  The  appearance  of  the  spinal  cord  is 
not  unlike  that  already  shown  in  Figs.  17  and  18,  page  51,  which 
demonstrate  secondary  lateral  sclerosis.;.  In  Neumark's  cases  a  sclerosis 
of  the  columns  of  Goll  was  also  present,  not  attended  by  any  sensory 
symptoms.  The  lesion  in  his  cases  was  almost  identical  with  that 
found  in  Friedreich's  ataxia  (q.  v.). 

Symptoms.  —  The  symptoms  of  spastic  paralysis  are  a  very  grad- 
ually increasing  stiffness  and  rigidity  of  the  muscles  of  the  legs 
attended  by  an  increase  in  the  reflexes  and  a  tendency  to  cramps  and 
tremor.  The  disease  may  begin  on  one  side,  but  soon  becomes  bi- 
lateral. The  patient  appreciates  difficulty  in  all  motions  of  the  legs; 
he  cannot  step  freely,  he  cannot  go  up  stairs  with  comfort  on  account 
of  great  stiffness  of  the  joints  and  muscles.  It  requires  a  great  effort 
to  produce  slight  movements,  and  passive  motion  is  as  difficult  as  active 
voluntary  motion. 

The  gait  is  characteristic  of  the  affection.  The  feet  are  not  lifted 
from  the  ground,  the  toes  are  dragged,  the  shoe  wears  out  on  its  inner 
surface  and  toe,  the  legs  cannot  be  abducted  freely,  and  the  knees  have 
a  tendency  to  overlap.  The  patient  shuffles  along  the  ground,  his  steps 
becoming  short,  there  being  trepidation  due  to  the  increase  of  reflex 
action,  causing  a  clonus  of  the  foot  at  every  step.  Much  fatigue  is 
felt  on  walking,  and  the  muscles  often  ache.  Little  by  little  the  stiff- 
ness increases  until  the  entire  lower  extremity  appears  to  be  moved  as 
a  mass  without  any  motion  of  the  ankle  or  knee-joints,  and  all  efforts, 
such  as  crossing  the  leg,  kneeling  down,  or  kicking,  are  very  much 
hampered  and  Anally  become  impossible. 

The  muscles  appear  to  be  made  of  hard^  tense  co.r<JS;  and  offer  r^r 


340 


LATEBAL    SCLEEOSIS.      SPASTIC   PABAPLEGIA. 


sistance  to  any  passive  movement.  Percussion  upon  the  muscle  or 
upon  its  tendon  is  immediately  attended  by  a  quick  response,  or  even 
by  severe  twitchings,  or  by  a  marked  clonus.  Such  twitchings  and 
spasms  may  occur  spontaneously  and  the  patients  complain  of  cramps 
and  of  twitchings  which  often  interfere  with  sleep.  Ankle  clonus 
appears  early ;  a  clonus  is  often  obtainable  in  the  toes,  and  pressure 
upon  the  patella  or  upon  the  adductor  tendons  of  the  thighs  may  elicit 


Cases  of  family  type  of  spastic  paraplegia  (two  sisters).    (Icon,  de  la  Salpetrifere,  April,  1910. ) 

a  clonus.  Sometimes  a  sudden  tonic  spasm  of  the  legs  causes  a 
straightening  out  of  the  limb  and  adduction  of  the  thighs.  The 
Babinski  reflex  appears  early.  The  muscles  of  the  hip  are  not  as 
early  or  seriously  affected  as  those  of  the  knee  and  ankle,  conse- 
quently the  patient  can  walk  for  several  years  after  the  disease  has  de- 
veloped and  can  move  the  thighs  in  bed,  even  when  unable  to  walk. 
But  as  the  disease  goes  on  and  the  patient  is  finally  confined  to  the 
chair  or  bed,  contractures  of  the  affected  muscles  occur,  the  knees  are 
drawn  up  and  overlapped,  the  heels  are  drawn  tightly  against  the 
buttocks,  and  the  greatest  efforts  of  the  examiner  fail  to  produce  an 
extension  of  the  legs.  In  this  condition,  when  the  muscles  cannot  be 
actively  or  passively  moved,  they  gradually  atrophy  from  disuse  until 
finally  the  legs  are  reduced  to  a  skeleton  appearance,  the  few  muscles 
left  being  still  contractured.  During  all  this  period  there  are  no 
sensory  symptoms  excepting  general  muscular  pains,  and  there  is  no 
disturbance  of  the  bladder  or  rectum.     There  are  no  trophic  changes 


TREATMENT.  341 

unless  toward  the  close  of  life  long-continued  pressure  or  lack  of  care 
results  ii/the  appearance  of  bed-sores.  The  electrical  contractility  of 
the  muscles  remains  normal. 

The  disease  may  come  to  a  standstill,  as  in  cases  reported  by  Erb 
which  had  been  stationary  for  twenty  to  twenty-six  years,  or  it  may  be 
a  very  slowly  progressive  one,  and  it  is  only  in  the  last  stages  that  any 
stiffness  or  rigidity  of  movement  appears  in  the  upper  extremities.  In 
fact,  very  often  these  escape  entirely.  When  they  are  involved  the 
extensors  are  first  affected  and  become  rigid  before  the  flexors ;  the 
tendon  reflexes  are  greatly  exaggerated.  Wrist  and  finger  clonus  is 
obtained,  and  a  tremor  often  appears  in  the  hands. 

Diagnosis.  —  The  diagnosis  of  primary  lateral  sclerosis  should  only 
be  made  after  a  careful  search  for  a  cause  (see  page  337),  and  every 
effort  should  be  made  to  discover  some  other  disease  to  which  the 
symptoms  of  spastic  paraplegia  are  secondary.  In  the  absence,  how- 
ever, of  any  such  affections  the  diagnosis  may  be  made.  Hysteria 
sometimes  causes  a  condition  of  spastic  paralysis,  but  the  general  his- 
tory of  the  patient  and  the  course  of  the  case,  especially  the  rapid  onset 
of  symptoms  in  hysteria,  will  enable  a  diagnosis  to  be  made. 

Prognosis.  —  The  prognosis  is  invariably  unfavorable  as  to  recovery, 
but  the  course  of  the  disease  is  so  very  slow  that  the  patients  may  be 
assured  of  many  years  of  usefulness  after  the  disease  is  well  developed. 
In  some  cases  a  stationary  period  occurs  under  careful  regime. 

Treatment.  —  The  treatment  is  palliative.  Overexertion  is  to  be 
avoided,  and  yet  walking  should  not  be  abandoned  until  impossible. 
The  general  health  should  be  kept  in  good  condition,  and  every  means 
known  to  increase  the  nutrition  of  the  nervous  system  should  be 
employed. 

These  means  are  fully  discussed  in  the  treatment  of  locomotor  ataxia, 
and  what  is  there  recommended  to  arrest  the  progress  of  the  disease  is 
equally  applicable  to  the  treatment  of  lateral  sclerosis. 

The  symptom  that  gives  greatest  annoyance  in  spastic  paraplegia  is 
the  spasmodic  contraction  of  the  muscles,  causing  jumping  of  the  legs 
or  sudden  extensor  spasm.  This  can  often  be  controlled  by  hot  baths 
or  by  the  application  of  hot  bags  to  the  spine.  It  may  be  much  relieved 
by  the  use  of  bromides  in  full  doses  or  by  bromide  and  chloral  com- 
bined, or  by  the  use  of  the  coal-tar  preparations,  of  which  antipyrine 
is  the  best.  Massage  of  the  affected  muscles  may  also  give  some  relief 
if  the  spasm  occurs  at  night  and  disturbs  sleep. 


CHAPTER   XIX. 

LOCOMOTOR  ATAXIA.     TABES   DORSALIS.     POSTERIOR 
SPINAL  SCLEROSIS. 

Locomotor  ataxia  is  a  chronic  disease  of  the  sensory  portion  of  the 
spinal  cord  and  cerebral  axis. 

Pathology.  —  The  primary  lesion  lies  in  the  posterior  spinal  gan- 
glia and  in  the  ganglia  of  the  cranial  nerves.  The  ganglia  of  the  sym- 
pathetic nervous  system  may  also  be  involved.  The  neurone  bodies 
lying  in  these  ganglia  are  affected  by  the  agent  producing  the  disease, 
and  consequently  undergo  processes  of  degeneration,  with  swelling, 
chromatolysis,  vacuolization,  pigmentation,  and  albuminoid  and  fatty 
degeneration  of  their  axones. 

It  will  be  remembered  that  the  structure  of  a  sensory  cell  differs 
wholly  from  that  of  a  motor  cell  (see  page  28).  It  is  a  flask-shaped 
body  with  but  one  process.  This  divides  at  a  little  distance  from  the 
body  into  two  axones,  of  which  one  passes  inward  through  the  posterior 
nerve  root  into  the  spinal  cord  or  into  the  brain  axis  ;  the  other  passes 
outward  in  the  nerve  to  the  surface  of  the  body.  (See  Plate  II.,  B.) 
When  degenerative  processes  attack  this  neurone  it  is  possible  for  the 
degeneration  to  appear  in  the  peripheral  termination  of  the  nerve  at 
the  surface  of  the  body  only.  This  occurs  in  many  forms  of  multiple 
neuritis.  Under  these  circumstances  the  further  away  the  degeneration 
is  from  the  body  of  the  cell  the  slighter  the  change  in  the  cell  body,  and 
this  in  many  specimens  escapes  notice.  In  some  cases  of  locomotor 
ataxia  a  degeneration  of  the  peripheral  nerves  has  been  found.  In 
other  cases  it  is  the  central  axone  of  the  cell  body,  the  one  extending 
inward  to  the  spinal  cord,  which  appears  to  be  primarily  affected,  and 
here  again  in  the  early  stage  few  changes  may  be  visible  in  the  cell 
body,  though  the  degeneration  of  the  terminal  filaments  of  the  central 
axone  may  be  complete.  This  is  the  case  in  locomotor  ataxia  in  the- 
early  stage  of  the  disease,  and  hence  for  many  years  the  disease  was 
supposed  to  be  a  primary  posterior  sclerosis,  as  the  lesion  was  appar- 
ently limited  to  the  posterior  columns  of  the  spinal  cord. 

But  recent  investigations  have  demonstrated  that  the  posterior 
columns  of  the  spinal  cord  are  made  up  of  a  number  of  distinct  sys- 
tems of  tracts  which  have  been  divided  into  two  categories  and  named 
"  exogenous  "  and  "  endogenous,"  according  to  the  origin  of  the  fibres 
taking  part  in  their  structure.  The  exogenous  fibres  are  those  that 
enter  the  posterior  columns  from  without,  that  is,  through  the  posterior 
nerve  roots,  and  these  are  the  fibres  which  are  primarily  involved  in 
locomotor  ataxia.  Endogenous  fibres  are  really  association  fibres  and 
arise  within  the  cord  from  cells  of  the  gray  matter  and  connect  the 

342 


PATHOLOGY.  343 

various  segments  with  one  another.  These  endogenous  fibres  develop 
later  in  enjbryonal  life  than  the  exogenous  fibres,  and  hence  can  be 
distinguished  from  them  by  the  method  of  Flechsig.  Flechsig  showed 
that  it  is  possible  to  separate  various  systems  of  fibres  in  the  spinal 
cord  from  one  another  by  a  study  of  their  development  in  foetal  life. 
The  axones  of  different  systems  are  covered  by  myelin  at  different 
periods.     In  Fig.  147  the  four  different  systems  of  fibres  are  shown  that 

Fig.  147. 


Development  of  posterior  columns  of  spinal  cord  in  embryo.  I.,  foetus,  24  cm.  ;  a,  undeveloped 
area ;  c,  first  system  of  iibres  ;  II.,  foetus  28  cm. ;  a,  beginning  myelininization  in  second  system ;  c, 
first  system  completely  developed  ;  III.,  foetus,  35  cm. ;  a,  partly  developed  second  system  ;  6,  partly  de- 
veloped third  system  ;  c,  fully  developed  first  system  ;  /,  partly  developed  fourth  system.     (Trepinski. ) 

can  be  distinguished  from  each  other  by  his  method  in  the  posterior 
columns  of  the  lumbar  region  of  the  cord.  If  three  specimens  from 
tabetic  patients  who  have  died  in  the  early,  middle,  and  last  stage  of 
the  disease  respectively.  Fig.  1 48,  be  compared  with  these  foetal  cords, 
it  becomes  evident  that  the  different  systems  of  fibres  in  the  cord  are 
involved  in  tabes  at  different  stages  of  the  disease,  and  that  one  system 
of  fibres,  viz.,  the  endogenous  system,  always  escapes. 

The  study  of  degenerations  in  the  spinal  cord  occurring  subsequently 
to  lesions  of  the  posterior  nerve  roots  at  different  levels  also  affords  a 
demonstration  of  the  existence  of  exogenous  as  distinguished  from 
endogenous  fibres,  the  latter  being  unaffected  in  lesions  of  the  posterior 
nerve  roots.  Sclerosis  following  such  external  lesions  is  identical  in 
its  situation  with  that  occurring  in  the  early  stages  of  tabes.  (See 
Figs.  24  to  26,  page  71. 

For  these  reasons  we  are  forced  to  conclude  that  tabes  is  not  pri- 
marily a  disease  of  the  spinal  cord,  but  that  it  is  a  disease  of  the  sen- 
sory neurones,  resulting  in  a  degeneration  of  the  exogenous  fibres 
pa.ssing  into  the  spinal  cord  from  those  neurones.  Such  degeneration, 
like  that  in  other  portions  of  the  spinal  cord,  is  followed  by  a  condition 
of  sclerosis,  and  this  sclerosis  is  necessarily  limited  to  the  situation  of 
the  degenerated  fibres. 


344  LOCOMOTOR    ATAXIA, 

It  will  be  remembered  that  the  posterior  nerve  root  on  entering  the 
spinal  cord  consists  of  a  number  of  different  sets  of  fibres  of  different 
destination.  (See  Figs.  22  and  23,  page  69).  Many  of  these  fibres 
bifurcate  on  entering  the  cord.  (1)  There  are  fibres  which  enter  directly 
at  the  apex  of  the  posterior  horn  and  turn  upward  in  the  small  bundle 
of  Lissauer  and  enter  the  posterior  gray  matter  at  a  level  a  little  higher 

Fig.  148. 


The  lesions  of  tabes.  I.,  the  first  system,  c,  is  first  affected  by  sclerosis  — in  the  early  stage,  a,  h, 
and/ escape  ;  II.,  the  second,  a,  and  third,  6,  systems  are  next  involved  in  the  sclerosis,  which  becomes 
complete  in  the  first  system,  c,  in  the  medium  stage  ;  III.,  last  stages  of  tabes  — all  systems  except  the 
fourth,/,  are  sclerotic.     (Trepinski.) 

than  their  entrance.  Some  fibres  turn  downward  as  well  as  upward 
in  the  column  of  Lissauer.  (2)  Other  fibres  enter  the  spinal  cord 
opposite  the  posterior  horn  and  penetrate  directly  into  the  horn,  where 
some  end  in  the  network  of  fibres  about  the  cells  of  the  gelatinous  sub- 
stance or  deeper  within  the  horn,  and  others  pass  forward  and  cross 
over  in  the  gray  commissure  to  the  opposite  side,  where  they  turn  out- 
ward into  the  antero-lateral  column  or  backward  into  the  column  of 
Goll.  (3)  The  majority  of  fibres  from  the  posterior  nerve  roots  enter 
the  spinal  cord  in  what  is  known  as  the  median  bundle,  and  these  pass 
directly  into  the  column  of  Burdach,  curve  around  the  median  surface 
of  the  posterior  horn,  and  form  what  is  known  as  the  root  zone  or  lateral 
zone  of  the  column  of  Burdach.  If  we  follow  these  fibres  after  their 
entrance  into  the  root  zone  we  find  them  distributed  in  every  possible 
way  in  their  passage  to  the  posterior  horn  of  the  cord,  (a)  Some  fibres 
turn  downward,  forming  the  comma-shaped  column  of  Schultze,  and 
these  terminate  in  the  second,  third,  and  fourth  segments  below  their 
point  of  entrance.  (6)  Others  pass  almost  directly  into  the  gray  matter 
at  the  level  of  their  entrance,  (c)  Others,  which  are  short,  pass  up- 
ward through  two  or  three  segments,  {d)  Others  still,  of  medium 
length,  pass  upward  through  four  or  six  segments,  forming  the  middle 
zone  of  the  column  of  Burdach.  (e)  The  remainder  (long  fibres)  pass 
all  the  way  up  to  the  medulla  oblongata,  occupying  the  column  of  Goll 
or  the  median  portion  of  the  column  of  Burdach.  An  attempt  is  made 
in  Figs.  22  and  23  (page  69)  to  demonstrate  this  distribution  of  the 


PATHOLOOY.  345 

Various  fibres  entering  at  different  levels.  (See  also  Plate  IX.,  p.  52.) 
All  these  fibres  are  degenerated  in  locomotor  ataxia.  The  extent  of 
the  degeneration  in  the  spinal  cord  will  depend  entirely  upon  the  severity 
of  the  disease  and  upon  the  number  of  posterior  nerve  roots  which  are 
involved  in  the  affection. 

In  the  early  stage  of  locomotor  ataxia,  when  but  few  fibres  are 
degenerated,  the  region  of  sclerosis  is  extremely  limited  in  extent.  As 
the  disease  begins  in  the  vast  majority  of  cases  in  the  neurones  of  the 
lumbar  nerves,  it  is  in  the  lumbar  segments  only  of  the  cord  that  the 
lesion  is  evident,  though,  inasmuch  as  these  lumbar  nerves  send  some 
fibres  all  the  way  up  to  the  medulla,  an  examination  will  show  some 
affection  of  every  segment  of  the  spinal  cord  at  the  area  through  which 

Fig.  149. 


First  lumbar  segment  of  the  cord  in  locomotor  ataxia  in  the  early  stage  of  the  disease.    The  sclerosis  Is 
more  evident  on  the  right  side  in  the  root  zone  of  the  column  of  Burdach. 

they  pass.  Figs.  149  and  150  demonstrate  the  distribution  of  the 
lesion  in  early  cases  of  tabes  where  the  lesion  was  thus  limited  to  the 
lumbar  enlargement. 

As  the  disease  advances,  a  larger  number  of  ganglia  and  posterior 
nerve  roots  are  involved,  and  a  greater  extent  of  tissue  is  degenerated 
in  the  posterior  columns.  The  series  of  sections  (Figs.  152  to  154) 
demonstrate  the  lesions  of  tabes.  The  original  lesion  has  destroyed 
the  posterior  columns  in  the  lumbar  region  and  has  extended  through 
the  dorsal  region,  and  involved  the  cervical  region  of  the  cord.  These 
figures  show  a  series  of  sections  at  various  levels  from  a  patient  who  suf- 
fered thirty  years  from  the  disease,  and  whose  symptoms  were  as  marked 
in  the  upper  extremities  as  in  the  lower.  In  all  these  cases  it  is  evi- 
dent that  the  chief  sclerosis  is  in  the  columns  of  Goll.  The  columns 
of  Goll  are  made  up  almost  entirely  of  fibres  which  have  come  from  the 
sacral  and  lumbar  regions  of  the  spinal  cord,  and  as  these  are  first  and 


346 


LOCOMOTOR    ATAXIA. 


chiefly  affected,  the  lesion  is  most  intense  in  them.     These  escape  in 
any  case  of  tabes  limited  to  the  cervical  region. 


Fig.  150. 


The  lesion  in  the  root  zones  in  the  early  stage  of  locomotor  ataxia.    Weigert  neuroglia  .stain. 
The  deeply  stained  tissue  is  sclerotic.     (Schmaus-Sacki.) 

In  a  case  of  medium  intensity,  when  the  spinal  cord  is  removed,  a 
thinning  of  the  posterior  nerve  roots  is  very  often  apparent.     They 

Fig.  151. 


Locomotor  ataxia.  Lower  cervical  region.  The  contrast  between  the  sclerosis  in  both  posterior 
columns  and  the  normal  tissue  in  the  lateral  columns  is  marked.  The  zone  near  the  posterior  com- 
missure contains  many  normal  fibres.  The  white  patch  in  the  left  lateral  column  is  a  defect  in  the 
cutting.     (Larkin.) 

are  smaller  in  calibre  than  the  anterior  nerve  roots.     The  posterior 
surface  of  the  spinal  cord  is  evidently  somewhat  flattened  in  the  sacral 


PATHOLOGY. 


347 


and  lumbar  regions,  and  this  can  be  seen  in  the  sections.  The  trans- 
verse sectioils  of  the  cord  at  different  levels  demonstrate  the  existence 
of  sclerosis  limited  to  the  posterior  columns  of  the  cord.  This 
sclerosis  may  be  in  early  cases  entirely  limited  to  a  small  region  ad- 
jacent to  the  posterior  horn  (Fig.  149).     In  other  cases  the  sclerosis  is 

Fig.  152. 


Locomotor  ataxia.    Lesion  at  the  lower  lumbar  region.    The  median  portion  of  the  column  of  Goll 
is  normal.    The  root  zone  is  chiefly  aiFected.     (Larkin. ) 

more  extensive  (Fig.  155),  but  in  all  cases  a  region  adjacent  to  the  pos- 
terior commissure  will  be  found  to  contain  normal  fibres,  and  another 


Fig.  153. 


Locomotor  ataxia.  Lesion  at  the  mid-dorsal  region.  The  posterior  marginal  zone  near  the  post- 
commissure  is  normal.  Both  columns  of  Goll  and  Burdach  and  Lissauer's  columns  are  affected.  Same 
case  as  Fig.  144.     (Larkin. ) 

region  adjacent  to  the  posterior  fissure  and  along  the  periphery  of  the 
cord  may  escape  (Fig.  154).  These  are  the  posterior  cornu-commis- 
sural  tract  and  the  sul co-marginal  tract,  which  consist  of  endogenous 
fibres.  A  careful  examination  of  the  sclerotic  region  will  also  demon- 
strate the  existence  witiiin  it  of  numerous  normal  fibres  which  are  also 


348 


LOCOMOTOR   ATAXIA. 


undoubtedly  of  endogenous  orgin,  belonging  to  the  third  set  of  fibres 
developed  in  embryonal  life.     (See  Fig.  147.) 

In  very  advanced  cases  of  tabes  the  lesion  through  the  cord  is  ex- 


Locomotor  ataxia.    Lesion  at  lower  cervical  region.    Both  posterior  columns  are  affected  excepting 
in  the  area  near  the  posterior  commissure.    Same  case  as  Fig.  152.     (Larkin.) 

tensive,  involving  both  the  columns  of  Burdach  and  of  Goll,  but  even 

here  the  escape  of  the  cornu-commissural  and  sulco-marginal  tracts  is 

manifest. 

Fig.  155. 


Locomotor  ataxia.    Lesion  at  the  mid-cervical  region.     (Larkin.) 

Degeneration  is  found  not  only  in  the  columns  of  the  cord  but  also 
in  the  posterior  horns  of  the  cord,  into  which  many  fibres,  as  already 
stated,  pass  from  the  posterior  columns.  It  will  be  remembered  that 
the  posterior  horn  of  the  cord  is  made  up  of  a  gelatinous  and  spongy 
substance,  the  substantia  spongiosa,  lying  nearer  to  the  periphery  than 
the  substantia  gelatinosa.     These  two  substances  are  permeated  by  the 


PLATE  XVIII 


m. 


^^Xs^y^ 


Sclerosis  of  the   Posterior  Column.      (Schmaus.) 

g.  Hyperplastic  glia  (stained  blue)  with  a  few  nerve  fibres  still  preserved,  n.  Nerve 
fibres,  yellow,  k.  Nuclei  of  the  glia.  b  b'.  Bloodvessels.  In  the  lower  part  normal 
fibres.      Weigert's  glia  stain.      X  350. 


PATHOLOGY.  ,  349 

fine  fibres  of  the  cord,  and  these  fibres  are  degenerated  within  the  gray 
matter,  but  lio  special  lesion  can  be  demonstrated  other  than  an  in- 
creased pallor  of  these  substances  when  methods  of  staining  such  as 
that  of  Weigert  are  employed. 

A  large  number  of  the  fibres  of  the  posterior  nerve  roots  pass  into 
the  column  of  Clarke  (a  column  of  large,  round  cells  lying  at  the  base 
of  the  posterior  horn).  These  fibres  are  also  degenerated  in  locomotor 
ataxia,  and  the  fine  plexus  of  terminal  filaments  about  the  cells  of  the 
column  of  Clarke  gradually  disappear.  The  cells  of  the  column  of 
Clarke  themselves  are  not  in  any  way  altered  in  the  disease. 

Many  fibres  that  enter  the  posterior  gray  horn  can  be  traced  in 
normal  cords  inward  through  the  central  gray  matter  into  the  anterior 
horns  of  the  cord,  where  they  terminate  about  the  cells  of  the  anterior 
horn.  These  are  supposed  to  convey  impulses  leading  to  reflex  action. 
These  fibres  also  degenerate  in  cases  of  locomotor  ataxia ;  hence  in  ad- 
vanced cases,  if  methods  of  staining  are  employed  to  demonstrate  the 
fine  network  of  fibres  within  the  gray  matter,  this  network  will  be  seen 
to  be  thin. 

The  process  of  sclerosis  present  in  tabes  resembles  that  in  other 
forms  of  sclerosis  of  the  spinal  cord.  There  is  a  thickening  of  the 
neuroglia  tissue  (Plate  XVIII.)  and  a  gradual  disappearance  of  the  nerve 
fibres.  Here  and  there  through  the  glia  are  found  spider  cells  and  a 
very  marked  increase  of  glia  fibres  is  present  everywhere,  as  shown  by 
the  Weigert  stain.  Nuclear  cells  are  distributed  throughout  the 
sclerotic  patch,  but  this  sclerosis  is  a  process  distinctly  secondary  to 
the  degeneration  of  the  nerve  fibres,  and  may  be  termed  a  substitution 
hyperplasia  rather  than  a  primary  formation  of  neuroglia. 

A  thickening  of  the  meninges  of  the  cord  is  commonly  present  in 
tabes,  though  slight  in  degree,  and  there  is  a  closer  adhesion  of  the 
meninges  to  the  cord  than  in  normal  cords.  This  fact  has  given  rise  to 
the  so-called  meningeal  theory  of  the  origin  of  tabes,  which  is  prob- 
ably true  for  a  certain  proportion  of  the  cases.  It  is  supposed  that  a 
primary  thickening  of  the  meninges,  either  by  syphilitic  deposits  or  by 
a  primary  connective-tissue  inflammation,  produces  a  compression  of 
the  posterior  roots  in  their  passage  through  the  meninges  into  the 
spinal  cord;  and  hence  a  secondary  degeneration  and  sclerosis  occur 
whose  distribution  would  naturally  under  these  circumstances  be 
identical  with  that  already  described.  In  cases  where  syphilis  is  the 
primary  cause  of  a  meningeal  thickening  a  syphilitic  exudation  is 
usually  present  in  the  bloodvessels,  with  thickening  of  the  intima, 
causing  a  reduction  of  the  calibre  of  the  vessels,  and  occasionally  an 
obliteration  of  the  lumen.  This  has  been  found  in  some  cases  of  tabes. 
Such  meningeal  thickening  or  connective-tissue  growth  in  the  pia 
mater  at  the  point  of  entrance  of  the  posterior  nerve  roots  may  cause 
constriction  at  the  entrance  of  these  nerve  roots  and  be  sufficient  to 
produce  a  degeneration  in  the  nerve  fibres. 

Redlich^  and  Nageotte^  have  laid  a  great  deal  of  stress  upon  this 
constriction  as  the  active  cause  of  degeneration  in  tabes ;  but  it  must 

^Path.  des  Tabes,  Jena,  1897.  ^BuL  de  la  Soc-  Anat,,  Nov.  and  Dec,  1894. 


350 


LOCOMOTOR    ATAXIA. 


be  admitted  that  this  is  not  a  constant  factor  and  that  it  is  not  present 
in  the  vast  majority  of  the  cases  of  tabes.  Another  fact  which  bears 
against  this  meningeal  hypothesis  of  the  origin  of  tabes  is  that  in  very 
many  cases  of  meningitis,  and  even  iu  cases  of  syphilitic  meningitis,  no 
such  ascending  degeneration  as  is  found  in  tabes  in  the  posterior  root 
fibres  can  be  demonstrated.  Schmaus  has  called  attention  also  to  the 
fact  that  the  absolutely  symmetrical  character  of  the  degeneration  in 

tabes  would  be  extremely  unlikely 
in  case  the  origin  were  a  diffuse 
meningeal  process. 

Finally  it  is  necessary  to  men- 
tion certain  changes  in  the  posterior 
spinal  ganglia  which  have  been 
found  in  tabes.  An  examination 
of  the  nerve  roots  on  the  periph- 
eral and  central  sides  of  these  gan- 
glia has  demonstrated  that  the 
degeneration  and  atrophy  of  the 
nerve  fibres  is  found  on  the  cen- 
tral side  only.  (See  Fig.  156.) 
And  a  marked  thickening  of  the 
connective  tissue  forming  the  epi- 
neurium  and  perineurium  of  these 
bundles  of  degenerated  nerve  fibres 
has  been  observed.  This  connec- 
tive-tissue growth  has  been  fol- 
lowed into  the  ganglion,  and  evi- 
dence of  an  extreme  degree  of 
interstitial  inflammation  has  been 
found  there.  Whether  this  inflam- 
mation is  the  primary  cause  of  the 
degeneration  of  the  sensory  neur- 
ones which  lie  in  the  ganglia  is  not 
yet  determined. 

While  the  lesion  in  tabes  is 
limited  to  the  sensory  neurones  of 
the  nervous  system,  it  must  be 
admitted  that  no  satisfactory  theory 
of  its  pathogenesis  exists.  It  is 
not  yet  known  why  the  lesion  is 
manifest  in  the  central  axone  of  the  sensory  neurone,  and  yet  is  in 
some  cases  wanting  in  the  neurone  body  and  in  the  peripheral  axone. 
Etiology.  —  The  most  common  predisposing  cause  of  locomotor 
ataxia  is  syphilis,  a  fact  to  which  Erb  was  the  first  to  call  attention. 
Recent  examination  of  the  blood  by  the  Wassermann  test  and  of  the 
spinal  fluid  by  Nonne's  tests  have  confirmed  this  fact  and  authorities 
now  agree  that  in  96  ^  of  cases  syphilis  is  the  cause  of  tabes.  The 
disease  however  is  a  parasyphilitic  affection  ;  that  is  it  follows  syphilis 
but  is  not  cured  by  antisyphilitic  treatjoient,     It  is  probable  that  the 


Spinal  ganglion  in  locomotor  ataxia,  showing 
the  degeneration  in  the  posterior  nerve  root  {hw) 
on  the  spinal  side  of  the  ganglion  as  contrasted 
with  the  normal  appearance  of  the  same  fibres  (p) 
on  the  peripheral  side  of  the  ganglion.  (Kedlich.) 


SYMPTOMS.  351 

anti-bodies  developed  in  the  blood  of  sypliilitics  have  in  some  persons  a 
specific  des^uctive  effect  on  the  posterior  spinal  ganglia,  and  cause  tabes. 

The  influence  of  syphilis  in  the  production  of  tabes  becomes  more 
apparent  when  attention  is  called  to  the  fact  that  it  is  present  in  only 
about  20  per  cent,  of  cases  of  other  forms  of  spinal-cord  or  nervous 
affections. 

There  appears  to  be  no  fixed  period  after  the  syphilitic  infection 
within  which  tabes  develops.  If  a  large  number  of  cases  (1,000)  be 
collected  it  will  be  found  that  the  disease  develops  within  five  years  of 
the  infection  in  less  than  20  per  cent. ;  that  it  develops  between  five 
and  ten  years  after  the  infection  in  25  per  cent. ;  between  ten  and 
fifteen  years  in  22  per  cent.;  between  fifteen  and  twenty  years  in  20 
per  cent.,  and  that  it  may  develop  as  late  as  thirty  or  even  thirty-five 
years  after  the  original  disease. 

Exposure  to  cold,  overexertion,  especially  by  long-continued  stand- 
ing, or  long  marches,  or  a  combination  of  these  causes,  such  as  occur 
in  workmen  who  stand  and  labor  in  damp  places  ;  traumatism,  such  as 
falls  or  blows  upon  the  back  or  upon  the  buttocks  or  upon  the  feet,  and 
sexual  excess  are  all  causes  of  locomotor  ataxia.  It  is  to  be  remem- 
bered that  individuals  whose  nutrition  is  poor  or  those  who  have 
indulged  in  alcoholic  excesses  are  much  more  liable  to  develop  tabes 
than  otherwise  healthy  individuals.  In  one  of  my  cases  the  symptoms 
followed  soon  after  a  severe  blow  in  the  dorsal  region,  and  in  a  num- 
ber of  my  cases  a  history  of  excessive  standing  or  walking  was 
obtained. 

Locomotor  ataxia  is  a  disease  of  adult  life.  It  is  true  that  a  few 
cases  of  juvenile  tabes  have  been  recorded.  These  are  very  exceptional, 
and  have  been  traced  in  all  cases  to  hereditary  syphilis.  The  follow- 
ing table  shows  the  age  of  patients  affected  in  1,000  cases : 

Table  VIII.  —  Age  at  Onset. 

2  cases. 

96     " 

460     " 

348     " 

90     " 

4     " 

The  disease  is  more  common  among  men  than  among  women.  Of  484 
cases  of  which  I  have  records,  431  were  males  and  53  were  females. 

Symptoms.  —  The  symptoms  of  locomotor  ataxia  are  very  numerous 
and  may  be  classified  according  to  the  time  of  their  appearance. 
Though  it  must  be  admitted  that  while  ordinarily  there  is  a  definite 
succession  in  the  appearance  of  these  symptoms,  all  cases  do  not  con- 
form to  the  rule.  We  distinguish  between  a  stage  of  pain,  a  stage  of 
ataxia,  and  a  stage  of  paralysis. 

The  First  Stage  or  Stage  of  Pain. — Parsesthesise  are  the  most  common 
of  the  early  symptoms  of  locomotor  ataxia.  These  were  complained 
of  iu  all  but  fivq  cases  out  of  four  hundred  aud  fifty-two.     The  patient 


"       20    " 

30 

■"^  'igc 

"       30    " 

40 

u       40    u 

50 

"       50    " 

60 

"       60    " 

70 

352  LOCOMOTOB  ATAXIA. 

complains  of  tingling  and  numbness  in  the  legs,  of  a  feeling  as  if  ants 
were  crawling  over  the  surface  (formication),  of  a  sensation  as  if  the 
limbs  were  asleep,  or  as  if  he  were  walking  upon  cotton  or  upon  pins ; 
of  unusual  sensations  of  heat  or  of  cold  in  various  regions  of  the  skin, 
of  sensations  of  itching  or  of  sharp  burning,  and  of  sensations  of  un- 
due fatigue  on  slight  exertion.  These  may  remain  during  the  entire 
course  of  the  case  and  give  rise  to  great  discomfort,  or  as  the  case  goes 
on  they  may  subside,  and  in  the  later  stages  no  longer  be  felt.  They 
are  evidence  of  irritation  in  the  sensory  nerve  fibres,  and  are  really 
illusional  sensations  not  due  to  any  actual  disturbance  in  the  parts  in 
which  the  sensations  are  felt,  but  having  their  origin  in  the  nerve 
roots  at  their  entrance  to  the  spinal  cord,  and  being  referred  by  con- 
sciousness not  to  their  actual  site  of  origin  but  to  the  periphery  from 
which  the  irritated  fibres  arise.  These  abnormal  sensations  are  usually 
felt  first  in  the  anterior  and  inner  surface  of  the  thighs  or  in  the  outer 
side  of  the  legs  below  the  knee,  and  gradually  increase  in  Qxtent  until 
the  entire  lower  extremities  are  affected.  They  may  then  be  felt  in  the 
body  and  trunk  and  thorax.  They  finally  reach  the  upper  extremities, 
being  felt  first  in  the  little  fingers  and  inner  half  of  the  hand,  and 
then  in  the  arms  or  even  in  the  neck  to  the  head.  The  so-called  girdle 
sensation  is  a  hypersensitive  condition  of  the  skin  with  the  production 
of  parsesthesia  on  pressure.  It  is  felt  about  the  waist,  but  as  the  dis- 
ease advances  may  ascend  little  by  little  until  it  is  felt  like  a  collar 
around  the  neck.  This  feeling  of  pressure  is  at  times  very  intense 
and  extremely  disagreeable.  Patients  describe  it  as  a  cord  or  baud 
tied  tightly  about  the  body. 

Pain  is  the  most  serious  and  distressing  of  all  the  symptoms  in 
locomotor  ataxia.  It  may  be  the  first  symptom  perceived ;  in  fact,  it 
is  very  often  the  only  symptom  present  for  many  months  before  the 
physical  signs  of  the  disease  appear.  It  was  complained  of  in  all  but 
nine  out  of  four  hundred  and  fifty-two  cases.  This  pain  is  of  the 
sharp,  shooting  character,  hence  often  termed  "lightning  pain."  It  is 
neuralgic  in  character,  is  not  continuous  for  any  length  of  time,  but 
the  attacks  may  occur  with  such  frequency  as  to  lead  to  periods  of  pain 
lasting  for  hours  or  even  for  days.  Pain  may  be  deep  in  the  limbs  or 
may  be  distinctly  localized  upon  the  surface  of  the  body  at  some  small 
area.  It  usually  begins  in  the  inner  surface  of  the  thigh,  or  on  the 
anterior  surface  of  the  thigh,  or  about  the  knee,  or  down  the  outer  side 
of  the  leg.  As  the  disease  advances  it  becomes  more  extensive  in  its 
distribution  and  advances  gradually  upward,  being  felt  about  the  body 
and  finally  in  the  hands  and  arms.  It  continues  as  a  prominent 
symptom  throughout  the  first  stage  of  the  disease,  but  does  not  by  any 
means  disappear  in  the  second  stage,  and  though  very  often  much  less 
intense,  it  may  continue  into  the  stage  of  paralysis.  Thus  in  one  case 
at  present  under  my  care  where  the  disease  has  been  present  for  twenty 
years  and  where  the  patient  is  completely  unable  to  walk  or  to  stand, 
attacks  of  pain  come  on  about  twice  a  year,  lasting  for  a  week  or  ten 
days  with  as  great  intensity  and  severity  as  at  the  outset.  In  this 
patient  these  attacks  are  continuous,  the  sharp  pain  intermitting  for  a 


SYMPTOMS.  353 

few  minutes  only  and  persisting  night  and  day,  preventing  all  rest  and 
interfering  paarkedly  with  nutrition  and  being  attended  by  rapid 
emaciation.  No  special  cause  can  be  ascertained  for  these  attaciss  of 
pain,  and  nothing  influences  them  excepting  enormous  doses  of  mor- 
phine, and  even  ten  or  twelve  grains  a  day  at  times  fail  to  hold  the 
pain  in  check.  In  this  case  the  pains  are  entirely  limited,  as  are  all 
the  symptoms,  to  the  legs,  the  disease  never  having  advanced  above  the 
level  of  the  first  lumbar  segment  of  the  cord.  Sudden  attacks  of 
pain  may  be  located  about  the  body  and  in  the  epigastrium,  attended 
by  vomiting,  and  may  be  the  first  sign  of  the  disease  in  certain  cases. 
Neuralgia  of  the  trigeminal  nerve  is  extremely  rare  in  tabes.  Pain 
may  be  mistaken  in  the  early  stages  for  rheumatism  or  sciatica,  but,  as 
a  rule,  is  much  more  limited  in  extent  and  sharper  in  character  than 
rheumatic  pain,  and  is  in  the  early  stage  of  the  disease  usually  differ- 
ent in  its  distribution  from  the  pain  of  sciatica.  It  is  not  to  be  for- 
gotten, however,  that  sciatic  pain  may  be  the  first  sign  of  locomotor 
ataxia.  The  pain  is  different  from  that  in  multiple  neuritis,  inasmuch 
as  it  is  not  symmetrical  on  the  two  sides ;  it  rarely  affects  the  distal 
parts  of  the  extremities  first,  and  is  not  attended  by  any  tenderness 
along  the  nerves.  Pains  in  the  little  fingers  and  inner  side  of  the 
hand  may  be  the  first  evidence  that  the  disease  has  extended  from  the 
dorsal  to  the  cervical  region  of  the  spinal  cord.  Pain  in  the  back  and 
loins  of  an  aching  character  is  occasionally  felt,  but  sharp,  shooting 
pains  are  not  common  in  this  locality. 

Pain  is  commonly  attended  or  followed  by  a  very  hypersensitive 
state  of  the  region  in  which  it  appears.  This  hypersensitiveness  may 
be  so  great  as  to  prevent  the  patient  enduring  the  contact  of  clothing, 
or  it  may  be  more  intense  for  temperature  changes,  and  has  been  called 
thermal  hypersensitiveness  or  "  hypercry algesia."  It  is  commonly  a 
symptom  of  the  early  stage  of  the  disease;  hence  these  patients  are 
unable  to  endure  applications  of  heat  or  cold,  especially  of  cold,  as  the 
sensitiveness  of  the  body  to  cold  is  markedly  increased  even  when  the 
sensation  of  heat  is  not  affected. 

Patients  with  locomotor  ataxia  are  very  much  influenced  by  atmos- 
pheric conditions.  This  fact  is  to  be  thought  of  in  recommending  a 
climate  in  which  these  patients  can  dwell  with  most  comfort.  It  is 
found  that  a  low  state  of  the  barometer  combined  with  a  high  percentage 
of  humidity,  either  in  summer  or  winter,  is  frequently  followed  by  an 
attack  of  severe  pain.  It  is  true  that  the  patients  suffer  more  in  cold 
weather  than  in  warm,  and  in  a  cold  climate  more  than  in  a  warm 
climate ;  but  it  is  the  high  percentage  of  humidity  in  the  atmosphere, 
together,  possibly,  with  certain  electrical  conditions  not  yet  fully  under- 
stood, which  give  rise  to  the  greatest  amount  of  pain.  Thus  when  the 
humidity  is  over  70  per  cent,  and  the  barometer  is  between  29.65  and 
29.90  patients  are  almost  sure  to  have  an  attack  of  pain.  It  is  also 
found  that  many  patients  suffer  more  just  before  a  thunderstorm,  just 
as  many  neurotic  patients  are  found  to  have  great  discomfort  in  the 
form  of  motor  restlessness  and  headache  or  disagreeable  visceral  and 
cardiac  sensations  prior  to  or  during  a  thunderstorm.  It  is,  therefore, 
23 


354 


LOCOMOTOR   ATAXIA. 


to  atmospheric  conditions  not  fully  understood  that  the  sudden  unex- 
plained attacks  of  pain,  both  in  tabes  and  in  neuralgia  are  to  be 
ascribed. 

Pains  of  tabes  differ  from  neuralgic  pains  rather  in  their  distribution 
than  in  their  character.  The  distribution  of  the  pain  in  locomotor 
ataxia  is  in  the  region  of  the  skin  supplied  l)y  a  segment  of  the  cord. 
Pain  in  neuralgia  is  in  the  distribution  of  the  skin  supplied  by  definite 
peripheral  nerves.     (Compare  Plates  IV.  and  VII.) 

In  the  stage  of  pain,  even  when  parsesthesiae,  and  hypersensitiveness 
to  cold  and  heat  are  present,  it  is  not  common  to  discover  any  objective 
disturbance  of  sensation.     Sometimes,  however,  a  slight  difficulty  in 


Fig.  157. 


Areas  of  ansethesia  on  the  body  and  legs  in  cases  of  locomotor  ataxia.     (Bonar.) 

perception  of  sensations  of  cotton-wool  is  admitted  when  the  legs  are 
compared  with  the  hands,  but  it  is  not  to  be  forgotten  that  differences 
of  slight  sensation  are  perceived  normally  in  the  body  when  the  in- 
sensitive legs  are  compared  with  the  more  delicate  hands.  Laehr 
and  Patrick  have  called  attention  to  the  existence  of  small  bands  of 
anaesthesia  appearing  upon  the  trunk,  even  when  the  legs  are  not 
anaesthetic,  and  Russell  has  demonstrated  their  existence  on  the  inner 
surface  of  the  arm  from  the  axilla  downward,  even  in  the  early  stage 
of  pain.  Figs.  157  and  158  illustrate  this  condition  in  cases  observed 
in  my  own  clinic  by  Bonar  and  published  by  him.^  His  examination 
of  the  clinic  cases  demonstrated  the  presence  of  such  bands  of  anaes- 
thesia in  more  than  90  per  cent. 

Disturbance  in  the  Action  of  the  Bladder  and  Rectum  is  a  very  com- 
mon symptom  in  the  early  stage  of  the  disease.  In  fact,  it  may  be  the 
first  symptom  to  call  attention  to  a  spinal  affection.  It  was  present  in 
80  per  cent,  of  my  patients.  The  mechanism  controlling  these  organs 
lies  in  the  sacral  region  of  the  spinal  cord,  but  in  order  that  this 

^  Sensory  Disturbances  in  Locomotor  Ataxia.  New  York  Medical  Kecord,  1897,  p. 
721, 


SYMPTOMS. 


355 


Fig.  158. 


mechanism  should  act  perfectly  sensory  Impulses  must  reach  the 
organs.  Any  disturbance,  therefore,  in  the  reception  of  sensations 
from  the  mucous  membrane  or  muscle  fibres  of  the  bladder  will  pro- 
duce defective  action.  This  may  be  shown  by  an  insensitiveness,  so 
that  the  patient  is  not  aware  when  the  organ  is  overdistended,  or  it 
may  be  of  the  nature  of  abnormal  sensations,  causing  an  attempt  at 
the  emptying  of  the  bladder  when  it  is  not  at  all  full.  A  common 
complaint  of  the  patients  is  that  they  cannot  voluntarily  empty  the 
bladder  and  have  to  strain  and  press  for  some  time  before  the  efPort  is 
successful.  Retention  of  urine  is  not  uncommon 
in  the  disease,  and  not  infrequently  the  bladder  is 
never  fully  emptied,  and  hence  residual  urine  de- 
composing causes  cystitis.  It  is  rare,  however, 
for  a  patient  with  locomotor  ataxia  to  require 
catheterization,  and  many  patients  find  some  arti- 
ficial method  by  which  they  can  start  the  act  of 
urination.  This  may  be  by  assuming  an  unusual 
position  or  by  resorting  to  some  unusual  method. 
The  action  of  the  rectum  is  different  from  that  of 
the  bladder.  There  seems  to  be  a  loss  of  muscular 
contractile  power  in  the  involuntary  muscles  of 
the  intestine,  and  chronic  constipation  is  the  rule 
in  locomotor  ataxia.  There  is  a  loss  of  expulsive 
power  in  the  rectum  without  any  relaxation  of  the 
levator  or  sphincter  ani,  hence  it  is  usually  neces- 
sary to  evacuate  the  rectum  by  means  of  enemata. 

Impotence. — An  undue  degree  of  sexual  desire  Anesthetic  areas  in  a  case 
has  been  observed  in  the  early  stage  of  locomotor  of  tabes.  (Bonar.) 
ataxia  in  some  cases.  In  the  majority  however, 
there  is  a  gradual  loss  of  sexual  power,  and  in  the  stage  of  ataxia  the 
patients  are  usually  impotent.  This  symptom  appears  about  the  time 
disturbance  in  the  control  of  the  bladder  develops,  and  rarely  improves 
under  treatment.  Undue  attempts  at  coitus  are  often  followed  by  an 
increase  of  the  symptoms  of  pain  and  ataxia. 

The  Physical  Signs  of  the  disease  in  the  early  stage  are  the  loss  of 
patellar  tendon  reflex,  the  loss  of  the  pupil  reflex  to  light,  and  a  con- 
traction of  the  pupil. 

The  Loss  of  Knee-jerk  is  the  earliest  symptom  of  locomotor  ataxia, 
and  may  give  rise  to  a  fear  of  the  onset  of  this  disease,  even  when  no 
symptoms  are  present.  This  is  particularly  true  of  physicians.  It  is 
to  be  remembered  that  in  a  few  normal  individuals  the  knee-jerk  can- 
not be  elicited  (2  per  cent.?).  It  is  to  be  remembered  also  that  in  cer- 
tain post-febrile  conditions,  noticeably  after  diphtheria,  the  knee-jerk 
disappears  for  several  months.  It  is  also  to  be  remembered  that  any 
act  of  the  attention  directed  to  a  spinal  reflex  inhibits  it;  hence  in  many 
individuals  it  is  only  by  a  diversion  of  the  attention,  by  testing  the 
knee-jerk  when  unexpected,  or  by  deflection  of  the  inhibitory  impulses 
into  other  clianncsls  by  means  of  active  voluntary  effort,  such  as  clasp- 
ing the  hands  tightly  or  pulling  or  lifting  objects  while  the  test  is  made. 


356  LOCOMOTOE   ATAXIA. 

that  the  knee-jerk  can  be  elicited.  These  methods  of  reinforcement  of 
Jendrassik,  as  they  are  called,  are  not  to  be  neglected  in  applying  tests. 

The  knee-jerk  is  elicited  by  tapping  the  patellar  tendon  either  upon 
its  front  or  upon  one  side,  when  a  quick  contraction  of  the  quadriceps 
femoris  occurs,  causing  a  slight  kick.  We  need  not  enter  into  any  dis- 
cussion of  the  exact  nature  of  this  reflex  act,  as  to  whether  it  depends 
upon  a  transmission  of  impulses  from  the  point  irritated  through  the 
cord  and  outward  to  the  muscle,  or  whether  it  is  dependent  upon  a  cer- 
tain muscular  tonus  for  whose  existence  sensory  impressions  must  be 
acting  on  the  centres  of  the  spinal  cord.^  Whatever  theory  may  be 
accepted,  it  must  be  admitted  that  a  loss  of  tendon  reflex  of  the  knee 
is  one  of  the  very  earliest  signs  of  locomotor  ataxia.  This  sign  is  found 
in  98  per  cent,  of  the  cases.  It  is  called  Westphal's  sign  or  symptom, 
as  it  was  first  noticed  by  him.  The  location  of  the  mechanism  presid- 
ing over  this  reflex  act  is  in  the  second  and  third  lumbar  segments  of 
the  cord,  and  these  are  the  segments  first  affected  by  the  disease ;  hence 
its  value  as  an  early  sign  of  tabes. 

The  Loss  of  the  Reflex  Action  of  the  Pupil  to  Light,  its  contraction 
in  the  act  of  accommodation  being  preserved,  was  a  symptom  of  tabes 
first  pointed  out  by  Argyll-Robertson,  of  Edinburgh.  It  is  present  in 
90  per  cent,  of  the  cases.  The  mechanism  of  this  reflex  is  still  a  matter 
of  dispute,  and  it  is  not  definitely  ascertained  whether  the  break  in  the 
reflex  arc  lies  in  the  segment  between  the  second  and  third  nerves  near 
the  corpora  quadrigemina  or  is  through  the  sympathetic  nerve,  which 
has  its  origin  at  the  first  dorsal  segment  of  the  cord.  It  is  a  valuable 
and  early  sign  of  tabes.  It  may  be  easily  elicited  by  covering  the  eyes 
of  the  patient  and  then  suddenly  exposing  them  to  light,  or  by  putting 
the  patient  in  a  dark  room  and  with  an  ophthalmoscopic  mirror  throw- 
ing a  ray  of  light  into  the  eye.  Not  uncommonly  it  appears  in  one 
eye  some  time  before  it  appears  in  the  other.  A  continual  contraction 
of  the  pupil  (myosis  spinalis)  in  which  the  pupil  is  reduced  to  a  pin- 
point, does  not  react  to  light,  but  still  reacts  slightly  in  accommodation, 
is  observed  occasionally  in  the  early  stage  of  locomotor  ataxia,  but  in 
the  majority  of  cases  does  not  develop  until  the  later  stage  of  the  dis- 
ease. In  the  cases  in  which  the  pains  begin  in  the  arms  it  is  an  early 
symptom ;  hence  it  has  been  referred  to  the  lesion  in  the  first  dorsal 
segment  of  the  spinal  cord. 

Irregular  Modes  of  Onset. — The  preceding  symptoms  are  present  in 
the  first  stage  of  locomotor  ataxia  in  about  85  per  cent,  of  the  cases, 
but  in  the  other  15  per  cent,  the  first  stage  of  the  disease  presents  cer- 
tain anomalies.  In  fact,  the  disease  may  go  on  for  many  months  with- 
out the  proper  diagnosis  being  reached,  as  the  patient  may  be  treated 
by  his  family  physician  for  certain  unusual  symptoms  or  may  be  sent 
to  any  one  of  a  number  of  specialists  in  eye,  ear,  throat,  stomach, 
bladder,  or  mental  disease,  or  even  to  a  surgeon,  for  an  affection  of  the 
joints  or  bones.  It  is  necessary,  therefore,  to  consider  the  irregular 
modes  of  onset  in  locomotor  ataxia  in  the  first  stage  of  the  disease.    In 

*  Sherrington,  International  Medical  Congress,  Paris,  1900. 


SYMPTOMS.  357 

all  of  these  irregular  modes  of  onset,  however,  a  careful  examination 
of  the  patijsnt  will,  as  a  rule,  reveal,  either  at  the  beginning  or  in  the 
course  of  the  distressing  symptoms,  the  loss  of  knee-jerk  and  the  Argyll- 
Robertson  pupil.  These  physical  signs,  therefore,  are  essential  to  the 
diagnosis  of  the  disease. 

A  very  common  early  symptom  in  locomotor  ataxia  is  the  sudden 
development  of  strabismus.  This  was  the  first  symptom  in  4  per  cent, 
of  my  cases.  The  patient  notices  double  vision.  The  strabismus  may 
assume  any  one  of  the  various  possible  forms,  and  is  occasionally  at- 
tended by  ptosis.  The  paralysis  is  usually  limited  to  one  or  two  of  the 
muscles  moving  the  eyeball.  It  is  not  usual  for  the  ciliary  muscle  to 
be  paralyzed.  The  abducens  may  be  paralyzed,  but  the  trochlearis 
always  escapes.  This  condition  of  oculomotor  palsy  is,  as  a  rule,  tran- 
sient. Nystagmus  does  not  occur.  These  ocular  palsies  may  develop 
at  any  time  during  the  course  of  the  disease.  I  have  seen  them  both 
in  the  second  and  third  stages  of  the  affection.  In  some  cases  these 
palsies  are  of  syphilitic  origin  and  indicate  exudations  of  gummy  mate- 
rial upon  the  base  or  a  syphilitic  neuritis,  such  as  occurs  with  great 
frequency  in  the  oculomotor  nerve  and  occasionally  in  the  abducens 
nerve,  and  then  they  subside  rapidly  under  specific  treatment. 

Another  symptom  which  may  occur  early  is  a  beginning  blindness. 
This  was  the  first  symptom  in  2  per  cent,  of  my  cases.  It  is  due  as 
a  rule,  to  a  primary  optic-nerve  atrophy,  which  condition  may  be  the 
first  sign  of  tabes,  and  may  remain  without  further  symptoms  for  many 
years.  There  is  always,  therefore,  in  cases  of  primary  optic  atrophy  a 
suspicion  that  this  symptom  may  be  the  precursor  of  a  locomotor  ataxia. 
But  in  9  per  cent,  of  cases  of  locomotor  ataxia  of  the  ordinary  type, 
with  the  ordinary  onset,  optic  atrophy  develops  as  a  symptom.  It  may 
develop  in  the  stage  of  ataxia  or  in  the  last  stage  of  paralysis  ;  hence 
ophthalmoscopic  examinations  should  be  made  in  every  case  where  any 
suspicion  of  tabes  exists.  Where  it  is  the  initial  symptom  the  patient 
first  notices  a  slight  diminution  of  clear  vision  and  an  imperfect  per- 
ception of  colors,  and  careful  perimetrical  examination  of  the  visual  field 
will  demonstrate  a  diminution  of  the  visual  field  for  color  and  also  a  pro- 
gressive diminution  of  the  visual  field  for  light.  At  the  same  time  accu- 
racy of  central  vision  diminishes,  a  hazy  appearance  is  presented  to  all 
objects,  and  little  by  little  sight  is  lost.  The  ophthalmoscopic  appear- 
ances are  those  of  primary  optic  atrophy.  (Chapter  XXXV.)  Usually 
such  an  optic  atrophy  is  bilateral.  According  to  Erb's  statistics,  optic 
atrophy  is  the  early  symptom  in  1 J  per  cent,  of  the  cases  and  develops 
in  1 8  per  cent,  of  all  cases  before  the  end.  Of  450  cases  observed  by  me, 
41  cases  showed  optic  atrophy,  and  in  1 1  of  these  it  was  the  first  symptom. 
In  cases  in  which  it  is  not  the  first  symptom  of  the  disease,  but  develops 
subsequently,  changes  in  the  ophthalmoscopic  appearance  of  the  disk 
may  be  presented  and  be  visible  to  the  examiner  for  some  time  before 
the  patient  notices  any  diminution  of  sight ;  hence  from  the  very  out- 
set in  any  case  of  locomotor  ataxia  careful  examination  of  the  visual 
fields  as  well  as  of  the  optic  disks  should  be  made.  While  concentric 
diminution  of  the  visual  field  is  the  rule,  sometimes  the  temporal  half 


358  LOCOMOTOR   ATAXIA. 

of  the  field  is  more  diminished  than  the  nasal  half,  and  occasionally 
hemianopsia  has  been  observed.     Central  scotoma  is  very  rare. 

Deafness  and  symptoms  referable  to  the  auditory  nerve  may  be 
among  the  unexpected  and  early  signs  of  locomotor  ataxia.  It  is 
usually  preceded  by  ringing  in  the  ears  and  sometimes  by  attacks  of 
vertigo,  or  by  a  constant  sensation  of  swimming,  or  by  difficulty 
in  turning  the  head  and  eyes,  without  the  development  of  vertigo. 
Examination  in  such  cases  will  usually  show  a  deafness  to  high  notes, 
and  a  progressive  diminution  in  the  tone  field  will  ensue,  which  finally 
results  in  almost  total  deafness.  This  develops  usually  first  in  one 
ear,  but  soon  follows  in  the  other.  Where  there  i^  no  history  of 
hereditary  deafness  due  to  auditory  atrophy,  the  development  of  such 
a  condition  should  suggest  the  possibility  of  tabes.  It  does  not  de- 
velop, however,  with  by  any  means  the  frequency  of  optic  atrophy. 
In  fact,  statistics  show  that  deafness  is  present  in  but  1  per  cent,  of 
the  cases. 

Crises.  —  Another  mode  of  onset  of  locomotor  ataxia  is  by  the  de- 
velopment of  what  are  known  as  "  crises."  These  were  the  first  symp- 
toms in  18  of  my  cases  and  were  present  at  some  time  in  the  course  of 
the  disease  in  58  cases,  i.  e.  12  per  cent. 

The  most  common  crisis  is  the  gastric  crisis,  the  patients  are  often 
treated  for  several  years  for  supposed  diseases  of  the  stomach  and  for 
chronic  gastritis,  when  an  examination  of  the  eyes  or  of  the  patellar 
reflexes  would  have  easily  demonstrated  that  the  cause  of  the  symp- 
toms was  locomotor  ataxia.  The  gastric  crisis  begins  suddenly  with 
severe  pain  in  the  stomach,  with  retching  and  vomiting,  and  this 
vomiting  will  often  continue  for  several  hours  or  days,  all  material  put 
into  the  stomach  being  immediately  rejected.  Great  prostration,  as  a 
rule,  follows,  an  intense  anxiety  and  distress.  Careful  examination 
of  the  gastric  contents  and  of  the  gastric  juice  fails  to  reveal  any  con- 
stant changes.  In  some  cases  the  mucous  vomiting  is  extremely  acid, 
in  other  cases  there  seems  to  be  a  lack  of  acidity.  The  condition, 
therefore,  is  evidently  not  due  to  any  primary  gastritis  or  disturbance 
of  function  of  the  glands  of  the  stomach,  but  is  a  true  tenesmus  of 
the  stomach  of  nervous  origin.  I  have  known  it  to  continue  for  ten 
days,  reducing  the  patient  rapidly  in  weight  and  making  it  necessary 
to  sustain  life  by  nutritive  enema ta.  Pain  is  felt  constantly  in  the 
epigastrium,  occasionally  also  between  the  shoulder-blades,  and  may 
encircle  the  body  like  a  band.  There  is  usually  considerable  tender- 
ness in  the  epigastrium,  the  stomach  rejects  all  food ;  then  the  act  of 
vomiting  is  either  futile  or  mucus  and  bile  are  rejected ;  occasionally 
hemorrhasres  occur  in  the  stomach,  and  the  vomited  material  contains 
so-called  coffee-grounds.  The  vomiting  is  always  accompanied  by 
intense  nausea,  by  great  weakness,  frequently  by  pains  running  down 
the  arms,  and  oppression  across  the  chest,  by  palpitation  of  the  heart, 
and  by  vertigo.  It  is  not  uncommon  for  the  pains  of  locomotor  ataxia 
to  develop  in  the  legs  during  the  gastric  crisis,  and  if  the  crisis  occurs 
in  the  course  of  an  ordinary  case  these  pains  are  usually  intensified 
during  the  crisis.     The  agony  attendant  upon  such  a  crisis  is  so  great 


SYMPTOMS.  359 

as  to  throw  the  patient  into  a  state  almost  of  delirium,  in  which  he 
cries,  contojjts  the  body,  and  suffers  all  the  agonies  of  dissolution. 
Hiccoughs  and  intestinal  or  rectal  crises  may  accompany  the  gastric 
crisis. 

Gastric  crisis  terminates  suddenly,  either  under  the  influence  of 
treatment  or  spontaneously,  leaving  the  patients  in  a  state  of  great 
prostration,  but  usually  with  increased  appetite.  Care  has  to  be  taken, 
however,  in  feeding  them  to  use  simple  and  easily  digested  food,  in 
order  to  avoid  a  return  of  the  crisis ;  but  when  all  pain  has  ceased  for 
twenty-four  hours  it  is  safe  to  begin  a  rapidly  increasing  systematic 
course  of  nourishment,  as  the  patient  needs  as  much  food  as  the  stomach 
can  absorb.  Forty-nine  out  of  four  hundred  and  fifty-six  cases  had 
gastric  crises  at  some  time  during  the  disease.  In  one  patient  under 
my  observation  such  a  gastric  crisis  occurred  at  intervals,  first  of  six 
months,  then  of  three  or  four  months,  for  a  period  of  four  years 
before  a  loss  of  knee-jerk  and  the  development  of  the  Argyll-Robert- 
son pupil  made  it  positive  that  the  condition  present  was  one  of  tabes. 
In  the  meantime  he  had  been  treated  by  all  forms  of  diet,  by  lavage, 
etc.,  in  vain.  The  immediate  use  of  hypodermics  of  morphine  in 
large  amount  on  the  onset  of  the  crisis  seems  to  cut  short  each  attack, 
though  they  have  continued  to  the  present  time  at  intervals,  even  now 
when  the  general  symptoms  of  the  disease  are  quite  evident. 

Intestinal  and  rectal  crises  are  less  common  than  gastric  crises,  but 
may  also  be  the  first  signs  of  a  locomotor  ataxia.  They,  however, 
usually  occur  in  the  second  stage.  They  begin  with  severe  pains  in  the 
bowels  or  in  the  rectum,  are  attended  by  a  watery  diarrhoea  with  great 
tenesmus  and  rapid  exhaustion,  and  usually  by  great  thirst.  These 
attacks  may  continue  for  two  or  three  days,  every  attempt  at  taking 
fluid  or  food  being  followed  immediately  by  an  evacuation  of  the 
bowels.  After  the  ordinary  contents  are  discharged,  mucus  or  serum 
is  found  in  the  discharges.  These  attacks  cease  suddenly,  but  leave 
the  patient  in  a  state  of  great  prostration. 

Laryngeal  crises  are  next  in  frequency  to  gastric  crises.  The  patient 
is  usually  seized  by  a  sudden  and  severe  cough,  becomes  hoarse,  and 
has  great  difficulty  in  breathing,  on  account  of  an  adductor  spasm  of 
the  larynx.  The  cough  is  a  typical  nervous  cough,  very  loud  and 
harsh,  is  accompanied  by  dyspncBa,  and  the  attacks  are  very  severe  and 
occur  every  hour  or  two  for  several  minutes  at  a  time  and  after  a  short 
duration  are  usually  attended  by  great  frequency  of  respiration.  They 
may  be  attended  by  attacks  of  gaping.  Physical  examination  of  the 
lungs  fails  to  reveal  any  evidence  of  bronchitis,  but  after  the  attack 
has  lasted  for  some  time  inspection  of  the  larynx  usually  reveals  a  con- 
gestion of  the  vocal  cords,  and  not  infrequently  the  intensity  of  the 
cough  gives  rise  to  a  secretion  of  mucus.  The  attacks  cease  as  sud- 
denly as  they  appear,  and  are  thus  manifestly  of  nervous  origin.  But 
eight  of  my  four  hundred  and  fifty  patients  had  laryngeal  crises. 

Charcot  described  a  form  of  laryngeal  crisis  which  he  termed  laryn- 
geal vertigo,  in  which  the  patient  feels  a  tickling  or  feeling  of  heat  in 
the  throat,  followed  by  a  sense  of  suffocation  and  noisy  wheezing  breath- 


360  LOCOMOTOB   ATAXIA. 

ing.  This  is  immediately  followed  by  a  sense  of  vertigo,  and  the  patient 
often  falls  fainting  and  unconscious  to  the  ground.  In  some  persons, 
especially  those  of  a  very  nervous  constitution,  Oppenheira  has  dis- 
covered that  pressure  upon  the  hyoid  bone  near  to  the  larynx  may 
produce  reflex  attacks  not  unlike  those  occurring  in  laryngeal  vertigo 
and  in  laryngeal  crisis.  The  fact  should  be  remembered  if  patients 
show  these  symptoms,  in  order  that  they  may  avoid  wearing  a  tight 
collar  or  compressing  the  neck. 

Pharyngeal  crises  have  been  observed  by  Oppenheim.  They  con- 
sist of  painful,  rapidly  occurring  acts  of  swallowing,  and  as  each  act 
of  swallowing  is  accompanied  by  the  passage  of  a  certain  amount  of 
gas  into  the  stomach,  they  are  followed  by  attacks  of  belching.  The 
patient  may  swallow  as  many  as  twenty-four  times  a  minute  and  the 
attack  many  last  for  ten  minutes,  giving  rise  to  great  distress  and  a 
sense  of  nausea.  Attacks  of  hepatic  colic  with  pain  similar  to  that  of 
the  passage  of  a  gallstone,  and  attacks  of  renal  colic  with  pain  similar 
to  that  of  the  passage  of  a  renal  calculus,  occurring  in  the  course  of 
tabes  have  been  described  as  hepatic  and  nephritic  crises.  But  these 
are  extremely  rare,  and  their  nervous  origin  is  questionable. 

Vesical  and  urethral  crises  are  less  common.  They  are  attended  by 
severe  pain  in  the  region  of  the  bladder  and  in  the  urethra,  occurring 
like  colic  in  a  series  of  sudden  attacks  with  an  intense  desire  to  empty 
the  bladder  which  may,  however,  not  be  successful.  But  one  of  my 
patients  had  this  symptom. 

The  French  authors  describe  genital  crises  in  both  males  and  females, 
consisting  of  great  sexual  excitement  attended  by  sharp  pains  in  the 
organs.     I  have  never  seen  such  cases. 

Attacks  of  angina  pectoris  or  pseudo-angina  have  given  rise  to  the 
supposition  that  cardiac  crises  may  occur  in  the  course  of  locomotor 
ataxia.  Patients  are  seized  suddenly  by  pain  in  the  heart  and  by  pain 
running  down  the  left  arm  ;  they  turn  pale,  suffer  much  from  dyspnoea 
and  distress,  with  great  mental  anxiety,  occasionally  faint  away,  but 
gradually  recover,  though  the  attack  may  be  repeated  several  times  in 
twenty-four  hours  before  it  passes  away  completely.  Such  attacks  are 
always  attended  by  a  very  rapid  pulse,  which  is  not  infrequently 
irregular  and,  in  fact,  may  be  preceded  for  several  days  by  an  unduly 
rapid  heart  action.     The  cardiac  crises  are  extremely  rare. 

The  development  of  trophic  disturbances  in  the  joints  and  perforating 
ulcer  of  the  foot  rarely  occurs  as  an  early  symptom  of  locomotor 
ataxia;  they  are  much  more  common  as  complications  of  the  later 
stage  of  the  disease,  and,  therefore,  will  be  considered  after  the  symp- 
toms of  the  second  stage  have  been  studied. 

The  Second  or  Ataxic  Stage.  —  The  second  stage  of  tabes  has  been 
termed  the  stage  of  ataxia  and,  as  a  rule,  ataxia  only  develops  after  a 
preceding  stage  of  pain  or  after  the  onset  of  some  of  the  more  unusual 
early  symptoms  of  the  disease.  In  a  few  cases  (3  per  cent.),  however, 
I  have  seen  ataxia  appear  as  the  very  first  symptom  of  locomotor 
ataxia.     In  all  cases  of  the  disease,  however,  it  develops  finally,  and  is 


SYMPTOMS.  861 

one  of  the  most  important  and  characteristic  of  the  symptoms  of  the 
affection,  having  given  its  name  to  the  disease. 

Ataxia.  —  Ataxia  many  be  defined  as  imperfect  coordination  of  mus- 
cular action.  For  every  act  of  the  body  a  regular  succession  of  move- 
ments in  the  muscles  of  proper  degree  and  intensity  and  of  wide  dis- 
tribution is  necessary.  The  simplest  act,  such  as  closing  the  fist, 
crossing  the  knees,  or  the  more  complex  acts  of  rising  from  a  chair, 
standing  still,  taking  a  step,  or  the  finer  acts  of  writing,  or  buttoning 
the  clothing,  or  playing  a  musical  instrument,  really  involve  an  action 
in  almost  all  the  muscles  of  the  body,  for  there  must  be  a  proper 
fixation  of  the  joints  in  order  that  other  joints  may  act.  There  must 
be  a  proper  fixation  of  the  spine  in  order  that  the  balance  may  be  pre- 
served. There  must  be  a  proper  adjustment  of  one  side  of  the  body 
in  order  that  the  other  may  perform  its  movements.  Any  careful  study 
of  normal  action  will  reveal  at  once  the  highly  complex  process  which 
is  involved  in  every  movement,  and  the  length  of  time  that  is  required 
to  acquire  these  various  adjusted  movements  demonstrates  that  many 
difPerent  mechanisms  and  combinations  are  required  in  order  to  produce 
motor  effects  of  an  adjusted  character.  The  control  of  all  coordinated 
movements  involved  in  standing  and  walking  is  the  function  of  the 
cerebellum.  The  control  of  all  the  finer  movements  of  the  hands  is 
the  function  of  the  cerebrum ;  but  in  either  case  the  essential  condition 
of  a  proper  coordinated  act  is  the  reception  in  the  automatic  centres, 
whether  these  lie  in  the  spinal  cord,  the  medulla,  the  cerebellum,  or 
the  cerebrum,  of  sensory  impulses  coming  from  the  muscles,  skin,  and 
joints.  In  locomotor  ataxia  we  have  seen  that  the  lesion  cuts  off  such 
impulses  just  at  their  entrance  into  the  spinal  cord — the  sensory  tracts 
to  the  spinal  cord  being  affected  at  this  point — and  hence  impulses 
destined  for  the  gray  matter  of  the  cord,  for  the  medulla  by  way  of  the 
columns  of  Goll,  for  the  cerebellum  by  way  of  the  columns  of  Clarke 
and  direct  cerebellar  tract,  and  for  the  cerebrum  by  way  of  the  columns 
of  Goll  and  Burdach,  the  lemniscus,  and  the  internal  capsule  are  all 
interrupted.  Reference  to  the  diagram  (Plate  I.)  will  show  that  a 
lesion  of  the  posterior  nerve  roots  deprives  the  nerve  centres  of  all 
information  with  regard  to  the  position  of  the  limbs,  the  degree  of 
tension  of  the  muscles,  and  of  all  those  facts  which  go  to  condition 
coordinated  action  and  equilibrium.  Hence  it  is  to  be  expected  that 
in  posterior  sclerosis  ataxia  will  develop  and  will  be  exactly  propor- 
tionate in  degree  to  the  degree  of  the  sclerosis. 

Ataxia  is  due  to  a  loss  of  a  large  number  of  different  sensations.  It 
is  partly  due  to  anaesthesia  of  the  skin,  and  partly  to  a  loss  of  muscular 
sense,  the  sense  which  is  derived  from  the  muscle  spindles  in  the 
muscles  and  tendons.  This  is  proven  by  the  fact  that  in  almost  all 
cases  of  ataxia  a  diminution,  or  even  loss  of  muscular  sense,  can  be 
demonstrated.  This  is  the  sense  by  which  the  situation  and  move- 
ments of  the  limbs  are  appreciated  and  by  which  differences  of  pressure 
and  weight  are  perceived.  It  must  be  clearly  distinguished  from  the 
sense  of  effort  which  is  a  function  of  the  cortex  and  is  a  centrifugal 
rather  than  a  centripetal  function.     In  almost  all  cases  of  ataxia  care- 


362  LOCOMOTOR   ATAXIA. 

ful  examination  will  demonstrate  that  the  patient  is  not  sure  of  the 
exact  position  of  his  extremities  or  of  their  distal  parts ;  cannot  with 
his  eyes  closed  reproduce  in  one  hand  or  one  foot  artificial  positions 
given  to  the  other ;  cannot  distinguish  small  differences  in  weights,  as 
he  should  in  health,  and  instinctively  guides  his  movements  by  the 
sense  of  sight  rather  than  by  the  sense  of  muscular  feeling.  Another 
important  element  entering  into  the  ataxia  is  the  sensation  derived 
from  the  joints  and  ligaments  of  the  joints.  These  sensations  undoubt- 
edly enter  into  the  adjustment  of  motions,  especially  the  sensations 
derived  from  the  vertebrae.  It  is  therefore  evident  that  the  symptom 
of  ataxia  is  due  to  the  cutting  off  of  numerous  different  forms  of  sen- 
sation reaching  the  spinal  cord,  but  transmitted  through  it  to  the 
various  automatic  centres  which  control  adjusted  movements. 

The  symptom  of  ataxia  is  shown  by  awkwardness  of  motion,  de- 
veloping gradually  and  usually  beginning  in  the  legs.  A  patient  first 
notices  that,  on  closing  the  eyes  in  the  act  of  washing,  or  in  rising  in 
the  night  and  attempting  to  move  about  in  the  dark,  he  is  unsteady 
on  his  feet,  sways  unduly,  and  even  loses  his  balance.  He  then 
notices  that  in  attempting  to  dance  or  in  attempting  to  walk  a  straight 
line  he  sways  unduly.  Then  he  finds  that  in  going  up  stairs,  in  rising 
from  a  chair,  in  starting  off  to  walk,  in  turning  suddenly  while  walk- 
ing, or  in  attempting  to  step  with  precision  upon  a  car  or  into  a 
cab  his  feet  are  clumsy  and  he  stumbles.  If  at  this  stage  or  in  the 
stage  of  pain,  even  before  the  patient  has  noticed  any  difficulty  in 
walking,  he  is  asked  to  stand  with  his  feet  tight  together  and  his  eyes 
closed,  he  will  be  found  to  sway  unduly.  This  is  the  so-called  Rom- 
berg sign,  having  first  been  described  by  Romberg  in  1852.  If  this 
act  of  standing  with  eyes  closed  be  carefully  observed  it  will  be  noticed 
that  irregular  contractions  are  constantly  occurring  in  the  anterior  and 
posterior  tibial  muscles  and  in  the  muscles  of  the  feet,  unusual  muscu- 
lar effort  of  a  wholly  automatic  character  apparently  being  called  into 
play  to  aid  in  this  involuntary  act. 

The  Gait  in  Locomotor  Ataxia. — As  the  disease  advances  the  gait 
becomes  much  disturbed  and  a  typical  gait  develops.  The  steps  are 
irregular  in  their  length,  the  feet  often  being  placed  too  far  apart; 
the  legs  are  thrown  about,  their  muscular  act  being  done  to  excess  and 
without  proper  degree.  The  feet  are  thrown  forward  and  lifted  high  ; 
they  come  down  with  a  slap  upon  the  floor,  and  it  often  seems  as  if  the 
joints  were  too  loose,  the  legs  being  thrown  about  with  a  flail-like 
action.  In  the  later  stages  of  the  disease  the  knees  are  frequently 
bent  backward  in  the  acts  both  of  standing  and  walking,  and  the 
ankles  may  turn.  In  rising  from  a  chair  such  a  patient  is  apt  to  lean 
forward  unduly  and  sway  backward  and  forward  for  a  moment  before 
gaining  his  proper  balance.  He  has  difficulty  in  starting  off,  usually 
taking  hold  of  some  adjacent  object  or  steadying  himself  by  a  cane. 
These  patients  uniformly  watch  the  action  of  their  feet  carefully,  and 
many  who  can  balance  themselves  fairly  by  the  aid  of  eyesight  are 
wholly  unable  to  walk  in  the  dark  or  when  blindfolded.  This  con- 
dition is  termed  static  ataxia  in  distinction  from  motor  ataxia,  which  is 


SYMPTOMS. 


863 


Fig.  159. 


a  state  of  incoordination  developing  in  movements  not  connected 
with  standifSg.  Any  test  which  will  involve  the  performance  of  a 
carefully  adjusted  movement  will  reveal  the  uncertainty  of  motion  in 
these  patients.  The  common  test  for  motor  ataxia  is  requesting  the 
patient  to  follow  a  line  on  the  carpet  with  his  toe,  to  touch  the  toe  to 
the  finger  of  the  examiner  held  in  diiferent  positions,  to  touch  one 
heel  to  the  opposite  knee,  to  cross  the  legs  slowly,  making  the  toe  de- 
scribe a  complete  circle  in  the  air. 

The  hands  may  also  become  ataxic.  Tests  applied  to  the  hands  may 
be  made  by  asking  the  patient  to  touch  some  object — his  nose  or  the 
opposite  ear — with  the  tip  of  the  finger,  with  his  eyes  closed,  or  to 
perform  some  simple  act,  such  as  buttoning  his  clothing  or  writing. 
These  tests  will  reveal  the  beginning  ataxia  in  the  hands  when  the 
patient  perhaps  is  not  aware  of  any  disturbance  in  them.  Later  on, 
when  the  motor  ataxia  is  well  devel- 
oped, the  action  of  the  hands  is  quite 
characteristic.  The  patient  extends 
the  fingers  widely  in  making  any 
attempt  at  grasping,  takes  hold  of 
objects  too  tightly,  thereby  crushing 
delicate  objects  unintentionally ;  has 
great  difficulty  in  buttoning  his 
clothing,  and  the  awkwardness  of 
movement  is  apparent  in  every  act 
of  the  hands.  In  the  later  stages  he 
may  be  reduced  to  such  a  condition 
of  ataxia  as  to  be  unable  to  write,  or 
to  feed  himself,  or  to  dress  himself. 
If  he  is  told  to  pick  up  a  small  object 
there  may  be  not  only  an  undue  open- 
ing of  the  fingers,  but  irregular  and 
unexpected  jerks  of  the  elbow  and 
shoulder. 

When  the  ataxia  is  well  developed 
tests  demonstrate  an  actual  loss  of 
the  power  of  perceiving  differences  of 
weight.  Thus  the  patient  may  be 
unable  to  distinguish  between  a  penny 
and  a  half-dollar  in  the  two  hands, 
and  when  tested  more  accurately  by 
means  of  balls  of  various  weights  will 
show  a  variation  from  the  normal 
equations.  The  sense  of  pressure  may  also  be  diminished,  so  that  when 
objects  are  piled  upon  the  supported  hand,  such  as  two  or  three  books, 
the  patient  will  be  unable  to  distinguish  between  differences  that  should 
be  apparent. 

Tests  also  will  reveal  a  lack  of  knowledge  of  the  position  of  the 
limbs  in  space  when  the  eyes  are  closed.  If  the  finger  or  toe  be  grasped 
by  the  examiner  on  two  sides  and  movements  conveyed  to  the  joints, 


Abnormal  position  of  the  knees  in  standing 
in  locomotor  ataxia.     (Dejerine.) 


364  LOCOMOTOE  ATAXIA. 

the  patient  may  be  unable  to  tell  whether  the  finger  or  toe  is  extended 
or  flexed.  If  the  grasp  is  made  on  front  and  back  of  toe  or  finger  and 
the  pressure  sense  is  preserved,  this  may  convey  the  desired  informa- 
tion, and  the  test  be  imperfect.  This  loss  of  the  sense  of  position  may 
be  so  extreme  that  the  patient  is  unable  to  say  which  leg  is  placed  over 
the  other.  He  may  lose  his  legs  in  bed  and  be  unaware  that  one  is 
hanging  out  and  is  exposed  to  the  cold.  One  patient  of  mine  always 
had  to  be  carefully  helped  into  the  carriage,  as  he  was  liable  to  leave 
one  leg  hanging  out  of  the  door.  In  the  most  extreme  form  of  ataxia 
the  patient  is  wholly  unable  to  stand  or  to  walk  or  to  use  his  hands, 
being  thus  apparently  incapacitated  for  all  movement ;  and  he  is  then 
said  to  be  in  the  stage  of  paralysis.  Thus  the  stage  of  ataxia  may  go 
on  slowly  and  merge  into  that  of  paralysis  without  there  being  any  sud- 
den transition  between  the  two  stages. 

Hypotonia.  —  A  condition  of  the  muscles  characterized  by  a  loss  of 
the  normal  tone  and  a  tendency  to  over-stretching  when  pulled  has 
been  observed  in  many  cases  of  locomotor  ataxia,  and  has  been  named 
hypotonia.  It  often  increases  the  ataxia  of  movement.  It  leads  to  an 
undue  mobility  of  the  joints,  the  muscles  no  longer  holding  the  articular 
surfaces  together  and  it  is  the  cause  of  such  deformity  as  is  shown  in 
Fig.  159. 

Anaesthesia.  —  When  the  stage  of  ataxia  is  fully  developed  decided 
loss  of  sensation  is  usually  present.  The  first  sense  to  be  lost  is  usually 
the  sense  of  pain.  Irritation  of  the  skin  by  a  pin  or  by  a  needle  will 
develop  an  inability  to  perceive  painful  sensations  or  a  very  marked 
delay  in  their  perception.  Thus  several  seconds  may  elapse  from  the 
impact  of  the  needle  to  the  perception  of  pain,  and  the  location  of  the 
pain  may  be  erroneous.  Usually  the  painful  sensation  is  erroneously 
located  at  a  level  somewhat  higher  than  that  at  which  it  really  occurs. 
The  impairment  of  the  sense  of  pain  leads  very  often  to  the  neglect  of 
slight  injuries,  especially  injuries  about  the  joints,  and  it  is  undoubtedly 
the  cause  of  the  development  of  many  trophic  disturbances.  This  lack 
of  sense  of  pain  may  also  prevent  the  patient  from  noticing  the  painful 
effects  of  heat  or  cold,  even  when  these  produce  injuries  to  the  skin. 
The  analgesia  is  usually  attended  by  thermo-ansesthesia,  in  which  the 
patient  loses  the  sensation  of  temperature  both  to  heat  and  to  cold.  It 
is  my  experience  that  cold  sensations  are  usually  well  perceived  and 
that  the  patients  are  intensely  sensitive  to  all  cold  applications,  even 
during  the  well-marked  stage  of  ataxia.  But  heat  is  very  often  not 
duly  perceived,  and  very  hot  objects  may  seem  only  slightly  warm ; 
hence  the  patients  are  in  danger  or  being  burned  if  they  are  allowed  to 
take  hot  baths  or  if  any  hot  objects,  such  as  bags,  are  laid  upon  the 
body.  The  sensation  of  temperature  may  also  be  delayed  in  the  early 
stage,  even  when  felt. 

Sensations  of  tickling  and  of  vibration  as  tested  with  a  tuning  fork 
are  commonly  impaired  in  the  stage  of  ataxia. 

While  anaesthesia  is  a  rather  rare  symptom  in  the  stage  of  pain,  it  is 
always  present  in  some  degree  in  the  stage  of  ataxia.  The  distribution 
of  this  anaesthesia  corresponds  to  the  distribution  of  the  sensory  nerve 


SYMPTOMS. 


365 


roots  and  does  not  correspond  to  the  distribution  of  the  peripheral 
nerves.^  Thus  anaesthesia  is  very  commonly  delayed  on  the  outer  sur- 
face of  the  legs,  in  the  third  lumbar  area  (Plate  VII.),  in  the  outer 
half  of  the  feet,  or  only  on  the  inner  half  of  the  foot  in  the  first  sacral 
area.  Or  it  may  deveolp  on  the  anterior  surface  of  the  thighs  in  the 
second  lumbar  area.  It  has  a  tendency  to  extend  from  one  segmental 
area  of  the  legs  to  another  until  the  entire  lower  extremities  are  partially 
anaesthetic.  The  anaesthesia  sometimes  may  appear  high  up  on  the 
trunk  and  on  the  inner  surface  of  the  arm  from  the  axilla  downward, 
even  when  the  trunk  shows  no  sign  of  anaesthesia.  An  insensitiveness 
to  pressure  upon  the  nerve  trunk  attends  the  anaesthesia,  so  that  pressure 
over  the  peripheral  nerve  as  it  curves  around  the  fibula,  while  pro- 
ductive of  tingling  in  the  foot,  is  not  attended  by  pain  ;  and  pressure 
upon  the  ulnar  nerve  at  the  elbow,  while  productive  of  tingling  in  the 
fingers,  is  not  attended  by  pain.  This  latter  symptom  was  first  pointed 
out  by  Biernacki. 

Fig.  160. 


Disease  of  both  knee-joints  and  both  ankles  in  locomotor  ataxia. 


Joint  Diseases.  —  An  unusual  laxity  of  the  joints  is  not  uncommon 
in  the  stage  of  ataxia.  The  ankles  are  turned  unduly  in  walking, 
either  in  or  out,  the  patient  frequently  stepping  upon  the  side  of  the 
foot  without  being  aware  of  it.  The  knees  appear  to  be  relaxed  so  far 
as  all  muscular  tension  about  them  is  concerned  and  turned  backward 
in  the  act  of  standing  or  in  the  act  of  walking.  (Fig.  160.)  The 
1  See  Leahr.     Arch.  f.  Psych.,  Bd.  xxvii.,  688,  and  Bd.  xxix.,  648. 


866 


LOCOMOTOB   ATAXIA. 


thighs  appears  to  be  loose,  so  that  subluxation  often  appears  to  be 
imminent.  The  same  is  true  of  the  joints  of  the  fingers,  of  the  wrists, 
and  of  the  elbows,  and  I  have  never  seen  a  case  of  well-marked  ataxia 
in  which  undue  extension  of  the  joints  was  not  easily  possible. 

It  is  this  relaxation  of  the  ligaments,  together  with  the  absence  of 
the  signs  of  pain  on  the  production  of  such  unusual  positions,  which 
are  probably  the  active  causes  of  the  development  of  the  joint  diseases 
of  locomotor  ataxia,  although  these  joint  affections  are  commonly  termed 
trophic  complications.  They  have  been  named  Charcot  joints,  as  he 
was  the  first  to  describe  them.  Patients  very  commonly  do  not  per- 
ceive the  beginning  of  these  joint  affections,  and  only  apply  to  a  phy- 
sician when  the  joint  is  enormously 
Fig.  161.  swollen  and  full  of  fluid.     Some  trau- 

matism is  undoubtedly  the  active  cause 
of  the  development  of  Charcot  joints. 
I  have  seen  them  very  commonly  in 
clinic  cases  but  very  rarely  among  the 
higher  classes,  who  are  not,  as  a  rule, 
exposed  to  injuries.  Among  one  hun- 
dred and  twenty-six  private  patients 

Fig.  162. 


Disease  of  both  hip-joints,  with  disloca^ 
tion,  in  locomotor  ataxia.  (Icon,  de  la  Sal- 
petrifere. ) 


Arthropathies  in  shoulder  and  elbow  in  locomotor 
ataxia.     (Dejerine.) 


four  had  an  affection  of  the  knee,  two  of  the  ankle,  two  of  the  wrist,  and 
one  of  the  toe.  The  joints  affected  are  most  frequently  the  knee-joints 
or  the  ankle,  though  the  elbow  and  the  wrist  may  be  involved.  The 
small  joints  of  toes  and  fingers  are  rarely  affected.  Several  forms  of 
joint  disease  may  develop,  but  it  is  to  be  remarked  that  these  diseases 
do  not  follow  the  typical  forms  of  arthritis  and  that  they  are  not 
attended  by  pain.     The  first  symptom  is  usually  an  efiiision  within 


SYMPTOMS. 


367 


Fig.  163. 


the  joint  -which  goes  on  rapidly  until  the  cavity  of  the  joint  is  enor- 
mously dis^nded  by  fluid.  Thus  the  knee  may  be  twice  the  size  of 
the  unaflPected  knee,  or  the  ankle  may  be  twice  the  size  of  the  unaffected 
ankle.  The  distention  of  the  joint  with  fluid  separates  the  articular 
surfaces  of  the  bones,  which  thus  play  irregularly  upon  one  another, 
and  then  pathological  changes  develop  in  the  articular  surface  and  in 
the  bones  themselves.  The  tissue  all  about  the  joint  may  also  become 
infiltrated  with  fluid  and  cedematous  and  pit  upon  pressure.  There  is, 
however,  no  heat,  no  redness,  no  tenderness  of  the  parts,  and  no  pain. 
In  mild  cases  effusion  gradually  subsides  under  rest  and  the  joint 
returns  to  its  normal  form,  cartilage  and  bones  not  having  been  per- 
manently affected.  In  other  cases  an  enormous  thickening  develops  in 
the  ends  of  the  bones  and  in  the  cartilages,  and  a  permanently  enlarged 
joint  that  is  only  partly  useful  remains.  If  the  process  goes  further 
this  enlargement  is  followed  by  a  progressive  atrophy,  by  disintegration 
and  disappearance  of  the  cartilage,  by  erosion  of  the  end  of  the  bone, 
no  trace  of  the  articular  surface  finally  remaining.  In  this  last  stage 
unusual  mobility  of  the  joints  follows,  and  the  anterior  surface  of  the 
leg  may  be  laid  upon  the  anterior  surface  of  the  thigh  without  pain,  or 
the  hips  may  be  placed  in  all  sorts  of  positions  without  resistance.  In 
cases  where  trophic  disturbances  of  the  joints  occur  it  is  not  uncommon 
to  find  an  undue  friability  of  the  bones,  so  that  spontaneous  fractures 
on  very  slight  injury  or  muscular  strain  are 
produced  in  the  long  bones,  especially  of  the 
lower  limbs.  Pathological  examination  has 
usually  shown  a  dilatation  of  the  Haversian 
canals  in  the  long  bones  in  these  states,  with 
a  thinning  and  fragility  of  the  compact  sub- 
stance. This  has  led  to  the  general  accept- 
ance of  the  theory  of  a  primary  trophic 
disturbance.  Charcot's  statement  that  the 
affection  of  the  joints  cannot  be  entirely  ex- 
plained in  every  case  by  traumatism  or  by 
neglect  of  painful  affections  must  therefore 
be  admitted. 

Another  trophic  disturbance  closely  allied 
to  joint  affection  is  a  permanent  distortion  of 
the  foot  which  develops  gradually  and  resem- 
bles club-foot.  A  hard  swelling  usually 
appears  upon  the  back  of  the  foot,  and  the 
inner  surface  of  the  foot  becomes  more  promi- 
nent on  account  of  an  adducted  and  inverted 
position,  and  arch  of  the  foot  becomes  flat, 
the  toes  are  extremely  flexed,  the  whole  foot  is  shortened,  and  in  at- 
tempts to  walk  the  weight  rests  upon  the  outer  surface  of  the  foot. 
It  is  probable  that  this  is  due  to  a  combination  of  factors,  the  lack  of 
muscular  adjustment,  the  relaxation  of  the  ligaments,  and  a  trophic 
disturbance  of  the  bones  of  the  foot  coinciding  to  produce  it. 

Another  trophic  affection   which   is  present  in  a  few  cases  in  the 


Perforating  ulcer  of  the  foot  in  loco- 
motor ataxia.     (Obersteiner.) 


368  LOCOMOTOB   ATAXIA. 

ataxic  stage  of  the  disease  is  the  so-called  perforating  ulcer  of  the  foot. 
It  may  originate  in  a  neglected  corn  or  bunion,  it  may  be  due  to  an 
injury  of  one  of  the  small  bones  of  the  foot,  with  consequent  caries 
and  suppuration,  or  it  may  be  due  to  a  neglected  hemorrhage  (so-called 
"  stone  boil").  Whatever  the  origin,  the  lesion  is  a  small  sinus  usually 
situated  on  the  ball  of  the  great  toe,  or  between  the  great  and  next 
toe,  or  under  the  little  toe,  which  sinus  secretes  a  thin  ichorous  fluid 
and  refuses  to  heal  under  surgical  treatment.  (Fig.  163.)  In  the  vast 
majority  of  cases  the  absence  of  pain  leads  the  patient  to  take  no 
notice  of  the  foot  until  the  ulcer  is  fully  formed,  and  then  examination 
shows  a  sinus  leading  down  to  a  bit  of  carious  bone  wholly  insensitive 
and  surrounded  on  the  surface  by  an  ulcerated  dermal  tissue  and 
deeper  by  granulating  tissue.  Occasionally  a  gangrenous  spot  may 
form.  Scraping  this  ulcer  or  its  sinus,  injecting  it  with  antiseptic 
solutions  or  with  iodine  usually  fails  to  be  followed  by  healing,  and 
occasionally,  if  the  disease  gives  rise  to  great  discomfort,  which  is 
rare,  amputation  of  the  toe  or  exsection  of  the  joint  may  be  necessary. 
I  have  known  such  an  ulcer  to  be  neglected  by  the  patient,  as  it  caused 
no  pain  for  several  years  and  produced  no  deleterious  effects. 

Peculiar  Skin  Affections  are  occasionally  mentioned  as  complicating 
trophic  disorders  in  the  second  stage  of  ataxia.  These  are  usually  of 
the  form  of  herpetic  eruptions  which  may  be  quite  extensive  in  the 
legs  or  about  the  body,  or  of  the  form  of  subcutaneous  ecchymoses,  or 
quite  widespread  effusions  under  the  skin.  Pemphigus  has  also  been 
observed.  Lelow  has  described  a  condition  of  thickening  of  the  skin 
with  loss  of  color  and  desquamation  not  unlike  ichthyosis.  An  im- 
perfect growth  of  the  nails  with  unusual  ridging,  peculiar  curvature,  or 
falling  out  of  the  nails  is  not  a  very  uncommon  complicating  trophic 
disturbance.     Here  again,  however,  traumatism  is  the  usual  cause. 

Among  trophic  disturbances  described  loosening  and  falling  of  the 
teeth  have  been  mentioned  with  or  without  severe  toothache.  In  a  few 
cases  this  condition  has  gone  on  to  a  necrosis  of  the  bone  of  the  jaw 
with  atrophy  of  the  alveolar  process. 

Irregular  atrophy  in  the  various  muscles  of  the  body,  especially  in 
the  muscles  supplied  by  the  external  popliteal  nerve  in  the  legs  and 
the  ulnar  nerve  in  the  hands,  has  been  observed  in  a  number  of  cases. 
These  localized  atrophies  are  undoubtedly  due  to  a  complicating  neu- 
ritis. In  the  last  stage  of  the  disease,  however,  when  the  patient  is 
confined  to  the  bed,  the  entire  muscular  system  may  become  atrophied. 
And  in  some  of  these  cases  a  complicating  amyotrophic  lateral  sclerosis 
has  been  found  on  examination  of  the  spinal  cord.  The  atrophy  is  a 
slowly  advancing  one,  and  is  attended  by  paralysis  and  by  reaction  of 
degeneration.  Sudden  transient  palsies  have  been  described  as  occur- 
ring in  tabes  at  any  of  the  stages  of  the  disease  as  similar  to  the 
muscular  palsies  in  the  eyes.  These  may  be  due  to  a  complicating 
neuritis  which  subsequently  recovers. 

Even  in  the  stage  of  ataxia  the  muscular  strength  of  the  patient 
may  be  good,  though  the  lack  of  coordination  may  make  it  apparent 
that  he  has  a  true  paralysis.     It  is  only  in  the  last  stage  or  stage  of 


SYMPTOMS.  369 

paralysis  that  any  real  weakness  of  the  muscles  develops.  Erb  affirms 
that  in  tho^early  stage  the  electrical  contractility  of  the  muscles  is 
slightly  increased  both  to  faradism  and  galvanism,  especially  in  the 
region  of  the  peronei,  and  that  in  the  later  stages  there  is  a  diminution 
of  electrical  contractility  in  these  muscles,  but  never  a  reaction  or  de- 
generation. 

It  is  not  so  easy  to  explain  the  sudden  occurrence  of  hemiplegia  in 
tabes,  although  cases  have  been  recorded  in  which  this  has  appeared 
both  in  the  early  and  in  the  later  stages  of  the  disease,  being  tempo- 
rary in  character  and  occasionally  accompanied  by  epileptiform  con- 
vulsions. It  seems  likely,  however,  that  such  attacks  of  hemiplegia, 
unless  developing  as  a  symptom  of  general  paresis,  are  wholly  inde- 
pendent of  the  disease. 

The  general  nutrition  of  the  patient  with  tabes  usually  suffers  in  the 
course  of  the  case  from  time  to  time.  In  the  early  stage  a  rapid  loss 
of  weight  with  anaemia  is  not  at  all  uncommon,  and,  as  a  rule,  all  the 
symptoms  of  the  disease  are  exaggerated  during  this  period  of  tem- 
porary malnutrition.  If,  by  artificial  means — good  food,  assistants  to 
digestion,  tonics,  change  of  climate,  baths — this  failing  nutrition  be 
arrested  and  the  patient  begin  to  gain  in  weight  and  strength,  the 
symptoms  of  the  disease  will  gradually  subside,  and  thus  it  is  evident 
that  the  general  condition  of  the  patient  has  a  great  deal  to  do  with 
the  degree  of  suffering  that  he  undergoes  during  the  course  of  locomotor 
ataxia.  Hence  the  very  great  importance  of  supervising  the  care  of 
the  general  health  in  tabetic  patients,  and  whenever  a  severe  onset  of 
symptoms,  either  an  exaggeration  of  pain  or  rapid  development  of 
ataxia,  ensues,  every  means  should  be  used  to  increase  the  general 
nutrition. 

Mental  Symptoms.  —  In  a  certain  number  of  cases  of  locomotor 
ataxia  sudden  attacks  of  insanity  have  been  recorded.  These  usually 
take  the  form  of  acute  maniacal  excitement  or  of  the  less  alarming  con- 
dition of  delusions  of  grandeur  in  the  form  of  delusions  of  recovery  from 
the  disease,  of  unusual  conditions  of  health,  of  unusual  mental  capacity, 
or  of  unusual  wealth.  Such  insane  states  may  last  for  several  weeks 
and  then  gradually  subside,  leaving  the  patient  in  a  perfectly  normal 
state  of  mind  with  a  fair  recollection  of  what  has  occurred.  I  have 
watched  one  such  case  in  which  all  the  symptoms  suggested  paresis, 
but  in  which  their  disappearance  in  the  course  of  three  months  and 
their  failure  to  return  during  the  subsequent  six  years  convinced  me 
that  the  condition  was  not  paresis.  This  is  a  very  rare  complication, 
and  must  undoubtedly  be  ascribed  to  some  intercurrent  affection,  either 
of  a  toxic  character  or  a  psychosis  developing  in  the  course  of  tabes. 
In  a  second  group  of  cases  it  is  the  first  sign  of  a  beginning  general 
paresis,  which  may  go  on  through  its  typical  stages  and  lead  to  dementia 
and  paralysis.  I  have  seen  this  many  times.  There  is  undoubtedly  a 
close  connection  between  tabes  and  general  paresis,  for  not  only  may 
tabetics  develop  general  paresis,  but  many  paretics  develop  symptoms 
of  tabes.  Hence  the  development  of  mental  complications  should  give 
24 


370  LOCOMOTOB   ATAXIA. 

rise  to  the  suspicion  that  the  tabetic  patient  is  about  to  develop  general 
paresis. 

The  third  condition  under  which  mental  complications  may  arise  is 
a  condition  of  widespread  syphilitic  endarteritis  of  the  cortex,  and, 
inasmuch  as  syphilis  is  such  a  common  cause  of  tabes,  this  pathological 
condition  is  to  be  expected.  Such  a  syphilis  of  the  brain  may  produce 
all  the  symptoms  of  paresis,  but  may  yield  to  antisyphilitic  treatment, 
with  the  result  that  the  symptoms  will  gradually  disappear,  leading 
possibly  to  some  disturbances  of  memory  or  some  lack  of  power  of  self- 
control.  It  is  evident,  therefore,  that  the  occurrence  of  mental  symp- 
toms in  the  course  of  tabes  may  be  due  to  several  different  causes. 

Rare  Symptoms.  —  A  few  symptoms  remain  to  be  noticed  which 
have  been  recorded  as  developing  occasionally  in  the  course  of  tabes. 
They  are  certainly  extremely  rare. 

The  oculomotor  palsies  occasionally  recur  or  become  permanent  from 
the  outset  and  go  on  to  develop  all  the  symptoms  of  ophthalmoplegia 
externa  and  interna.  Under  these  circumstances  the  strabismus  and 
double  vision  become  permanent,  and  ptosis  is  added.  Very  often  an 
increase  of  the  difficulty  of  walking  is  a  result  of  the  double  vision. 
Some  form  of  ocular  palsy  was  present  at  some  period  in  one  quarter 
of  my  cases.     It  was  permanent  in  but  5  per  cent. 

Hemiatrophy  of  the  tongue  has  been  recorded  as  occasionally  de- 
veloping in  the  course  of  tabes. 

Bulbar  palsy  with  all  its  distressing  symptoms  has  been  recorded  as 
developing  occasionally  in  tabetic  patients  and  being  the  active  cause 
of  death. 

Symptoms  of  exophthalmic  goitre  have  been  known  to  develop  in 
tabetic  patients,  and  usually  have  a  worse  prognosis  than  in  the  ordi- 
nary types  of  the  disease,  the  exophthalmos  being  extreme,  the  goitre 
large,  the  tachycardia  constant,  and  the  nervous  tremor  of  the  head 
and  body  and  increased  sweating  being  very  distressing  to  the  patient. 

The  laryngeal  crises  occasionally  leave  a  condition  of  permanent 
adductor  palsy  of  the  larynx  which  results  in  difficulty  of  respiration 
that  may  make  tracheotomy  necessary. 

Muscular  atrophies  with  paralysis  may  develop  in  almost  any  muscle 
of  the  body. 

Involuntary  movements  either  athetoid  or  of  the  nature  of  spasmodic 
twitchings  of  the  muscles  have  been  described  by  various  observers  as 
an  occasional  symptom  in  tabes.^ 

In  a  few  cases  a  sudden  rupture  of  the  tendon  Achilles  has  been 
observed,  usually  occurring  after  extraordinary  efforts  of  the  ataxic 
patient  to  preserve  the  balance. 

Paralysis -agitans  has  been  known  to  develop  in  the  course  of  tabes. 

Many  authors  have  pointed  out  the  frequency  of  aortic  insufficiency 
with  stenosis  and  general  arterial  disease  in  tabetics.  In  one  hundred 
and  twenty-six  cases  I  found  an  aortic  murmur  in  nine.  This  is  prob- 
ably owing  to  the  fact  that  syphilis  is  the  common  cause  in  both  affec- 

*  Rhein,  Journal  of  Nervous  and  Mental  Diseases,  July,  1902. 


COUBSE.  371 

tions.     It  is  hardly  to  be  thought  that  there  is  any  necessary  connec- 
tion betwe^fti  the  diseases  of  the  bloodvessels  and  locomotor  ataxia. 

Many  symptoms  of  neurasthenia  and  of  hysteria  may  develop  in 
tabetic  patients,  but  these  are  to  be  ascribed  rather  to  the  mental  dis- 
tress of  long  suffering  than  to  any  direct  effect  of  the  lesion  in  the 
spinal  cord.  Occasionally  diabetes  develops  in  the  course  of  tabes. 
This  again  must  be  considered  an  accidental  occurrence. 

Stage  of  Paralysis.  —  The  stage  of  paralysis  is  spoken  of  as  the  third 
stage  of  tabes,  but  there  is  no  hard-and-fast  line  between  the  second 
and  third  stages.  When  ataxia  becomes  so  extreme  as  to  confine  the 
patient  to  his  chair  or  couch  he  may  be  said  to  have  entered  upon  the 
stage  of  paralysis.  In  this  stage  the  symptoms  hitherto  described  are 
exaggerated  in  intensity,  and  the  danger  of  a  fatal  termination  from 
complications  becomes  greater  than  in  the  second  stage.  As  time  goes 
on  various  more  unusual  symptoms  of  the  disease  are  actively  developed 
and  the  patient  in  the  stage  of  paralysis  presents  very  numerous  and 
diverse  symptoms.  The  chief  danger  of  death  is  from  a  complicating 
cystitis  or  pyelonephritis ;  from  extreme  exhaustion  owing  to  a  sudden 
crisis  of  some  character ;  from  general  exhaustion  due  to  malnutrition ; 
from  bulbar  paralysis,  or  from  some  intercurrent  disease.  As  a  matter 
of  fact.  Very  few  patients  die  of  locomotor  ataxia,  though  cases  have 
been  recorded  in  which  heart  failure  has  developed  and  carried  the 
patient  away  after  a  long  period  of  tachycardia.  The  vast  majority 
of  patients,  however,  die  of  some  intercurrent  disease.  And  for  this 
reason  many  patients  do  not  reach  the  stage  of  paralysis,  but  die  while 
in  the  stage  of  ataxia. 

Course.  —  The  course  of  the  disease  in  locomotor  ataxia  is  by  no 
means  a  constant  one.  While  the  majority  of  patients  develop  the 
first  stage  of  pain  and  go  on  slowly  after  three  or  four  years  to  the 
stage  of  ataxia,  and  remain  in  that  stage  with  gradually  increasing 
symptoms  for  fifteen  or  twenty  years,  then  becoming  paralyzed,  this 
typical  course  is  not  conformed  to  in  all  cases.  A  typical  history  of 
this  kind  was  obtained  in  but  eighty-seven  out  of  one  hundred  and 
twenty-six  private  cases. 

Occasionally,  after  a  short  stage  of  pain  or  without  any  preceding 
stage  of  pain,  the  symptoms  of  ataxia  develop  very  rapidly,  the 
patient  showing  extreme  incoordination  of  gait  within  three  or  four 
months  of  the  onset  of  the  disease.  This  was  the  history  in  four  of 
my  patients,  and  ataxia  developed  so  rapidly  as  to  be  classed  as  of 
acute  onset  in  six  other  patients.  It  developed  very  early  in  the 
disease  in  fourteen  other  cases.  Hence  the  early  appearance  of  ataxia 
may  be  taken  as  typical  in  about  20  per  cent,  of  the  cases.  The  same 
condition  of  acute  ataxia  may  be  due  to  an  acute  disseminated  myelitis, 
in  which  case  the  further  progress  of  the  disease  will  fail  to  reveal  the 
typical  symptoms  of  tabes.  Acute  ataxia  is  a  symptom  in  multiple 
neuritis,  but  the  history  of  the  causation  and  the  analysis  of  other 
symptoms,  together  with  the  progressive  course  to  recovery  in  these 
cases,  will  leave  no  doubt  about  the  diagnosis. 

There  is  a  type  of  case  in  which  blindness  upon  optic  atrophy  de- 


372  LOCOMOTOR    ATAXIA. 

velops  early.  The  patient  shows  no  other  signs  of  the  desease  for 
several  years,  then  loses  his  knee-jerk,  develops  slight  lightning  pains 
and  some  uncertainty  of  movement,  but  remains  for  many  years 
capable  of  going  about,  and  does  not  suifer  from  extreme  ataxia  until 
years  have  elapsed  since  the  occurrence  of  the  blindness.  This  history 
was  obtained  in  nine  cases. 

In  another  class  of  patients  gastric  crises  develop  as  an  early 
symptom,  but  are  found  to  be  associated  with  Argyll- Robertson  pupil 
and  loss  of  knee-jerk,  and  then,  after  several  years  of  paroxysmal 
attacks  of  vomiting  and  pain,  the  other  symptoms  of  locomotor  ataxia 
—  pains,  ocular  palsies,  and  ataxia — gradually  develop.  This  history 
was  obtained  in  six  cases. 

In  other  cases,  still,  the  occurrence  of  oculomotor  palsies  associated 
with  optic  atrophy,  rapid  pulse,  laryngeal  crises,  deafness,  and  bulbar 
symptoms  make  it  evident  that  almost  all  the  cranial  nerves  are  com- 
plicated without  much  aifection  of  the  spinal  cord.  It  is  true  that  in 
these  cases  the  Romberg  symptom  may  be  present  and  a  loss  of  knee- 
jerk  may  develop ;  but  the  ordinary  pains  and  ataxia  may  not  appear 
for  many  years  after  the  cranial  nerve  palsies  have  rendered  the  patient 
a  chronic  invalid.     This  history  was  obtained  in  five  cases. 

A  few  cases  have  been  described  of  so-called  "cervical  tabes"  in 
which  the  symptoms  of  pain,  numbness,  and  ataxia  have  gradually 
developed  in  hands  and  arms,  associated  with  Argyll-Robertson  pupil 
and  very  often  with  gastric  crises,  but  in  which  the  patients  have 
remained  for  many  years  perfectly  capable  of  walking  about,  without 
any  pains  in  the  legs  and  without  any  loss  of  knee-jerk.  But  one  of 
my  cases  showed  this  mode  of  onset  and  course 

In  other  cases,  still,  the  sudden  occurrence  of  hemiplegia,  of  epi- 
leptiform convulsions^  or  of  psychical  disturbance  of  a  temporary 
character  have  preceded  the  ordinary  development  of  locomotor  ataxia 
by  one  or  two  years.  These  symptoms,  however,  should  not  be  con- 
sidered as  belonging  to  the  disease  locomotor  ataxia  and  should  not  be 
considered  as  prodromata  of  tabes. 

The  further  course  of  the  case  after  the  development  of  any  one  of 
these  methods  of  onset  is  usually  a  slow  one  in  locomotor  ataxia.  I 
am  convinced  that  the  disease  may  come  to  a  spontaneous  standstill  at 
almost  any  stage.  Thus  I  have  under  my  observation  a  gentleman, 
aged  sixty-five  years,  who  for  thirty-five  years  has  had  slight  ataxic 
pains,  slight  ataxia  in  walking,  which  requires  him  to  use  a  cane,  but 
does  not  prevent  his  going  about  at  night  without  it,  slight  disturbances 
in  the  function  of  the  bladder,  and  all  three  physical  signs  of  the 
disease.  The  case  is  clearly  not  a  syphilitic  one  and  has  made  no 
progress  whatever  during  the  past  thirty-five  years,  during  which  time 
his  life  has  been  one  of  eminent  usefulness.  I  have  known  other  cases 
where  the  stage  of  pain  has  been  known  to  last  for  twenty  years  with- 
out the  development  of  any  ataxia,  all  the  physical  signs  of  the 
disease  being  present  and  the  patient  expecting  all  the  time  to  be 
incapacitated.  I  have  known  a  condition  of  locomotor  ataxia  to  ad- 
vance rapidly  and  by  the  end  of  four  years  to  have  reached  such  a 


DIAGNOSIS.  873 

state  as  to  render  the  patient  incapable  of  walking  without  the 
assistance  Q'f  two  men,  the  ataxia  of  the  legs  being  extreme.  The 
progress  then  came  to  a  spontaneous  standstill ;  the  patient  has  never 
had  a  single  symptom  above  the  level  of  the  umbilicus,  his  ataxia  has 
varied  very  much  in  intensity ;  at  times  he  will  be  confined  to  his  bed  ; 
at  times  he  will  be  able  to  walk  with  the  aid  of  one  cane.  He  is 
subject  about  twice  a  year  to  sudden  severe  attacks  of  pain  that  are 
most  agonizing  and  usually  last  from  two  to  three  weeks,  during  which 
time  it  is  necessary  for  him  to  take  from  ten  to  fifteen  grains  of  mor- 
phine daily,  but  these  subside  as  suddenly  as  they  have  come,  and, 
though  leaving  him  weak  for  a  time,  do  not  distress  him  in  the  interval. 
This  has  been  his  history  for  fifteen  years,  and  he  has  never  during 
this  time  required  the  use  of  a  catheter  or  had  any  gastric  or  intestinal 
disturbance.  I  have  known  the  stage  of  ataxia  to  be  arrested  after  a 
very  slight  inco5rdination  has  developed  and  the  patient  to  remain  but 
slightly  ataxic,  with  few  other  signs  of  the  disease,  for  twelve  years. 
Under  these  circumstances  it  is  necessary  to  be  very  guarded  in  giving 
a  prognosis  with  regard  to  the  course  of  a  case  of  locomotor  ataxia. 
Such  patients  are  much  more  happy  when  occupied  with  their  ordinary 
vocations  and  living  as  nearly  a  normal  life  as  possible.  Therefore,  if 
there  is  no  tendency  to  progression  in  the  case,  it  is  far  better  to  dis- 
courage a  life  of  invalidism  with  constant  treatment  and  constant 
seeking  for  a  favorable  climate  and  for  a  new  cure.  It  is  preferable  to 
give  these  patients  a  favorable  prognosis,  making  them  understand  that 
they  have  lost  certain  nerve  functions  which  cannot  be  regained, 
because  of  a  destruction  of  nervous  tissue,  but  that  there  is  every  hope 
that  further  destruction  can  be  averted  by  a  fair  amount  of  care. 

Diagnosis.  —  The  diagnosis  of  locomotor  ataxia  does  not  often  pre- 
sent any  difficulties  if  the  history  of  the  case  is  carefully  studied  with 
a  knowledge  of  all  the  various  methods  of  onset  that  may  occur.  The 
absolute  diagnosis  rests  rather  upon  the  presence  of  certain  physical 
signs  than  upon  the  existence  of  subjective  symptoms.  These  are  the 
absence  of  contraction  of  the  pupil  to  light,  the  loss  of  knee-jerk,  and 
the  swaying  when  the  eyes  are  closed  and  feet  are  approximated.  When 
any  one  of  these  symptoms  is  present,  combined  with  lightning  pains, 
with  the  undue  fatigue  after  exertion,  with  parsesthesise,  or  with  ataxia, 
there  should  be  no  doubt  about  the  diagnosis.  But  there  are  certain 
diseases  which  may  be  mistaken  for  locomotor  ataxia,  and  hence  a  dif- 
ferential diagnosis  must  be  carefully  considered.  Multiple  neuritis  fol- 
lowing alcoholism,  or  poisoning  by  arsenic,  or  developing  subsequently 
to  diphtheria  or  other  infectious  diseases,  or  developing  without  known 
cause  may  produce  pains,  ataxia,  and  a  loss  of  knee-jerk,  and  parses- 
thesise in  the  limbs.  But  in  the  majority  of  these  cases  the  onset  of 
the  symptoms  and  the  development  of  the  ataxia  are  far  more  rapid 
than  in  tabes,  all  the  symptoms  developing  within  three  to  six  weeks, 
and  the  patient  from  being  a  person  in  fairly  good  health  is  early 
reduced  to  a  stage  of  extreme  ataxia.  The  history  of  such  cases  will 
u.sually  enable  a  diagnosis  to  be  reached.  An  examination  of  such 
patients  demonstrates  that  there  is  no  Argyll -Robertson  pupil,  even 


374  LOCOMOTOR   ATAXIA. 

when  there  is  a  loss  of  knee-jerk,  and  that  the  symptoms  are  as  wide- 
spread in  the  upper  as  in  the  lower  extremities,  though  in  tabes  the 
hands  are  commonly  not  involved  until  the  patient  has  been  ataxic  in 
the  legs  for  several  years.  In  peripheral  neuritis  the  bladder  and  rec- 
tum are  rarely,  if  ever,  affected,  while  disturbance  of  their  mechanism 
is  an  early  sign  in  locomotor  ataxia.  In  peripheral  neuritis  the  distri- 
bution of  the  anaesthesia,  when  present,  is  in  the  stocking-shaped  and 
glove-shaped  areas  of  the  skin,  whereas,  as  already  stated,  in  ataxia  the 
distribution  of  the  anaesthesia  corresponds  to  the  posterior  nerve  root 
or  segment  regions  of  the  skin,  and  is  particularly  frequent  upon  the 
trunk  in  the  form  of  a  band,  a  condition  never  found  in  multiple 
neuritis.  These  points  are  usually  sufficient  to  establish  a  differential 
diagnosis  between  these  two  affections,  and  yet  I  am  constantly  seeing 
cases  in  which  there  is  some  difficulty  in  distinguishing  clearly  between 
these  diseases,  which  a  careful  study  of  the  history  always  removes. 
Inasmuch  as  tabes  is  an  incurable  affection,  while  peripheral  neuritis 
almost  invariably  recovers,  the  importance  of  the  diagnosis,  as  far  as 
prognosis  is  concerned,  cannot  be  too  closely  insisted  upon. 

The  diagnosis  of  locomotor  ataxia  from  general  paresis  would  seem 
to  be  very  easy  in  the  majority  of  cases,  inasmuch  as  one  is  a  brain 
and  one  a  spinal-cord  affection.  But  there  are  a  few  cases  of  general 
paresis  which  present  the  typical  symptoms  of  the  first  stage  of  loco- 
motor ataxia  —  lightning  pains,  great  fatigue  in  the  limbs,  a  loss  of 
knee-jerk,  and  slight  ataxia — and  which  also  present  the  Argyll- 
Robertson  pupil.  These  symptoms  develop  simultaneously  with  or 
soon  after  the  mental  irritability,  the  loss  of  memory,  the  lack  of 
power  of  concentration,  and  the  visionary  and  exalted  ideas  which  are 
characteristic  of  this  disease.  Not  infrequently  patients  show  some 
disturbance  of  speech,  tremulousness  of  the  face,  and  tingling  and 
tremor  of  the  hands.  It  is  in  these  cases  that  sudden  transient  attacks 
of  hemiplegia  are  to  be  expected.  In  such  cases  the  symptoms  of  tabes 
do  not  progress  beyond  the  slight  degree  of  ataxia  and  pain ;  do  not, 
as  a  rule,  play  a  very  important  role.  It  is  therefore  evident  that  one 
should  be  on  the  watch  for  the  development  of  mental  symptoms  in 
any  case  of  tabes,  and  when  they  occur  the  diagnosis  of  a  general 
nervous  affection  in  which  both  posterior  columns  of  the  cord  and 
cortical  lesions  of  the  brain  are  present  may  be  made.  Nonne  finds  a 
positive  Wassermann  reaction  in  the  blood  in  the  majority  of  cases  of 
tabes  but  fails  to  find  a  positive  reaction  in  the  cerebrospinal  fluid  in 
a  majority ;  while  in  general  paresis  almost  all  cases  give  a  positive 
reaction  in  the  cerebrospinal  fluid. 

The  differential  diagnosis  between  tabes  and  ataxic  paraplegia  is  made 
by  the  existence  in  that  disease  of  the  symptom  of  spastic  paralysis, 
stiffness  and  rigidity  of  the  limbs,  and  an  increased  knee-jerk.  The 
development  of  these  additional  symptoms  will  be  sufficient  to  make 
it  clear  that  a  lateral  sclerosis  as  well  as  a  posterior  sclerosis  is  present. 
Many  cases  of  ataxic  paraplegia  develop  the  spastic  symptoms  with  the 
ataxia,  but  the  patients  do  not  suffer  from  lightning  pains.  Both  dis- 
eases are  progressive  and  equally  unfavorable  in  prognosis. 


TBEATMENT.  375 

The  diagnosis  between  locomotor  ataxia  and  disease  of  the  cerebellum 
many  occasionally  cause  some  diflficulty,  inasmuch  as  ataxia  occurs  in 
cerebellar  affections.  But  cerebellar  ataxia  is  wholly  a  static  ataxia  — 
i.  e.,  an  ataxia  of  walking  and  of  balancing  in  the  upright  position  — 
and  when  the  patient  is  lying  down  with  the  trunk  and  head  supported 
there  is  no  ataxia  of  the  hands  and  feet.  The  staggering  gait  of  cere- 
bellar disease  is  much  more  irregular  than  the  gait  of  locomotor  ataxia, 
and  it  is  not  uncommon  in  cerebellar  affections  to  find  a  tendency  to 
stagger  toward  one  side.  The  stamping  gait  with  throwing  of  the  feet 
high  is  not  present  in  cerebellar  disease.  In  many  cases  of  cerebellar  dis- 
ease the  knee-jerks  are  absent,  but  the  pupil  reacts  to  light,  there  are 
no  lightning  pains  or  numbness,  and  rarely  any  bladder  disturbances. 

The  differential  diagnosis  of  tabes  from  disseminated  sclerosis  may  be 
made  from  the  absence  of  pain  in  that  disease,  from  the  usual  exaggera- 
tion of  knee-jerk,  from  the  prompt  action  of  the  pupils,  from  the  pres- 
ence of  intentional  tremor,  and  from  the  presence  of  nystagmus.  The 
very  many  other  symptoms  of  tabes  mentioned  fail  to  appear  in  the 
course  of  multiple  sclerosis. 

In  many  cases  of  neurasthenia,  especially  among  physicians,  the  sup- 
posed absence  of  knee-jerk  is  liable  to  give  rise  to  a  fear  of  the  exist- 
ence of  locomotor  ataxia,  and  then  the  easy  fatigue  upon  effort,  together 
with  the  occurrence  of  occasional  neuralgic  pains  and  possibly  slight 
irritability  of  the  bladder,  due  to  the  presence  of  uric  acid  or  due  to 
the  secretion  of  large  amounts  of  hysterical  urine,  may  lead  the  patient 
to  suppose  that  the  more  serious  disease  is  imminent.  I  have  even 
seen  an  uncertainty  of  gait,  swaying  when  the  eyes  are  closed,  and  a 
complaint  of  a  girdle  sensation  in  such  patients.  But  a  careful  exami- 
nation will  always  demonstrate  the  contraction  of  the  pupil  to  light ; 
the  knee-jerks  will  usually  be  elicited  by  the  Jendrassik  method  or  by 
tapping  the  tendon  sharply  when  the  patient  is  not  expecting  this  exami- 
nation, and  the  very  slight  intensity  of  the  symptoms  and  the  lack  of 
development  of  any  of  tlie  various  serious  symptoms  of  ataxia,  such  as 
gastric  crises  or  true  anaesthesia  to  pain,  will  enable  a  diagnosis  to  be 
reached. 

Differential  diagnosis  between  syphilis  of  the  spinal  cord  or  subacute 
syphilitic  meningitis  and  locomotor  ataxia  is  extremely  difficult  in  some 
cases,  for  a  syphilitic  meningitis  will  cause  lightning  pains,  parsesthesise, 
or  even  ataxia,  and  not  infrequently  in  this  affection  there  is  a  diminu- 
tion or  loss  of  knee-jerk ;  but  the  history  of  the  case,  as  a  rule,  will 
give  a  clue  to  the  diagnosis,  for  syphilitic  meningitis  comes  on  more 
rapidly  than  locomotor  ataxia.  Its  symptoms  are  not  as  symmetrical 
as  those  in  tabes  ;  it  is  more  likely  to  be  attended  by  pain  in  the  back 
and  by  hypers^sthesia  of  the  trunk.  The  knee-jerk  is  rarely  lost,  even 
though  diminished,  and  may  usually  be  elicited  by  reinforcement. 
There  is  no  loss  of  the  pupil  reflex  to  light.  The  pain  in  the  back  is 
worse  at  night,  and  bladder  and  rectal  symptoms  are  usually  absent. 

Treatment.  —  Thcsn;  are  two  facts  to  be  noticed  before  entering  upon 
a  consideration  of  the  treatment  of  locomotor  ataxia  : 


376  LOCOMOTOE   ATAXIA. 

1.  The  disease  from  its  very  beginning  depends  upon  certain  changes 
in  the  neurones  of  the  spinal  ganglia  which  are  permanent  in  character, 
and  hence  complete  recovery  in  any  stage  is  impossible.  All  that  thera- 
peutic measures  can  hope  to  accomplish  is  to  arrest  the  progress  of  the 
changes  at  the  point  reached,  so  that  further  symptoms  may  not  develop, 
and  this,  in  the  majority  of  cases,  is  impossible  with  our  present 
knowledge. 

2.  The  natural  history  of  the  disease  is  one  of  very  slow  progress, 
with  periods  of  decided  remission  in  some  symptoms  and  of  spontaneous 
disappearance  of  other  symptoms,  and  also  with  long  periods  during 
which  the  patient  remains  in  a  stationary  condition.  Therapeutic 
measures  miy  result  in  producing  an  arrest  of  the  disease  or  in  caus- 
ing a  remission  of  certain  symptoms,  yet  the  history  of  the  use  of 
numerous  remedies  formerly  employed  but  now  discarded,  should  teach 
us  that  it  is  often  a  mistake  to  ascribe  results  which  may  be  natural  to 
means  which  have  been  employed.  It  is  never  to  be  forgotten  that 
the  desire  for  relief  and  the  expectation  that  it  is  to  follow  the  use  of 
a  remedy  are  potent  factors  in  producing  temporary  results.  When, 
therefore,  remarkable  results  are  claimed  for  any  new  remedy  in  the 
treatment  of  locomotor  ataxia — as,  for  example,  electrical  applications, 
nerve  stretching,  and  suspension  and  the  use  of  the  animal  extracts  — 
the  wise  physician  will  show  a  becoming  skepticism,  even  when  making 
use  of  every  means  which  may  give  the  patient  relief,  and  will  wait 
for  time  to  prove  what  is  of  actual  service. 

Climate.  —  Patients  suffering  from  locomotor  ataxia  are  usually  very 
susceptible  to  changes  in  the  barometer  and  in  the  humidity  of  the 
atmosphere.  They  are  more  comfortable  in  a  warm,  dry  climate,  and 
any  sudden  change  to  cold  or  to  wet  weather  produces  an  increase  in 
their  pain  and  incoordination.  They  also  appear  to  suffer  more  in  high 
altitudes.  Therefore,  it  is  well  for  those  who  can  afford  it  to  seek  to 
mitigate  their  sufferings  by  residing  in  the  South  during  the  winter  and 
in  the  North  during  the  summer.  The  climate  of  southern  California 
is  perhaps  the  most  equable  during  the  winter  months,  and  is  certainly 
less  cold  and  damp  than  that  of  Florida  or  of  the  Riviera,  although 
these  latter  are  preferable  to  a  northern  climate.  The  increased  sus- 
ceptibility to  cold  makes  it  necessary  for  the  comfort  of  the  patient  to 
keep  the  temperature  of  his  house  during  the  winter  at  about  70°  F. 
and  to  protect  him  from  draughts.  Yet  it  is  rarely,  if  ever,  advisable 
for  him  to  abandon  such  an  amount  of  out-of-door  life  as  is  most  con- 
ducive to  health  and  vigor. 

Diet.  —  It  cannot  be  claimed  that  any  particular  form  of  diet  is 
advisable  in  this  disease.  It  is  not  to  be  forgotten,  however,  that  it  is 
of  great  importance  to  keep  the  patient  in  a  good  general  condition 
and  to  increase,  if  possible,  the  nutrition  of  the  body,  thereby  retard- 
ing the  disintegration  of  nerve  elements.  Any  system  of  diet  which, 
by  excluding  certain  classes  of  foods,  tends  to  derange  the  processes 
of  digestion  and  nutrition  should  be  avoided.  I  have  seen  patients 
much  harmed  by  following  rigid  methods  of  diet,  such  as  a  strictly 


TBEATMENT.  377 

non-nitrogenous  diet,  or  a  diet  of  meat,  bread,  and  hot  water,  or  a 
vegetarian  "^iet,  and,  therefore,  I  believe  that  a  generous  diet,  including 
all  forms  of  food,  with  a  slight  excess  of  fat  in  the  form  of  cream  or 
cod-liver  oil,  is  to  be  recommended.  A  rather  free  supply  of  water  is 
conducive  to  the  elimination  of  waste  products,  and  the  use  of  coffee, 
tea,  and  beer  or  light  wines  in  moderation  need  not  be  forbidden, 
though  any  indulgence  in  spirits  is  to  be  avoided.  Tobacco  may  also 
be  allowed. 

Exercise.  —  From  the  earliest  stage  of  the  disease  the  patients  com- 
plain that  any  effort,  especially  that  of  walking  far  or  standing  for  a 
long  time,  produces  an  unusual  sense  of  fatigue.  It  seems  reasonable, 
therefore,  to  limit  from  the  outset  the  amount  of  exercise  taken,  and, 
without  confining  the  patient  to  the  house  or  to  his  chair,  it  is  well  to 
caution  him  against  any  form  of  exertion  which  is  sufficient  to  produce 
discomfort.  Later  in  the  course  of  the  disease,  when  any  movement 
is  attended  with  difficulty,  it  may  be  necessary  to  urge  upon  the  patient 
the  dangers  of  taking  to  his  couch  or  bed,  and  even  in  the  stage  of 
helplessness  it  is  far  better  for  him  to  sit  up  during  the  greater  part  of 
the  day,  as  there  is  no  question  that  the  venous  congestion  of  the  spine 
produced  by  constant  lying  on  the  back  is  productive  of  an  increase 
of  pain  in  this  disease. 

Massage.  —  During  the  early  stage  and  during  the  last  stage  massage 
may  be  of  service.  In  the  early  stage  deep  massage  to  the  muscles  of 
the  back  promotes  the  flow  of  venous  blood  through  the  spinal  vessels 
and  their  anastomotic  branches,  and  is  the  best  means  of  relieving  the 
congestion  which  is  supposed  to  exist.  Given  at  night,  it  often  pre- 
vents the  onset  of  paroxysms  of  pain,  and  secures  sleep,  especially  if 
the  patient  can  be  taught  to  lie  on  his  belly  and  not  on  his  back.  In 
the  last  stage,  when  almost  all  voluntary  motion  is  impossible,  general 
massage,  by  promoting  venous  return,  gives  much  comfort  and  aids 
nutrition.  In  the  long  stationary  stage  of  ataxia,  when  pains  are  not 
severe  and  when  sufficient  exercise  can  be  taken  to  keep  up  a  fair  cir- 
culation, massage  is  not  needed.  In  some  cases  the  tenderness  of  the 
skin  prevents  its  use  at  all  times. 

Baths.  —  Almost  every  form  of  hydrotherapy  has  had  its  advocates, 
and  it  must  be  admitted  that  there  is  no  method  of  treatment  more 
serviceable  in  chronic  spinal  affections  than  the  use  of  baths.  To  obtain 
the  best  results  the  patient  must  be  sent  to  some  mineral  bath  in  the 
country  where  the  combined  influences  of  change  of  air,  scene,  sur- 
roundings, and  diet,  with  the  regime  of  a  water-cure  establishment 
properly  conducted,  can  be  secured.  In  the  summer  months  a  cool 
mountain  resort  is  to  be  selected.  In  the  winter  a  southern  mild 
climate  is  to  be  sought.  It  is  much  to  be  regretted  that  this  country 
offers  few  such  establishments  as  are  to  be  found  in  France  and  Ger- 
many. It  is  possible,  however,  to  find  in  many  regions  the  proper 
facilities  for  bathing,  and  well-arranged  establishments  are  rapidly 
multiplying  as  the  need  is  felt.  The  mineral  constituents  of  the  water 
are  of  much  less  importance  than  the  temperature  of  the  bath,  and 


378  LOCOMOTOR   ATAXIA. 

when  the  latter  is  correctly  regulated  it  makes  little  difference  whether 
the  spring  furnishes  sulphur  or  saline  or  iron  water. 

It  appears  to  be  quite  generally  admitted  by  those  who  have  had 
experience  with  the  use  of  baths  in  this  disease  that  tepid  and  warm 
baths  are  preferable  to  all  others.  Hot  baths  —  of  a  temperature 
exceeding  97°  F. — are  considered  injurious.  They  may  relieve  cer- 
tain symptoms  temporarily;  in  fact,  no  means  will  act  more  quickly 
than  a  hot  bath  for  the  relief  of  pain ;  but  the  ultimate  effect  of  a  suc- 
cession of  hot  baths  is  to  increase  the  rapidity  of  progress  of  the  disease 
and  to  intensify  the  symptoms.  Cold  baths  at  a  temperature  below 
65°  F.  are  also  injurious.  It  seems  probable  that  damp  cold  air  or 
exposure  to  wet  and  cold  are  factors  in  the  production  of  locomotor 
ataxia,  and  the  discomfort  resulting  from  a  cold  bath  is  very  great  to 
the  majority  of  patients.  When  baths  are  used  it  is  better  to  have  the 
temperature  of  the  water  vary  between  75°  and  95°  F.,  as  within  these 
limits  a  sufficient  alternation  of  heat  and  cold  can  be  secured. 

There  are  many  methods  of  using  water  in  baths.  Spinal  douches 
may  be  given  by  directing  a  stream  of  water  of  some  force  from  a  spout 
against  the  entire  length  of  the  spine,  the  temperature  of  the  water  being 
varied  during  the  douche  from  90°  F.  down  to  75°  F.,  or  else  being 
kept  constantly  at  85°  F.  This  may  be  used  for  ten  seconds  daily. 
Or  the  patient  may  have  the  spine  sponged  with  water  while  he  sits  on 
the  edge  of  a  tub  or  in  a  sitz  bath.  The  sudden  alternation  from  heat 
to  cold  —  90°  to  75°  F.  —  in  such  sponging  may  be  employed  at  the 
end  of  the  bath.  Both  these  methods  are  to  be  followed  by  brisk  rub- 
bing with  warm  towels.  A  full  bath  at  90°  F.  for  twenty  minutes  is 
a  method  frequently  employed,  and  when  a  course  of  iodide  of  potas- 
sium is  being  pursued  this  is  to  be  used  daily.  The  "  salt  rubs  "  and 
"  alcohol  baths  "  of  various  establishments  are  not  objectionable,  pro- 
vided the  temperature  of  the  water  be  within  the  limits  prescribed. 

It  is  evident  that  all  these  procedures  act  upon  the  spinal  circula- 
tion, either  by  affecting  the  calibre  of  the  vascular  system  in  general 
or  by  producing  such  peripheral  irritation  of  the  skin  as  to  cause  reflex 
vasomotor  effects.  In  either  case  the  circulation  in  the  cord  is  stimu- 
lated ;  congestion,  whether  arterial  or  venous,  is  relieved,  and  waste 
products  removed,  with  the  result  of  increasing  the  nutrition.  Such 
measures  may  be  carried  on  at  home,  provided  the  patient's  means  do 
not  admit  of  the  expense  of  a  residence  in  an  establishment. 

Baths  are  not  to  be  continued  for  very  long  periods  consecutively. 
It  is  better  for  this  method  of  treatment  to  be  used  thoroughly  twice 
or  three  times  a  year  for  a  period  of  two  or  three  months,  and  then 
stopped.  The  use  of  tepid  packs  to  the  extremities  or  to  the  abdomen 
for  an  hour  or  more  for  the  relief  of  pain  is  highly  recommended.  The 
extremity  is  enveloped  in  flannel  wrung  out  in  warm  water  and  covered 
with  oiled  silk.  The  damp  heat  often  relieves  the  lancinating  pains 
promptly. 

Counter-irritation.  —  Counter-irritation  to  the  spine,  whether  by  blis- 
ters, cautery,  setons,  ice-bags,  poultices,  or  cups,  has  been  generally 


TbeatmenT.  S7^ 

abandoned  as  a  means  of  cure.  In  some  cases  pain,  if  severe,  may  be 
relieved  by^the  application  of  an  ice-bag  to  the  spine  for  a  short  time 
or  by  the  use  of  dry  cupping.  These,  however,  are  of  but  temporary 
service,  and  are  probably  not  without  a  harmful  influence  upon  the 
progress  of  the  lesion. 

A  mild  form  of  counter-irritation  to  painful  parts  is  sometimes  of 
service  in  relieving  the  severe  pain.  The  part  may  be  stroked  with  a 
faradic  brush,  may  be  heated  by  a  mustard  poultice,  blistering  being 
avoided,  or  may  be  stimulated  by  liniment,  the  surface  irritation  appear- 
ing to  counteract  the  pain  of  central  origin.  In  all  local  applications 
in  locomotor  ataxia  it  should  be  remembered  that  great  care  is  to  be 
observed,  for  the  senses  of  pain  and  temperature  are  often  impaired, 
so  that  the  patient  is  unable  to  perceive  degrees  of  heat  or  pain  which 
are  harmful.  If  hot  foot  baths  are  used  the  feet  should  not  be  blistered 
unwittingly,  and  if  hot  bags  or  poultices  are  applied  they  must  be 
watched,  as  no  reliance  can  be  placed  on  the  patient's  sensations. 

Electricity.  —  Electricity  is  an  agent  which  has  for  the  past  fifteen 
years  been  used  quite  generally  in  the  treatment  of  chronic  spinal  dis- 
eases, including  locomotor  ataxia.  A  galvanic  current  has  been  recom- 
mended by  Erb  and  others  as  the  only  form  of  electricity  which  in- 
fluences the  progress  of  the  disease.  It  is  applied  to  the  back,  one 
pole  being  placed  upon  the  neck  or  between  the  shoulders,  the  other 
low  down  upon  the  lumbar  region.  The  upper  pole  is  sometimes 
placed  over  the.  sympathetic  ganglia  in  the  side  of  the  neck,  though  no 
evidence  has  ever  been  furnished  of  any  direct  action  upon  the  sympa- 
thetic nerves,  and  if  this  method  is  used  it  is  wholly  empirical.  A  cur- 
rent of  medium  intensity  is  usually  preferred,  that  is,  a  current  not 
greater  than  twenty  milliamperes  in  strength,  the  electrodes  employed 
having  an  area  of  about  one  hundred  square  centimetres  (three  by  five 
inches).  The  direction  of  the  current,  whether  ascending  or  descend- 
ing, appears  to  be  a  matter  of  indiiference.  The  duration  of  the  appli- 
cation may  be  from  three  to  ten  minutes  daily  or  every  other  day. 

Those  who  were  at  first  enthusiastic  regarding  the  effects  of  elec- 
tricity in  locomotor  ataxia  appear  to  be  gradually  modifying  their 
opinions,  and,  although  this  method  of  treatment  is  still  pursued  by 
some  specialists,  it  is  now  discarded  by  many  others.  It  is  a  matter 
of  much  doubt  whether  a  current  as  weak  as  that  mentioned  affects  the 
spinal  cord  at  all.  It  certainly  fails  to  produce  any  subjective  periph- 
eral sensations,  which  is  the  test  of  its  reaching  and  affecting  a  periph- 
eral nerve,  such  as  the  sciatic.  On  the  other  hand,  when  much 
stronger  currents  are  used  there  seems  to  be  some  danger  of  unpleasant 
results,  such  as  vertigo  and  a  sudden  increase  of  the  symptoms  of  the 
disease.  So  little  is  still  known  of  the  exact  effect  of  galvanism  on 
the  spine,  and  the  knowledge  of  its  action  elsewhere  leads  so  directly 
to  the  conclusion  that  it  cannot  have  any  effect  upon  the  progress  of  a 
sclerosis,  that  it  seems  incumbent  upon  those  who  persist  in  this  treat- 
ment cither  to  bring  forward  some  positive  results  or  to  clear  themselves 
in  some  other  manner  of  a  suspicion  of  great  credulity.     For  my  own 


380  LOCOMOTOR  ATAXIA. 

part,  I  agree  with  Gowers  and  M5bius  that  as  a  therapeutic  agent  in 
locomotor  ataxia  it  is  useless,  either  as  a  direct  agent  aifecting  the 
progress  of  the  disease  or  as  a  means  of  treating  individual  symptoms. 

Suspension.  —  A  method  of  treatment  of  some  value  is  that  of 
suspending  the  patient  by  his  head  and  arms  for  half  a  minute  or 
longer  daily.  This  is  done  by  means  of  an  apparatus  used  by  ortho- 
pedic surgeons  for  suspending  a  person  during  the  application  of  a 
plaster  jacket.  The  only  care  necessary  is  so  to  adjust  the  supports 
as  to  make  less  traction  by  the  head  than  by  the  arms  and  to  pad  the 
support  under  the  arms,  so  that  serious  compression  of  the  nerves  and 
veins  of  the  axilla  shall  be  avoided.  The  patient  is  pulled  up  slowly 
until  the  feet  are  clear  of  the  floor.  On  any  expression  of  discomfort 
or  any  appearance  of  syncope  the  process  is  to  be  stopped  at  once.  It 
is  thought  best  to  begin  by  a  short  seance — one-half  minute  —  and 
gradually  increase  the  duration  to  three  or  even  four  minutes  daily  or 
twice  daily. 

It  occasionally  relieves  some  of  the  symptoms,  especially  pain,  sexual 
impotence,  and  imperfect  control  of  the  bladder  or  rectum  ;  and  in  a 
few  cases  the  ataxia  has  been  improved. 

Medicinal  Treatment.  —  In  the  majority  of  cases  of  locomotor  ataxia 
syphilis  has  been  the  probable  cause.  It  is  not  supposed  that  the 
disease  is  an  actual  manifestation  of  syphilis,  but  that  it  is  a  sequel  of  it. 

It  is  well  to  make  a  thorough  trial  of  treatment  by  salvarsan  or  by 
mercury  and  iodide  of  potassium  (Chap.  XXXVII)  in  cases  of  locomo- 
tor ataxia,  and  if  a  decided  improvement  takes  place  it  is  well  to  continue 
the  iodide  and  to  repeat  the  course  of  mercurial  inunctions  at  intervals 
of  four  months,  each  mercurial  course  lasting  from  two  to  four  weeks, 
according  to  the  toleration  shown  by  the  patient.  In  cases  in  which 
there  is  a  subacute  spinal  meningitis  with  the  sclerosis  this  treatment 
gives  the  best  results.  Xonne,  whose  experience  in  the  treatment  of 
locomotor  ataxia  by  salvarsan  is  extensive,  reports  greater  improve- 
ment after  its  use  than  after  the  use  of  mercurial  treatment.  He  thinks 
that  it  retards  the  progress  of  the  disease.  My  experience  coincides 
with  his.  I  do  not  think  that  it  is  curative.  Sachs  confirms  this 
opinion.^  It  is  to  be  remembered  that  the  use  of  mercury  hastens  the 
progress  of  optic-nerve  atrophy,  and  therefore  in  the  class  of  cases  of 
locomotor  ataxia  in  which  optic-nerve  atrophy  is  the  initial  symptom  — 
a  class  quite  easily  separated  from  the  ordinary  type  —  this  line  of 
treatment  is  not  to  be  employed. 

In  cases  that  are  not  syphilitic  in  origin  or  in  which  specific  treat- 
ment has  failed  one  may  use  other  drugs.  The  best  line  of  treatment, 
in  my  opinion,  is  the  use  of  arsenic  in  small  doses  (arsenious  acid,  -^-^ 
grain  three  times  daily,  or  the  cacodylate  of  sodium,  Jg.  to  1  grain  three 
times  daily)  or  the  continued  use  of  corrosive  sublimate,  -^-^  grain  three 
times  daily,  or  both  used  alternately.  Arsenic  is  one  of  the  best  nerve 
tonics,  and  may  possibly  retard  the  disintegration  of  nerve  fibres  in  the 
sclerotic  areas  of  the  cord.  It  is  best  administered  by  hypodermic 
IN.  Y.  Medical  Eecord,  Feb.  2,  1912. 


TBEATMENT.  381 

injections  of  the  cacodylate  of  soda  0.05  at  a  dose  daily.  Corrosive 
sublimate  is^believed  by  many  to  retard  the  production  of  connective- 
tissue  inflammations. 

Ergot,  which  was  formerly  extensively  employed,  either  in  the  form 
of  fluid  extract,  |  drachm,  or  of  ergotin,  |^  to  1  grain  three  times  daily, 
is  not,  in  my  experience,  of  any  permanent  benefit.  If  it  is  used  it 
should  not  be  given  continuously,  but  only  four  days  in  every  week 
(Charcot). 

Nitrate  of  silver  has  also  been  used  for  many  years.  It  should  be 
administered  with  kaolin  in  a  capsule.  When  so  given  it  is  very  liable 
to  disturb  the  stomach,  and  if  kept  up  for  any  length  of  time  discolors 
the  gums,  mucous  membranes,  and  skin  a  dark  blue.  On  the  first  sign 
of  such  an  eifect  it  should  be  stopped. 

Strychnine  is  universally  discarded  in  the  treatment  of  ataxia,  except- 
ing as  a  temporary  remedy  for  the  relief  of  incontinence  of  urine,  in 
which  it  occasionally  succeeds. 

Belladonna  has  also  been  tried,  but  seems  to  have  little  claim  to  use- 
fulness. There  has  not  as  yet  been  found  any  drug  which  can  be  said 
to  be  curative  in  the  disease.  I  have  never  seen  any  benefit  from  the 
use  of  animal  extracts,  and  the  so-called  "goat's  lymph  treatment"  has 
failed  to  be  of  the  slightest  use  in  a  number  of  my  patients  who  have 
tried  it. 

There  are  certain  symptoms  of  locomotor  ataxia  which  require  treat- 
ment. Pain  is  the  chief  onC;  and  for  this  the  most  valuable  remedies 
have  been  discovered  recently  in  antipyrine,  acetanilid,  and  phenacetin. 
Given  preferably  in  a  triturated  powder  in  their  ordinary  dose  and 
repeated  two  or  three  times  a  day,  they  succeed  in  quieting  pain  very 
well.  I  prefer  to  prescribe  each  remedy  separately,  using  each  for  one 
week  at  a  time  and  changing  about,  so  that  the  dose  need  not  be  con- 
stantly increased.  Salicylate  of  sodium  in  10-grain  to  20-grain  doses 
sometimes  acts  when  the  drugs  just  mentioned  fail.  In  many  cases, 
however,  recourse  must  be  had  to  opium,  codeine,  or  morphine.  It  is 
often  possible  to  combine  these  drugs  with  others  which  neutralize  their  ' 
disagreeable  affects,  as  in  Brown-Sgquard's  neuralgic  pill.  Eventually, 
the  hypodermic  use  of  morphine  may  be  resorted  to,  and  seems  to  be 
wholly  justifiable  in  a  disease  which  is  very  painful  and  chronic.  Gowers 
uses  chloride  of  aluminium  in  5-  to  10-grain  doses  three  times  a  day. 

The  treatment  of  painful  areas  by  the  faradic  brush,  by  cold  or  hot 
applications,  or  by  cupping  the  spine,  has  already  been  alluded  to. 

Against  the  crises  of  locomotor  ataxia  we  have  but  one  sure  remedy 
—  viz.,  morphine  hypodermically  —  and  this  should  be  used  freely  to 
relieve  these  distressing  symptoms.  Antipyrine  and  phenacetin  have 
been  recently  recommended  in  the  treatment  of  gastric  crises ;  also 
oxalate  of  cerium  in  large  doses  frequently  repeated ;  also  hypodermic 
injections  of  strychnine. 

The  sense  of  stiifness  which  attends  the  stage  of  ataxia  is  frequently 
relieved  Ijy  the  use  of  warm  baths,  as  already  described. 

The  jumping  of  the  limbs  which  often  annoys  the  patient  toward 


382  LOCOMOTOB   ATAXIA. 

evening  is  best  controlled  by  bromide  of  sodium  given  with  Hoffman's 
anodyne. 

The  difficulty  of  micturition  and  in  the  control  of  the  rectum  some- 
times arises  from  the  existence  of  ulcers  or  old  strictures  in  the 
urethra.  If  such  conditions  are  found  and  are  successfully  treated 
these  symptoms  may  be  relieved. 

The  chronic  constipation  usually  requires  the  habitual  use  of  some 
laxative  or  enemata,  especially  if  opium  is  being  employed  for  the 
relief  of  pain. 

Chronic  cystitis,  joint  diseases,  and  ulcers  of  the  foot  must  be  treated 
according  to  the  usual  methods  described  in  surgical  text-books. 

For  the  optic-nerve  atrophy  no  treatment  seems  to  be  of  avail, 
although  hypodermic  injections  of  strychnine  and  the  use  of  iodide  of 
potassium  have  their  adherents. 

For  the  relief  of  the  ataxia  a  method  of  training  has  been  invented 
by  Frenkel,  of  Heiden,  which,  if  faithfully  carried  out,  is  usually 
successful.  This  method  depends  upon  the  fact  that  the  act  of  walk- 
ing, while  ordinarily  automatic,  may  be  voluntarily  directed.  In  loco- 
motor ataxia  the  automatic  mechanism  is  disturbed,  but  an  effort  of  the 
attention  combined  with  a  progressive  training  of  the  muscles  may 
enable  one  to  acquire  a  voluntary  gait  which  assists  or  takes  the  place 
of  the  automatic  gait.  The  Frenkel  movements  consist  of  attempts 
to  follow  a  straight  line,  or  a  curved  line,  or  a  triangle  upon  the  floor 
with  the  tip  of  the  toe ;  then,  when  these  motions  can  be  accurately 
performed  by  the  aid  of  sight,  further  motions,  such  as  taking  a  step 
of  a  definite  length,  going  up  stairs  and  going  through  various  bodily 
exercises,  are  learned.  These  exercises  should  be  gone  through  with 
several  times  a  day,  the  patient  resting  for  five  minutes  after  every 
five  minutes  of  exercise.  While  patients  can  acquire  these  motions 
themselves  after  instruction,  yet  it  is  found  that  they  improve  much 
more  rapidly  if  they  are  personally  instructed  by  the  physician  every 
day.  As  a  result  of  such  continued  instruction  the  gait  may  be 
materially  improved.  I  have  known  patients  who  had  been  unable  to 
walk  for  several  years  to  regain  the  power.  For  details  of  this 
method  the  reader  is  referred  to  Frenkel  and  Goldschneider's  books  on 
the  treatment  of  ataxia. 

Well-fitted  boots  with  side  supports  to  the  ankle  and  rubber  heels 
may  assist  the  patient  to  walk. 


CHAPTER  XX. 

COMBINED   SCLEROSIS. 

Acquired  and  Congenital  Ataxic  Paraplegia.     Friedreich's  Ataxia. 

History.  —  Westphal,  in  1867,  observed  a  case  in  which  both  pos- 
terior and  lateral  columns  of  the  cord  were  sclerotic.  Soon  after  Erb, 
Pierret,  and  von  Leyden  recognized  the  fact  that  this  may  be  the 
terminal  lesion  in  some  cases  of  locomotor  ataxia.  In  1877  Kahler 
and  Pick  and  in  1878  Westphal  described  combined  sclerosis  as  an 
independent  affection.  In  1886  Gowers  presented  a  clinical  picture  of 
a  disease  which  he  named  ataxic  paraplegia  and  showed  its  dependence 
upon  a  sclerosis  of  the  posterior  and  lateral  columns  of  the  cord.  Since 
that  time  a  number  of  similar  cases  have  been  published,  especially  by 
Striimpell,  Oppenheim,  Dana,  and  Putnam.  But  considerable  discus- 
sion has  arisen  in  regard  to  the  exact  nature  of  the  disease.  Some 
maintain  that  it  is  a  "  system  disease  "  of  the  spinal  cord,  by  which  it 
is  intended  to  imply  that  the  lesion  begins  in  and  is  limited  to  certain 
definite  columns.  They  have  seen  cases  in  which  the  columns  of  Goll, 
Burdach,  and  the  lateral  pyramidal  and  direct  cerebellar  columns  were 
affected  together.  Others  have  shown  that  the  lesion  is  not  strictly 
limited  to  the  various  functional  tracts,  but  is  much  more  diffuse  than 
it  is  in  such  diseases  as  tabes  or  amyotrophic  lateral  sclerosis.  In  the 
cases  examined  by  them  the  root  zone  of  the  column  of  Burdach  was 
much  less  affected  than  the  remainder  of  the  column,  the  septo-marginal 
and  cornu-commissural  tracts  were  affected,  the  lateral  limiting  layer, 
the  column  of  Gowers,  and  some  parts  of  the  antero-lateral  column 
were  involved ;  in  a  word,  there  was  a  diffuse  sclerotic  process  of  vary- 
ing intensity  at  different  levels  of  the  cord,  from  which  the  anterior 
portions  only  and  the  anterior  horns  were  exempt.  In  some  of  these 
cases  there  was  considerable  atrophy  of  the  gray  matter  about  the  cen- 
tral canal  and  of  the  columns  of  Clarke.  Hence,  Leyden  has  recently 
reaffirmed  that  combined  sclerosis  is  merely  a  form  of  chronic  diffuse 
myelitis ;  that  the  distribution  of  the  lesion  through  long  tracts  of  the 
cord  is  merely  due  to  the  structure  of  the  organ,  any  local  lesion  being 
necessarily  followed  by  secondary  degenerations  upward  or  downward 
that  are  greater  in  extent  the  nearer  the  lesion  to  the  periphery  of  the 
cord,  since  the  longest  fibres  lie  on  the  surface,  and  he  denies  the 
existence  of  a  special  disease  aside  from  chronic  myelitis.  Whatever 
the  outcome  of  this  pathological  controversy,  it  remains  a  fact  that  the 
majority  of  clinical  observers  recognize  a  combination  of  symptoms  that 
constitute  a  special  disease,  inasmuch  as  they  differ  from  other  recog- 
nized diseases.     And  the  origin  of  this  disease  appears  to  have  been 

383 


384 


CO  M  BIN  ED    HCLEROHI^. 


rightly  disclosed  by  the  investigations  of  Marie,  who  traces  it  to  a 
vascular  cause.  It  is  well  known  that  the  spinal  cord  is  supplied  by 
two  sets  of  arteries,  one  which  enters  deep  within  the  organ  through 
the  anterior  fissure  and  supplies  its  anterior  half  and  its  gray  matter, 
the  other  which  sends  numerous  branches  into  the  lateral  and  pos- 
terior columns.  (Fig,  164.)  Any  disease  in  these  arteries  causing 
an  anaemia  of  the  parts  supplied,  any  thickening  of  the  membrane 
upon  the  posterior  half  of  the  cord  compressing  these  arteries,  or  any 
condition  impairing  the  nutrition  of  the  cord  by  blood  reaching  it 
through  these  vessels  might  produce  a  degeneration.  And  the  situ- 
ation of  this  degeneration  would  necessarily  be  in  the  posterior  and 
lateral  columns  to  a  greater  or  less  depth.  This  theory  seems  to 
explain  both  the  location  of  the  lesion  and  its  diffuse  character. 

Fig.  164. 


The  distribution  of  the  >pinal  arteries.  The  shaded  areas  are  the  regions  which  are  sclerotic  in 
combined  sclerosis,  and  correspond  to  the  distribution  of  the  peripheral  system  of  arteries.     (Marie. ) 

Pathology.  —  Combined  sclerosis  consists  of  a  diffuse  sclerotic  proc- 
ess in  the  spinal  cord  chiefly  limited  to  the  posterior  and  lateral  columns. 
In  cases  of  moderate  duration  the  lesion  affects  the  column  of  Goll  in 
its  entire  length,  the  column  of  Burdach  in  part  (the  root  zone,  the 
peripheral  portion  and  the  part  adjacent  to  the  commissure  often  escap- 
ing), the  direct  cerebellar  tract,  and  the  lateral  pyramidal  tract.  In 
cases  of  long  duration  the  lateral  limiting  layer,  the  tract  of  Gowers, 
and  a  part  of  the  antero-lateral  tract  adjacent  to  the  pyramidal  tract, 
as  well  as  the  cells  of  Clarke's  column  and  of  the  central  gray  matter, 
are  also  degenerated  and  the  sclerosis  is  more  complete  in  the  columns 
first  affected.  The  extent  of  the  lesion  vertically  also  differs  in  differ- 
ent cases,  according  to  their  duration.  In  the  majority  of  cases  the 
dorsal  region  of  the  cord  is  the  part  first  involved,  and  as  the  case  goes 
on  secondary  degeneration  as  well  as  the  extension  of  the  original  lesion 
increase  the  vertical  extent  of  the  sclerotic  process.  In  some  cases  the 
lateral  columns  are  more  deeply  involved  than  in  others.     In  some 


PATHOLOGY. 


385 


cases  the  posterior  columns  are  those  first  and  most  seriously  affected. 
But  if  the  pStient  lives  long  enough  the  terminal  condition  is  one  of 
invasion  of  both  columns  in  their  entire  length.     The  varying  descrip- 


FiG.  165. 


Fig.  166. 


Fig.  167. 


Fig.  168. 


Fig.  169. 


The  situation  of  the  lesion  in  combined  sclerosis  of  vascular  origin.  Fig.  165,  cervical ;  Fig.  166 
middle  dorsal ;  Fig.  167,  lower  dorsal ;  Fig.  168,  lumbar  ;  Fig.  169,  sacral  regions.  In  the  lumbar  and 
sacral  region  the  lesion  is  one  of  descending  degeneration  only.    (Ballet  et  Minor.) 

tions  presented  by  different  authors  of  these  lesions  are  largely  due  to 
the  omission  of  any  reference  to  the  duration  of  the  disease  in  the  dif- 
ferent cases,  Putnam  being  the  only  observer  to  distinguish  between 
symptoms  of  long  and  of  short  standing  in  the  same  case. 

In  addition  to  the  sclerosis  of  the  cord  the  majority  of  observers  have 
noticed  a  thickening  of  the  pia  mater,  especially  upon  the  posterior  and 
lateral  surfaces,  with  adhesion  to  the  cord.  Some  have  seen  a  state  of 
arterio-capillary  fibrosis  or  a  true  endarteritis  in  the  spinal  vessels. 
The  anterior  nerve  roots  have  been  found  atrophied. 

The  combination  of  secondary  degeneration  of  the  motor  and  sensory 
tracts  sometimes  found  in  advanced  cases  of  general  paresis  is  not  to 
be  classed  with  this  disease. 
25 


386 


COMBINED    SCLEROSIS. 


Etiology.  —  Little  is  known  of  the  cause  of  ataxic  paraplegia,  but 
the  widespread  degeneration  in  many  columns  of  the  cord  has  given 
rise  to  the  supposition  that  it  is  due  to  an  inherent  want  of  vitality  in 
the  nervous  system  with  a  tendency  to  degeneration  from  general  mal- 
nutrition of  the  neurones.     It  is  certainly  more  common  in  persons  of 


Fig.  170. 


Section  through  the  lower  cervical  cord  showing  combined  sclerosis  of  the  lateral  and  posterior 
columns.     (Larkin.) 

neurotic  tendency.  It  develops  usually  in  males  in  early  adult  life,  is 
not  particularly  related  to  syphilis,  though  it  has  been  traced  to  it  in 
some  cases.  In  many  cases  it  follows  extreme  muscular  exertion,  and 
hence  is  more  common  in  workmen  and  those  subject  to  long  marches 
or  heavy  labor.  It  has  been  known  to  develop  after  severe  exhaust- 
ing diseases,  such  as  anaemia,  leucocythsemia,  and  cancer,  and  especially 
as  a  complication  of  pernicious  anaemia.  Marie  traces  it  to  endarteritis 
in  the  spinal  vessels. 

Symptoms. — Patients  suffering  from  combined  sclerosis  complain 
first  of  considerable  fatigue  in  the  legs  after  short  walks,  a  sense  of 
numbness  and  heaviness  in  the  legs,  and  of  stiffness  of  the  muscles  and 
of  an  unsteadiness  of  gait.  This  unsteadiness  of  gait  is  usually  worse 
in  the  dark,  and  gradually  as  months  go  by  the  patient  becomes  dis- 
tinctly ataxic.  But  this  ataxia  is  combined  with  a  rather  marked  stiff- 
ness in  the  action  of  the  legs  similar  to  that  seen  in  lateral  sclerosis ; 
the  feet  are  not  thrown  high  as  in  locomotor  ataxia,  but  are  dragged 
along  the  ground.  There  is  a  marked  swaying  when  the  eyes  are 
closed.     There  is  slight  diminution  of  tactile  sensibility  in  the  feet,  less 


SYMPTOMS.  387 

in  the  thigh^,  and  there  is  no  girdle  sensation.  There  is  an  exaggera- 
tion of  the  knee-jerks,  and  ankle  clonus  and  Babinski's  reflex  are  pres- 
ent. These  patients  do  not  complain  of  lightning  pains,  as  in  locomotor 
ataxia,  and  they  are  not  subject  to  crises.  They  often  suffer  from  a 
dull  aching  sensation  in  the  sacrum. 

As  the  case  goes  on,  after  three  or  four  years  the  spastic  symptoms 
appear  to  surpass  the  ataxic  symptoms  in  intensity,  and  spastic  para- 
plegia, together  with  its  characteristic  gait,  obscures  the  ataxia.  These 
patients  sometimes  suffer  from  disturbance  of  the  action  of  the  bladder 
and  rectum  as  the  disease  goes  on.  When  the  ataxia  reaches  the 
upper  extremities  the  reflexes  are  increased.  The  Argyll-Robertson 
pupil  is  absent,  but  nystagmus  is  not  infrequently  developed,  and  this 
may  give  rise  to  the  supposition  that  the  disease  is  really  disseminated 
sclerosis.     Optic  atrophy  has  been  observed. 

The  course  of  the  case  is  a  very  chronic  one,  but  is  progressive,  in 
this  respect  differing  from  the  spontaneous  arrest  frequently  seen  in 
locomotor  ataxia.  In  the  course  of  six  or  ten  years  these  patients 
become  fully  paralyzed,  are  confined  to  the  bed  with  limbs  drawn  up 
and  rigid,  and  with  all  the  distressing  twitchings  of  the  muscles,  spasms 
of  the  legs  and  back,  and  tendency  to  bed-sores,  'which  are  present  in 
cases  of  lateral  sclerosis  in  the  later  stages.  They  usually  die  of  some 
complication.  Some  cases  are  more  rapid  in  their  progress.  This  is 
especially  true  of  those  associated  with  and  due  to  pernicious  anaemia 
or  some  form  of  toxaemia.  In  these  cases  the  final  termination  is 
reached  within  a  year,  emaciation,  diarrhoea,  and  exhaustion  preceding 
death.  Dana  and  Putnam  have  described  these  cases  most  fully.  As 
to  the  manner  of  onset  and  course  of  these  cases  there  is  a  word  to  be 
said.  The  facts  presented  in  the  discussion  of  the  pathology  would 
indicate  that  the  symptoms  in  different  cases  may  be  quite  different. 
Oppenheim  distinguishes  two  groups  of  cases  :  one  in  which  the  symp- 
toms of  spastic  paraplegia  are  more  prominent ;  the  other  in  which 
the  symptoms  of  locomotor  ataxia  are  more  marked.  In  the  first  the 
spastic  paralysis  is  soon  followed  by  the  symptoms  of  ataxia.  In  the 
second  the  reverse  order  is  seen.  In  my  experience  the  latter  class  is 
more  frequent  than  the  former,  and  it  is  this  class  which  corresponds 
to  the  ataxic  paraplegia  of  Gowers.  It  is  a  very  rare  type  of  spinal 
affection. 

Diagnosis.  —  The  diagnosis  of  the  disease  presents  no  difficulties,  as 
is  evident  from  the  description  of  the  symptoms.  It  cannot  be  mis- 
taken eventually  for  tabes  or  for  lateral  sclerosis  because  symptoms  of 
each  disease  appear  in  addition  to  those  of  the  other. 

Prognosis.  —  The  disease  is  a  slowly  progressive  one,  and  no  arrest 
in  its  course  can  be  expected.  Yet  occasionally  periods  of  remission, 
like  those  occurring  in  locomotor  ataxia,  have  been  observed. 

Treatment. — Treatment  is  the  same  as  that  in  locomotor  ataxia. 


388 


FRIEDREICH'S    ATAXIA. 


FRIEDREICH'S  ATAXIA. 

A  defective  development  of  the  spinal  cord,  with  the  production  of 
neuroglia  tissue  taking  the  place  of  the  defective  or  degenerated  fibres 
in  a  number  of  various  tracts  of  the  spinal  cord,  was  first  described  by 
Friedreich,  of  Heidelberg,  in  1863  and  1876,  and  was  named  Fried- 
reich's ataxia.  Friedreich  supposed  it  to  be  a  juvenile  form  of  loco- 
motor ataxia,  and  called  attention  to  its  congenital  origin  and  to  the 
frequency  with  which  it  appeared  in  several  members  of  a  family.  To 
Schultze  must  be  given  the  credit  of  showing,  in  1877,  that  the  disease 
is  not  related  in  any  way  to  locomotor  ataxia,  but  is  due  to  a  defective 
development  of  the  spinal  cord.  There  is,  apparently,  a  thickened  and 
sclerotic  tissue  in  the  posterior  and  lateral  columns  of  the  spinal  cord. 

Fig.  171. 


The  lesion  of  Friedreich's  hereditary  ataxia.  Maldevelopment  and  sclerosis  of  the  lateral  and 
posterior  columns.     (Schultze,  Lehrbk.  d.  Nervenkr.,  Taf.  iv.) 

Pathology. — Fig.  171,  which  is  taken  from  one  of  Friedreich's 
original  cases,  demonstrates  the  peculiar  appearance  of  the  spinal  cord 
in  this  condition.  The  spinal  cord  in  all  of  these  cases  appears  unusu- 
ally thin  and  small,  and  there  is  usually  a  thickening  of  the  pia  mater 
about  it,  especially  upon  the  posterior  surface.  Microscopic  examina- 
tion shows  the  presence  of  extensive  degeneration  and  the  remains  of  a 
few  nerve  fibres  only  in  the  direct  cerebellar  tracts,  the  column  of  Gow- 
ers,  the  lateral  pyramidal  tracts,  and  in  the  columns  of  Goll  and  Burdach. 
The  exogenous  and  endogenous  fibres  are  both  involved  in  the  lesion. 
The  column  of  Goll  is  degenerated  in  its  entire  length  and  is  more  com- 
pletely affected  than  the  column  of  Burdach.  The  root  zone  of  the 
column  of  Burdach  and  the  column  of  Lissauer  often  contain  very  many 
normal  fibres.  The  direct  cerebellar  column  is  affected  as  well  as  the 
pyramidal  tract.  In  the  antero-lateral  column,  Blocq  and  Marinesco 
have  noticed  a  diminution  in  the  normal  number  of  fibres.  It  is  evi- 
dent, therefore,  that  a  defective  development  in  all  the  fibres  of  the 


SYMPTOMS.  389 

spinal  cord  is  present  which  is  more  marked  in  the  posterior  than  in 
the  anterioi?*  half.  The  gray  matter  of  the  cord  also  shows  changes. 
There  is  an  atrophy  of  the  cells  in  both  anterior  and  posterior  horns, 
fewer  cells  being  present  than  in  a  normal  cord.  The  cells  of  the 
column  of  Clarke  are  notably  degenerated  and  are  very  few  in  num- 
ber. Occasionally  around  the  central  canal  there  is  a  thickening  of 
the  ependyma  and  a  mass  of  small  round  cells.  Both  posterior  and 
anterior  nerve  roots  have  been  found  somewhat  atrophied.  Wherever 
the  nerve  fibres  are  wanting,  a  thickening  of  the  glia  tissue  is  evident, 
but  it  is  secondary  and  not  primary.  All  authors  agree  that  the  dis- 
ease is  due  to  an  arrest  of  development  of  the  various  systems  of  fibres 
in  the  spinal  cord.  Further  investigations  are  necessary  to  confirm  the 
statements  of  Marchi  that  secondary  changes  in  the  cerebellum  are 
always  present,  Nonne  and  Menzel  have  shown,  however,  that  an 
atrophy  due  to  a  defective  development  of  the  cerebellum  will  give 
rise  to  symptoms  almost  identical  with  those  of  Friedreich's  ataxia. 
These  symptoms  have  been  best  described  by  Marie,  and  it  is  often 
difficult  to  differentiate  Friedreich's  ataxia  from  Marie's  form  of 
cerebellar  atrophy. 

Etiology. — The  disease  appears  in  childhood,  and  is  sometimes 
present  in  several  members  of  the  same  family;  hence  it  has  been 
termed  hereditary  In  the  cases  observed  by  me,  however,  but  one 
child  out  of  several  was  affected.  It  occasionally  develops  after  some 
one  of  the  infectious  diseases  of  childhood,  which  infection  is  supposed 
to  accelerate  an  inherent  tendency  to  imperfect  growth.  In  Friedreich's 
cases  alcoholism  in  the  parents  was  supposed  to  be  the  cause.  Oppen- 
heim  believes  that  it  may  be  a  manifestation  of  hereditary  syphilis. 
It  may  be  congenital,  the  children  never  developing  the  power  to 
stand  or  walk.  It  is  usual,  however,  for  the  symptoms  to  make  their 
appearance  from  the  sixth  to  the  eighth  year  of  life,  though  cases  are 
on  record  of  as  late  a  development  as  the  sixteenth  year.  Boys  are 
more  frequently  affected  than  girls. 

Symptoms. — A  slow  onset  during  childhood  of  symptoms  which 
are  a  combination  of  spastic  paraplegia  and  ataxia  is  characteristic  of 
this  disease.  As  the  child  learns  to  walk  it  is  found  that  he  is  unsteady 
on  his  feet  and  awkward  in  his  gait,  being  liable  to  fall,  and  he  stag- 
gers in  a  manner  suggestive  of  cerebellar  disease.  He  walks  slowly 
with  little  steps,  unsteady,  with  feet  far  apart,  and  some  tendency  to 
stamp  the  foot,  but  the  legs  are  not  thrown  about  to  the  degree  that  is 
present  in  tabes.  When  the  child  attempts  to  stand  he  sways  from 
side  to  side,  the  muscles  of  the  legs  contract,  and  the  head  moves  as 
well  as  the  body  in  the  attempts  to  preserve  the  balance.  Closing  the 
eyes  increases  this  unsteadiness.  As  the  ataxia  increases  a  certain 
rigidity  of  the  legs  begins  to  appear,  and  this  is  commonly  attended 
by  a  contracture  of  the  posterior  tibial  group  of  muscles,  causing 
a  talipes  equinus.  This  deformity  of  the  foot  makes  walking  still 
more  difficult  until,  little  by  little,  the  child  becomes  incapable  of 
standing  or  walking  alone.     Fig.  172  shows  the  ordinary  appearance 


390 


FRIEDREICH'S   ATAXIA. 


Fig.  172. 


of  one  of  these  children  in  standing,  and  Fig.  173  shows  the  char- 
acteristic deformity  of  the  foot.  When  the  child  is  quiet  and  seated 
constant  oscillation  of  the  body  and  of  the  head  is  observed,  a  symptom 
which  prevented  the  photograph.  Fig.  172,  from  being  clear  in  out- 
line.    This  is  not  a  chorea-like  twitching,  because  it  is  slower  than  the 

movement  of  chorea,  and  as  effort  to  keep 
still  increases  this  oscillation  it  reminds  one 
of  the  intention  tremor  of  disseminated 
sclerosis.  The  arms  are  less  completely 
affected  than  the  legs,  but  they  share  in  the 
ataxia  and  in  the  intentional  tremor,  so  that 
the  child  is  soon  incapacitated  from  feeding 
himself  or  dressing  himself. 

In  the  later  stages  of  the  affection  a  true 
paralysis  may  develop  in  the  legs  of  the  type 
of  spastic  paraplegia,  the  muscles  being  so 
contractured  and  rigid  that  they  cannot  be 
voluntarily  moved. 

Early  in  the  disease  nystagmus  develops. 
It  is  not  present  when  the  eyes  are  quiet, 
but  becomes  visible  on  any  attempt  at  turn- 
ing the  eyes  from  side  to  side.  Paralysis  of 
the  ocular  muscles,  however,  does  not  occur, 
and  there  is  no  tendency  to  optic  atrophy. 

A  disturbance  of  speech  is  commonly  pres- 
ent in  these  children.  The  speech  is  slow 
and  difficult  and  indistinct,  not  unlike  the 
speech  of  disseminated  sclerosis.  The  tongue 
can  be  protruded,  but  usually  shows  fibrillary 
tremors. 

There  is  rarely  any  disturbance  of  sensi- 
bility in  the  affected  limbs,  and  shooting 
pains  are  not  complained  of.  Sometimes  the 
rigidity  of  the  limbs  gives  rise  to  muscular 
pain.  The  patellar  reflex  is  diminished  or 
lost  early  in  the  disease,  but  the  pupil  reflex 
remains  normal.  There  is  no  disturbance  of 
the  bladder  or  rectum.  As  a  rule,  the  chil- 
dren remain  rather  infantile  in  their  cerebral 
development.  It  is  difficult  to  give  them  an 
education,  and  even  when  reaching  adult  life 
they  appear  to  be  weak-minded.  But  this  is 
not  always  the  case,  though  the  difficulty  of 
speech  and  the  rather  stupid  appearance  of 
the  face  may  lead  to  the  supposition  that  the  child  is  less  capable  than  he 
really  is.  The  course  of  the  case  is  a  gradually  progressive  one.  It  usu- 
ally appears  at  the  age  of  six  or  seven  years,  rendering  the  child  incapa- 
ble and  paralyzed  by  the  age  of  fifteen  years.     It  never  begins  after  the 


Friedreich's  liereditary  ataxia. 
The  awkward  posture  and  forward 
bending  of  the  body  are  noticeable; 
also  the  slight  flexion  of  knees  and 
elbows. 


MA&m&i^. 


391 


age  of  sixteen  years.  It  is  not  iu  itself  a  fatal  affection,  and  therefore, 
if  the  children  are  well  cared  for  they  may  grow  up.  They  die  of  some 
intercurrent  aifection  after  many  years  of  a  crippled  existence. 

Diagnosis.  —  The  diagnosis  of  the  affection  is  not  difficult  on  account 
of  the  characteristic  appearance  of  the  symptoms  in  childhood.  As 
children  are  almost  exempt  from  locomotor  ataxia,  this  is  the  only  affec- 
tion, exceptmg  multiple  neuritis,  that  will  produce  ataxia  in  children. 

Fig.  173. 


Contrast  Ijetween  the  deformed  foot  in  Friedreich's  ataxia  and  a  normal  foot. 

There  is  no  difficulty  in  differentiating  it  from  multiple  neuritis  on 
account  of  the  history  of  the  case.  Marie's  cerebellar  hereditary  ataxia 
presents  very  similar  symptoms,  but  this  develops  between  the  twen- 
tieth and  thirtieth  years  ;  it  affects  the  arms  ;  the  patellar  tendon  reflex 
Is  preserved.  The  characteristic  contracture  and  deformity  of  the  foot 
are  not  present,  while,  on  the  other  hand,  loss  of  pupil  reflex,  paralysis 
of  the  ocular  muscles,  and  optic-nerve  atrophy  are  frequently  observed. 
The  prognosis  is  unfavorable,  and  there  is  no  form  of  treatment 
which  is  known  to  arrest  the  progress  of  the  disease. 


CHAPTER   XXI. 

MYELITIS   AND   MYELOMALACIA. 

Hj-penemia  and  A.iiaemia  of  the  Spinal  Cord.     Lesions  of  Pernicious  Anremia.     Acute 
Myelitis.     Myelomalacia.     Caisson  Disease.     Chronic  Myelitis. 

HYPEU^MIA   AND   ANEMIA    OF    THE    SPINAL    CORD. 

While  hypersemia  of  the  spinal  cord  may  occur  as  an  accompani- 
ment of  a  general  disease  of  a  febrile  character,  yet  as  a  primary  affec- 
tion it  is  questionable  whether  it  exists,  and  it  certainly  cannot  be 
recognized.  Older  writers,  it  is  true,  devoted  some  consideration  to  it, 
and  ascribed  numerous  symptoms  to  a  congestion  of  the  spinal  cord, 
but  these  symptoms  are  better  explained  at  the  present  time  as  being 
due  to  neurasthenia,  hysteria,  or  general  affections  of  a  toxic  or  infec- 
tious nature,  and  are  certainly  in  no  way  referable  to  either  arterial  or 
venous  congestion  of  the  spinal  cord. 

Anaemia  of  the  spinal  cord  may  be  said  to  be  equally  difficult  of 
diagnosis,  as  it  produces  absolutely  no  characteristic  symptoms.  If  an 
anaemia  from  obstruction  in  the  bloodvessels  is  sufficiently  intense  to 
cut  off  the  circulation  in  the  cord,  processes  of  softening  ensue  which 
can  be  recognized  and  which  are  described  under  the  head  of  myelo- 
malacia. In  conditions  of  general  anaemia,  the  result  of  long-con- 
tinued malnutrition  of  the  spinal  cord  is  to  produce  degenerations  of 
variable  degree  and  variable  extent  throughout  the  organ.  When  these 
are  sufficiently  intense  the  symptoms  caused  are  those  of  a  chronic 
myelitis.  It  is  very  remarkable,  however,  that  considerable  degrees 
of  degeneration  have  been  observed  after  death  in  cases  of  severe 
anaemia  when,  during  life,  very  few  symptoms  referable  to  the  spinal 
cord  have  been  observed.  We  may  therefore  say  that  both  in  acute 
and  chronic  anaemia  of  the  spinal  cord  it  is  impossible  to  make  a 
diagnosis  of  the  condition ;  we  can  only  suspect  their  presence. 

PERNICIOUS  AN.a:MIA  AND  ITS  EFFECTS  ON  THE  SPINAL  CORD. 

There  are  some  very  marked  changes  produced  in  the  nervous  sys- 
tem by  a  state  of  pernicious  anaemia,^  and  although  these  changes  when 
they  are  slight  may  not  produce  symptoms,  it  is  as  a  rule  possible  to 
detect  in  these  cases  evidence  of  disease  of  the  spinal  cord. 

Patholog"y.  —  A  diffuse  sclerosis  has  been  found  in  all  the  cases 
reported.     This  is  not  limited  to  any  one  system  of  fibres,  but  involves 

^  Frank  Billings,  The  Shattuck  Lecture,  1902.  Putnam  and  Taylor,  Journal  of 
Nervous  and  Mental  Disease,  1901,  vol.  xxviii,  pp.  1  and  74. 

392 


PERNICIOUS   AN.^MIA    OF   SPINAL    CORD. 


393 


any  or  all  of  the  white  columns.     As  a  rule  it  is  more  intense  in  the 
posterior  golumns,  and  more  marked  in  the  cervical  and  upper  dorsal 


Fig.  174. 


Diffuse  sclerosis  of  the  cervical  region  in  pernicious  ansemia.     (Billings.) 

segments  of  the  cord  than  elsewhere;  but  the  lateral  and  anterior 
columns  do  not  escape,  and  the  entire  length  of  the  cord  may  be  affected. 
Figs.  174  to  176  demonstrate  the  lesion  in  cases  reported  by  Billings. 

Fig.  175. 


Diffuse  sclerosis  of  the  cord  in  pernicious  anaemia.     (Billings.) 

The  sclerosis  is  not  always  diffuse,  but  may  occur  in  focal  areas  not 
symmetrically  distributed.  It  resembles  multiple  sclerosis  in  not  caus- 
ing secondary  degenerations  in  the  various  tracts.  This  is  due  to  the 
fact  that  in  some  cases  there  is  a  degeneration  in  the  myelin  only,  the 
axone  being  unaffected.  The  gray  matter  of  the  cord  is  not  usually 
involved.  Some  pigmentation  of  the  neurone  bodies  has  been  seen, 
but  chromatolysis  and  degeneration  have  not  been  found.  The  pos- 
terior ganglia  also  escape  degeneration.  Similar  areas  of  sclerosis  have 
been  found  in  the  cerebral  axis,  and  also  in  various  parts  of  the  cere- 
bral hemis])heres;  leading  to  bulbar  palsies  and  also  to  symptoms 
of  dementia. 


394  MYELITIS  AND   MYELOMALACIA. 

Symptoms.  —  The  general  symptoms  of  pernicious  anaemia  precede 
the  nervous  symptoms  for  several  months.  The  patients  complain  first 
of  numbness  in  the  legs  and  arms,  attended  by  a  girdle  sensation  and 
a  slight  loss  of  sensation.  Imperfect  control  of  the  bladder  and  rectum 
follows,  sexual  power  is  impaired,  and  the  limbs  become  weak,  and 
reflexes  are  lost.  Then  a  general  paralysis  develops,  with  some  emacia- 
tion, but  without  atrophy  or  electrical  changes  in  the  muscles.     Some- 

FiG.  176. 


Sclerosis  of  the  posterior  columns  of  the  cord  in  pernicious  anaemia.    (Billings.) 

times  an  ataxic  gait  precedes  the  paralysis  ;  sometimes  the  gait  is  spastic 
rather  than  ataxic.  The  course  of  the  disease  is  slow,  with  marked 
remissions  in  the  symptoms.  In  severe  cases  the  patients  become 
paralyzed  before  the  disease  terminates  fatally.  Mental  symptoms, 
similar  to  those  of  senile  dementia,  develop  in  the  later  stages.  The 
diagnosis  is  to  be  made  from  the  examination  of  the  blood. 

Treatment.  —  The  treatment  must  be  directed  to  the  underlying 
condition  of  pernicious  anaemia,  by  food,  tonics,  and  the  use  of  bone- 
marrow  and  of  arsenic.  This  is  not  often  successful,  and  the  percent- 
age of  recovery  from  pernicious  anaemia  is  below  twenty.  The  exact 
nature  of  the  disease  is  as  yet  undetermined. 

MYELITIS. 

Myelitis  is  a  diffuse  inflammation  of  the  spinal  cord.  It  may  be 
acute,  subacute,  or  chronic.  It  may  be  limited  to  one  or  to  two  adja- 
cent segments,  and  then  it  is  called  transverse  myelitis.  It  may  be 
more  extensive  and  effect  various  levels,  and  then  it  is  called  dissemi- 
nated myelitis.  It  may  progress  either  upward  or  downward  from  its 
starting  point,  and  then  it  is  called  ascending  or  descending  myelitis. 
It  results  in  a  destruction  of  the  spinal  elements. 

Myelomalacia  is  a  condition  of  necrosis  of  the  spinal  cord  due  to 
embolism  or  thrombosis  of  the  spinal  arteries.     It,  too,  results  in  a  de- 


MYELITIS.      ^  395 

structlon  of  the  spinal  elements.  The  symptoms  of  the  two  affections 
so  closely  resemble  each  other  that  they  may  be  studied  together. 

In  former  years  myelitis  was  supposed  to  be  a  common  disease  and 
occupied  much  space  in  text-books.  But  as  the  spinal-cord  affections,  one 
after  another,  have  been  carefully  differentiated,  and  as  the  knowledge  of 
multiple  neuritis  in  its  various  forms  has  grown,  it  has  become  evident 
that  many  cases  formerly  considered  myelitis  were  actually  of  a  different 
nature.     My  own  experience  leads  me  to  regard  myelitis  as  a  rare  affection. 

Etiology.  —  Myelitis  is,  in  the  majority  of  cases,  an  acute  infectious 
disease.  It  may  be  the  only  manifestation  of  infection,  although  its 
bacillus  still  remains  undiscovered.  It  is  often  secondary  to  some 
other  infectious  disease. 

The  site  of  this  infection  may  be  a  cutaneous  or  mucous  surface 
which  is  open  to  the  admission  of  germs.  It  has  been  known  to  follow 
small-pox,  tonsilitis,  typhoid  fever,  dysentery,  gonorrhoea,  pneumonia, 
malaria,  grippe,  cystitis  and  pyelitis,  endocarditis,  measles,  felons  and 
abscesses.  All  forms  of  meningitis,  both  the  epidemic  cerebrospinal 
meningitis,  tuberculous  meningitis,  and  purulent  meningitis,  may  be 
followed  by  myelitis,  either  localized  or  general ;  and  after  wounds  of 
the  cord  or  operations  upon  the  cord  that  are  not  aseptic,  access  of 
microbes  directly  to  the  organ  may  cause  myelitis.  It  is  thought  that 
occasionally  the  access  of  germs  to  the  cord  may  be  along  the  track  of 
nerve  trunks  when  a  purulent  neuritis  is  followed  by  an  ascending 
neuritis  and  myelitis.  The  poison  of  rabies  may  cause  an  infectious 
myelitis.  Muller  ^  has  collected  thirty-six  cases  following  inoculations 
against  rabies  by  the  Pasteur  method. 

Cases  are  upon  record  in  which  myelitis  of  an  acute  type  has 
developed  subsequently  to  an  exposure  to  cold.  Thus  Schultze  has 
described  a  case  of  a  young  man,  otherwise  in  perfect  health,  who 
developed  an  acute  myelitis  after  travelling  in  a  very  cold  railway  car 
for  several  hours,  and  another  case  developing  in  a  young  man  who 
hunted  upon  a  very  cold  day  for  several  hours.  As  already  stated 
elsewhere,  it  is  probable  that  such  exposure  to  cold  results  in  a  lowered 
resisting  power  of  the  organs  to  the  inviasion  of  bacteria  already  present 
within  the  body  and  capable  of  setting  up  an  inflammatory  process  in 
any  organ  whose  circulatory  condition  is  materially  altered.  The  same 
lowering  of  vitality  may  account  for  the  successful  attack  by  the  bac- 
teria upon  the  spinal  cord  after  blows,  injuries,  or  falls. 

The  class  of  the  community  most  often  exposed  to  the  disease  is  the 
class  of  active  workers  —  porters,  longshoremen,  those  who  are  accus- 
tomed to  lifting  heavy  weights  or  doing  heavy  work,  and  those  who 
are  upon  their  feet  a  great  deal  —  also  soldiers  who  are  subject  to  long 
marches.  Overexertion  is  an  admitted  cause.  A  great  strain  may  be 
followed  either  by  hemorrhage  or  by  acute  myelitis.  Thus  in  a  case 
of  my  own  the  effort  made  by  a  porter  in  lifting  a  heavy  box  resulted 
in  a  sudden  attack  of  transverse  myelitis  attended  by  hemorrhage,  as 
the  autopsy  demonstrated.  Falls  and  blows  upon  the  back  are  said  to 
be  responsible  for  some  cases  of  myelitis.     When  these  produce  hemor- 

» Deut.  Arch,  fur  Nerv.,  April,  1908. 


396 


MYELITIS    AND    MYELOMALACIA. 


rhage  the  symptoms  resulting  may  be  those  of  a  transverse  or  dissemi- 
nated lesion,  but  an  acute  inflammatory  process  is  rarely  set  up  by  this 
cause.  A  low  grade  of  chronic  myelitis  is  known  to  follow  railway 
injuries  and  other  severe  injuries  of  the  spine,  but  cases  of  an  acute 
inflammatory  myelitis  have  not  been  observed.  A  certain  number  of 
cases  of  acute  myelitis  develop  after  confinement.  Whether  these  are 
due  to  infection  by  way  of  the  uterus,  or  whether  they  are  due  to 
anaemia  of  the  cord  consequent  upon  hemorrhage,  or  whether  they  are 
due  to  a  slowly  acting  venous  congestion  due  to  general  enfeeblement, 
has  not  been  determined.     Syphilis  is  a  common  cause  of  acute  myelitis. 

Fig.  177. 


Acute  infectious  myelitis  with  multiple  hemorrhages,and  hemorrhagic  infiltration.    Case  was  fatal 
in  twelve  days.     Entire  cord  was  affected.     (Larkin.) 

If  the  cord  is  not  affected  by  gummy  exudations  in  the  membranes  or 
syphilitic  deposits  within  the  cord  itself,  syphilitic  changes  in  the  blood- 
vessels with  obliterating  endarteritis  and  consequent  thrombosis  of  the 
finer  vessels  of  the  cord  is  the  method  by  which  the  disease  attacks  this 
organ.  The  form  of  disease  described  by  Erb  as  syphilitic  spastic 
paralysis  has  been  found  to  be  a  chronic  myelomalacia  of  the  lower 
dorsal  region  due  to  endarteritis  with  secondary  degeneration.^  In 
some  forms  of  poisoning,  notably  by  lead,  mercury,  phosphorus,  and 

'Lancet,  October,  1902. 


MYELITIS. 


397 


occasionally  alcohol,  disseminated  foci  of  myelitis  have  been  discovered, 
as  well  as  multiple  neuritis,  and  this  has  led  to  the  supposition  that 
the  cord  may  be  directly  affected  by  toxins  and  toxic  agents.  That 
certain  agents  have  a  selective  action  upon  the  spinal  cord  is  evident 
from  our  knowledge  of  the  action  of  strychnine,  which  excites  its  activity, 
and  of  the  coal-tar  products  and  bromide  and  chloral,  which  diminish 
its  activity.  It  is  not  impossible  that  the  general  feebleness  and  tremor 
occasionally  seen  in  chronic  bromide  poisoning  is  due  to  defective  nutri- 
tion of  the  spinal  cord. 


Fig.  178. 


Acute  tranverse  myelitis  in  dorsal  region,  due  to  the  presence  of  bacillus  coli  and  streptococci. 
The  white  columns  are  infiltrated  and  in  a  state  of  acute  Inflammation.  (Marincsco,  International 
Medical  Congress,  Paris,  1900.) 

Males  are  affected  more  frequently  than  females  by  acute  myelitis 
and  by  myelomalacia.  This  is  because  they  are  more  commonly 
exposed  to  the  usual  causes  of  the  affection.  While  no  age  is  exempt, 
the  majority  of  the  cases  occur  between  the  ages  of  twenty-five  and 
forty-five  years. 

Pathology.  —  The  pathological  changes  in  acute  myelitis  have  been 
best  described  by  Mari  nesco.^  The  location  of  the  lesion  may  be  limited 
to  one  or  two  segments  of  the  cord,  under  which  circumstances  one  of 
the  symptomatic  types  of  transverse  myelitis  is  produced,  or  the  lesion 
may  be  disseminated  through  the  various  segments  of  the  cord,  normal 
tissue  intervening  between  the  inflammatory  foci,  when  the  clinical 
aspect  is  that  of  disseminated  myelitis.  The  lesion  may  be  diffuse 
with  a  tendency  to  extend  from  one  segment  to  another,  either  upward 
or  downward,  or  in  both  directions  simultaneously,  in  which  case  the 
1  International  Congress  of  Medicine,  Par  is,  1900. 


398  MYELITIS   AND    MYELOMALACIA. 

clinical  picture  is  that  of  general  myelitis  or  of  an  ascending  or  de- 
scending myelitis. 

The  gross  appearance  of  the  cord  is  not  altered  in  these  conditions, 
though  palpation  may  reveal  certain  portions  opposite  the  lesion  which 
are  softened  to  the  touch.  On  cutting  the  cord  across  at  these  softened 
regions  it  swells  up  at  the  point  of  section,  presenting  a  semifluid, 
creamy  consistency,  all  differentiation  between  gray  and  white  matter 
being  obliterated,  and  the  mass  being  either  white  and  yellow  from  the 
admixture  of  pus,  or  red  from  the  admixture  of  blood.  When  the  dis- 
integration has  not  been  so  extreme  the  cross-section  shows  an  irregu- 
larity of  the  demarcation  between  gray  and  white  matter,  points  of 
congestion,  and  irregular  plaques  of  yellowish  or  reddish  color,  as 
evidence  of  infiltration  of  the  cord  with  inflammatory  corpuscles.  In 
other  cases  plaques  here  and  there  of  a  whitish-yellow  color  indicate 
the  formation  of  connective  tissue  as  the  result  of  a  process  which  has 
fully  run  its  course.     The  microscopic  appearance  of  a  section  of  the 

Fig.  179. 


y 


Motor  neurone  bofly  of  spinal  cord  from  a  case  of  experimental  myelitis  produced  by  streptococci. 
The  cell  is  invaded  by  leucocytes,  within  which  the  streptococci  are  visible.  (Marinesco,  International 
Medical  Congress,  Paris,  1900. ) 

cord  in  the  lesion  of  myelitis  is  shown  in  Figs.  177  to  180.  Fig.  177 
shows  a  condition  of  acute  hemorrhagic  myelitis,  the  same  appearance 
shown  here  being  visible  at  all  the  segments  of  the  cord. 

Congestion  of  the  vessels,  small  hemorrhages,  an  infiltration  of  the  tis- 
sues by  leucocytes  and  small  cells  are  found,  and  the  cells  of  the  cord 
are  seen  in  all  stages  of  degeneration,  as  are  also  the  nerve  fibres  within 
the  cord.  These  changes  are  shown  in  Plates  XIX.  and  XX.  Small 
cavities  in  the  cord  form  as  a  result  of  the  disintegration  of  the  nerv- 
ous tissues.  The  neuroglia  cells  are  increased  in  number  and  small 
nodules  of  neuroglia  occasionally  form.  Granular  bodies  are  found 
everywhere  about  the  degenerated  tissues  and  in  the  neuroglia. 


PLATE  XIX 


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Acute  Myelitis,   Infiltrating  Form.     (Schmaus.) 

a,  aj.  Less  altered  tissue.  6,  fc].  Swollen  fibres,  c,  q.  Distended  spaces  in  the  glia,  with  loss  of 
fibres,  d,  d^.  Granular  cells  with  their  fat  removed  f.  Remains  of  destroyed  nerve  fibre,  g.  Vessel 
with  many  red  and  white  cells.  To  the  left  of  the  picture  the  tissue  is  infiltrated  with  cells,  especially 
in  t  lie  iyinpli  sheaths. 


MYELITIS. 


399 


Many  of  these  cells  are  supposed  to  be  phagocytes  in  process  of 
destroying«1;he  myelin  and  the  axis  cylinders.  The  tendency  of  these 
phagocytes  to  invade  and  destroy  both  neurones  and  fibres  of  the 
nervous  tissue  has  led  Marinesco  to  name  them  "  neuronophages."  He 
believes  that  many  of  them  come  from  proliferated  neuroglia  cells, 
Various  bacteria  which  have  been  found  in  the  spinal  cord,  producing 
this  infectious  myelitis,  are  the  streptococcus,  the  white  staphylococcus, 
the  yellow  staphylococcus,  the  pneumococcus,  and  a  special  diplococcus. 

Fig.  180. 


Acute  infectious  meningo-myelitis  with  exudation  in  the  pia  arachnoid  and  hemorrhages  in  the 
gray  matter  chiefly.     Death  in  10  days.     Entire  cord  involved.     (Larkin.) 

The  result  of  such  a  disintegration  and. inflammation  of  the  spinal 
cord  may  be  its  total  destruction,  changing  it  into  a  softened  mass, 
purulent  in  character.  This  is  the  condition  in  the  rapidly  fatal  cases. 
In  other  cases  the  destructive  process  is  less  complete,  the  inflammatory 
conditions  gradually  subside,  and  there  is  left  a  mass  of  scar  tissue, 
plaques  of  neuroglia  replacing  the  nerve  elements  that  have  been 
destroyed.  Any  true  regeneration  of  tlicse  nervous  elements  in  the 
spinal  cord  appears  to  be  impossible  when  the  inflammation  has  gone 
beyond  a  certain  point.  It  is  true  that  the  cells  if  not  seriously  dam- 
aged may  resume  their  function,  but  when  nerve  fibres  within  the  spinal 
cord  have  b(?en  disintegrated  no  regeneration  appears  to  be  possible, 
and  hence  permanent  damage  to  tlie  cord  is  always  the  result  of  an 
acute  myelitis.  A  complicating  meningitis  is  not  infrequently  found  in 
infectious  myelitis. 


400-  MYELITIS   AND    MYELOMALACIA. 

There  are  many  cases  of  acute  myelitis  in  which  careful  investigation 
fails  to  reveal  the  presence  of  microbes.  It  is  possible,  first,  that  the 
microbes  have  been  present  and  have  set  up  an  inflammatory  process 
but  have  subsequently  disappeared  from  the  foci  of  inflammation,  a 
condition  which  is  known  to  occur  in  other  organs ;  or,  secondly,  that 
the  inflammatory  process  is  set  up  by  toxic  agents  in  the  blood  or  toxins 
and  not  by  the  direct  presence  of  bacteria.  That  the  spinal  cord  can 
be  attacked  by  toxins  is  evident  from  the  changes  that  have  been 
demonstrated  in  it  in  cases  of  alcohol,  arsenic,  and  lead  poisoning  (see 
page  212)  following  diphtheria,  after  poisoning  by  ergot,  by  lathyrus, 
and  by  strychnine.  Similar  changes  without  bacterial  influence  result 
from  trauma  and  are  described  in  the  chapter  upon  Injuries  of  the 
Spinal  Cord. 

MYELOMALACIA. 

Myelomalacia  or  softening  of  the  spinal  cord  is  a  lesion  resulting 
from  embolism  or  thrombosis  in  the  arteries  or  veins  of  the  spinal  cord. 
Such  a  softening  may  be  red  when  accompanied  by  a  transudation  of 
blood  cells  or  by  minute  hemorrhages,  or  white  when  the  condition  has 
been  one  of  simple  death  of  the  tissue,  or  it  may  be  yellow  if  the  process 
is  not  observed  until  after  a  fatty  degeneration  of  the  d6bris  and  of  the 
neuroglia  elements  has  ensued.  Microscopic  examination  shows  a  mass 
of  debris,  cells,  and  fibres  in  a  state  of  disintegration,  balls  of  myelin, 
swollen  or  small  granular  bits  of  axones,  drops  of  fat,  and  red  blood 
globules. 

In  all  of  these  conditions  the  neuroglia  presents  a  state  of  hyper- 
plasia with  nuclear  formations  which  may  be  either  primary  or  secon- 
dary. If  the  patient  lives  and  the  products  of  disintegration  are 
absorbed  their  place  is  taken  by  new  connective  tissue,  leaving  in  the 
place  of  the  nervous  tissue  masses  or  patches  of  sclerosis.  The  initial 
lesion,  as  well  as  the  sclerotic  patch  which  is  its  result,  is  irregular  in 
outline  and  very  variable  in  extent,  the  conditions  being  such  as  to 
prevent  any  strict  limitation  of  the  softening  in  any  direction,  as  has 
been  particularly  shown  by  Obersteiner  and  Redlich.^  The  result  of 
a  diminution  in  the  blood  supply  of  the  spinal  cord,  of  a  suspension 
of  nutrition  consequent  either  upon  ischsemia,  or  upon  inflammatory 
conditions,  is  a  parenchymatous  degeneration  of  the  neurones  and  of 
their  branches  in  the  region  affected ;  hence  the  changes  that  are 
characteristic  of  myelomalacia  may  be  present  in  the  vicinity  of  foci 
of  acute  myelitis.  In  these  conditions  the  nerve  cells  are  much 
swollen  and  disintegrated,  the  nerve  fibres  are  very  much  swollen, 
producing  distention  of  their  sheaths  and  the  cribriform  appearance  in 
the  white  matter  of  the  spinal  cord  already  described.  This  condition 
should  be  considered  as  a  necrosis  rather  than  as  an  acute  inflammation, 
but  its  result  is  the  production  of  scar  tissue  of  the  nature  of  neuroglia, 
and  hence  the  terminal  state  cannot  be  differentiated  from  the  terminal 
state  produced  by  acute  myelitis.  Sometimes,  however,  when  such  a 
scar  is  distinctly  wedge-shaped  with  base  toward  the  periphery  it  will 
^Handbuch  der  prakt.  Med.,  Ebstein  and  Schwalbe,  1900. 


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MYELOMALACIA.  401 

indicate  that  the  origin  of  the  trouble  was  an  obstruction  in  a  vessel 
entering  th^  cord  from  the  surface.  Marger  ^  has  demonstrated  that 
out  of  seven  cases  of  supposed  acute  myelitis  six  were  cases  of  pure 
necrosis,  and  Douglas  Singer^  has  found  this  to  have  been  the  condition 
in  seventeen  out  of  nineteen  cases  examined  by  him.  The  chief 
pathological  distinction  between  this  form  and  the  one  already  described 
consists  in  the  absence  of  any  emigration  of  leucocytes  about  the 
bloodvessels. 

In  many  cases  changes  in  the  walls  of  the  bloodvessels,  thickening 
of  the  intima,  or  an  infiltration  of  the  adventitia,  or  a  true  endarteritis 
obliterans,  with  diminution  of  the  lumen  of  the  vessel  and  the  forma- 
tion of  thrombi  within  it,  have  been  observed.  Marger  believes  that 
the  spinal  cord  is  particularly  liable  to  the  formation  of  necrobiotic 
spots  on  account  of  the  peculiarity  of  its  circulation  and  the  defective 
anastomosis  between  the  branches  of  the  bloodvessels  entering  by  the 
anterior  fissure  and  those  entering  from  the  periphery  of  the  cord.  It 
would  seem  as  erroneous  to  call  these  changes  of  myelomalacia  myelitis 
as  it  would  to  name  the  corresponding  changes  that  occur  in  the  brain 
after  embolic  and  thrombotic  softening  cerebritis.  In  the  disease 
clinically  described  as  myelitis  we  therefore  include  cases  of  myelo- 
malacia or  pure  softening  of  the  spinal  tissue,  which,  when  they  do  not 
cause  death,  go  on  to  the  formation  of  sclerotic  patches  in  the  spinal 
cord.     (Plate  XX.) 

Inasmuch  as  the  spinal  cord  does  not  appear  to  be  particularly  sus- 
ceptible to  attacks  of  bacteria,  as  is  shown  by  the  rarity  of  myelitis 
as  a  complication  of  the  infectious  diseases,  it  seems  probable  that  the 
majority  of  the  cases  of  so-called  acute  myelitis  are  really  of  vascu- 
lar origin.  Whether  a  clinical  distinction  can  be  reached  between 
these  two  types  on  account  of  the  development  in  the  infectious  type 
of  a  marked  febrile  invasion  with  symptoms  of  an  acute  infection  and 
leucocytosis,  which  are  wholly  absent  in  the  second  type,  is  a  matter 
for  further  investigation.  And  the  much  greater  frequency  of  lesions 
in  the  brain  depending  upon  vascular  changes  and  vascular  disease 
gives  certain  grounds  for  the  hypothesis  that  lesions  of  the  spinal  cord 
hitherto  thought  to  be  inflammatory  in  nature  are  actually  secondary 
to  vascular  disease.  This  fact,  first  pointed  out  with  much  force  by 
R.  T.  Williamson,'^  has  been  substantiated  by  many  subsequent  writers, 
and  is  now  generally  accepted. 

The  so-called  spinal  spastic  paralysis  of  syphilitic  origin  described 
by  Erb  and  kuown  as  spinal  syphilitic  paraplegia  is  really  a  myelo- 
malacia of  the  mid-dorsal  region  of  the  cord  due  to  endarteritis 
obliterans  of  syphilitic  origin  and  followed  by  secondary  degenerations. 
This  is  admitted  by  Erb  in  a  recent  review  of  the  subject^  from  a 
study  of  the  pathology  of  a  number  of  cases. 

'IJeber  Myelitis  Acuta,  Obersteiner'w  Arbeiten,  1900, 
*  Douglas  Singer,  Brain,  1902. 
^Manchester  Medical  Chronicle,  1895, 
26  *  Lancet,  October  13,  1902, 


402  MYELITIS    AND    MYELOMALACIA. 

Secondary  Degenerations.  —  When,  in  transverse  myelitis  or  in 
softening  or  injury  of  the  spinal  cord,  destruction  of  the  nerve  fibres 
passing  through  the  segment  affected  occurs,  these  fibres  are  necessarily 
cut  off  from  their  connection  with  their  cell  bodies.  And  inasmuch  as 
such  a  severance  of  connection  is  always  followed  by  a  degeneration 
from  the  point  of  injury  to  the  peripheral  termination  of  the  fibre,  it 
follows  that,  as  a  result  of  a  limited  transverse  lesion  in  the  cord, 
extensive  secondary  degenerations  are  produced.  These  degenerations 
begin  within  twenty-four  hours  of  the  original  lesion,  and  go  on  pro- 
gressively for  six  or  eight  weeks  until  the  nerve  fibres  are  completely 
broken  down.  As  a  result  of  this  degeneration  both  the  axone  and 
the  myelin  about  it  are  disintegrated  and  finally  liquefied  and  absorbed. 
Hand-in-hand  with  this  progressive  degeneration  of  the  fibres  there 
occurs  a  compensatory  thickening  of  the  neuroglia,  forming  a  frame- 
work about  the  fibres,  and  hence  as  the  terminal  lesion  of  such  secon- 
dary degenerations  we  have  sclerotic  conditions  in  the  cord,  of  greater 
or  less  extent,  according  to  the  greater  or  less  extent  of  the  fibres 
originally  degenerated.     (See  Plate  XXI.) 

Below  the  lesion  such  degeneration  is  termed  descending  degenera- 
tion, because  it  affects  the  fibres  whose  neurone  bodies  lie  at  or  above 
the  transverse  lesion.  Such  descending  degeneration  is  very  extensive 
near  to  the  lesion,  inasmuch  as  there  are  descending  fibres  in  all  the 
various  columns  of  the  spinal  cord.  (Plate  XXI.,  D.  VIII.)  At 
a  distance  of  several  segments  below  the  lesion  the  descending  degenera- 
tion is  more  closely  limited  to  the  long-descending  columns  of  the  cord. 
It  is  then  manifest  chiefly  in  the  anterior  median  and  in  the  lateral 
pyramidal  tracts  which  contain  the  long  motor  fibres  from  the  brain 
to  the  anterior  horns  of  the  cord.  (Plate  XXI.,  D.  XI.)  There 
are  descending  degenerations  also  evident  for  several  segments  in  the 
anterior  and  antero-lateral  columns,  these  degenerating  fibres  being  in 
the  association  tracts  of  the  cord.  (See  page  49.)  In  the  columns 
of  Burdach  there  is  also  a  small  area  of  degeneration,  the  comma- 
shaped  column  of  Schultze,  which  degenerates  downward  for  three  or 
four  segments.  (Plate  XXI.,  D.  X.)  This  column  contains  fibres 
of  the  posterior  nerve  roots  which  turn  downward  after  the  entrance 
into  the  cord  and  terminate  in  the  gray  matter  of  the  third  or  fourth 
segment  below  their  entrance.  In  the  column  of  Goll,  adjacent  to  the 
commissure,  lie  a  few  fibres  which  degenerate  downward  for  a  short 
distance  after  a  transverse  lesion.  If  the  transverse  lesion  is  in  the 
lumbar  or  upper  sacral  region  there  is  a  descending  degeneration  in  the 
septomarginal  tract  —  oval  field  of  Flechsig.     (Plate  XXI.,  S.  III.) 

Ascending  defeneration  after  a  transverse  lesion  is  found  in  the 
anterior  and  antero-lateral  columns  of  the  cord  for  a  short  distance, 
the  association  fibres  whose  neurone  bodies  lie  in  the  parts  below  the 
lesion  degenerating  upward.  The  column  of  Burdach  is  very  con- 
siderably degenerated  above  the  level  of  the  lesion,  and  the  fibres  of 
the  posterior  nerves,  which  come  into  the  damaged  segment,  degenerate 
upward  'together  with  other  fibres  that  have  entered  the  cord  below  the 


PLATE   XXI 


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Ascending  (D.  VI  to  C.  II)  and  descending  (D.  VIII  to  S.  V.)  degeneration  in  the 

spinal  cord  after  a  transverse  lesion  at  D.  VII.     Marchi  stain. 

(Hoohe,  Arch.  f.  Psych.,  vol.  xxviii.,  plate  ix.) 


DEGENERATION    OF    THE   SPINAL    COED.  403 

level  of  the  lesion,  but  have  not  yet  terminated  in  the  posterior  horns. 
But  as  nor'Saal  posterior  nerve  roots  enter  the  column  of  Burdach  at 
levels  above  the  lesion,  the  area  of  ascending  degeneration  in  this 
column  becomes  more  and  more  limited  as  higher  levels  are  examined. 
The  column  of  GoU  degenerates  upward  in  its  entire  length  after  a 
transverse  lesion  of  the  cord,  but  the  extent  of  this  degeneration  in 
any  single  segment  will  depend  upon  the  level  of  the  transverse  lesion. 
It  will  be  remembered  that  the  column  of  Goll  contains  all  the  fibres 
from  the  sacral,  lumbar,  and  mid-dorsal  segments  of  the  cord  that  pass 
upward  to  the  medulla.  The  higher  the  level  of  the  lesion  in  the 
cord  the  greater  the  extent  of  the  degeneration  in  the  column  of  Goll. 
Figs.  27  and  28  show  the  varying  extent  of  secondary  degenerations 
after  transverse  lesions  at  different  levels.  The  direct  cerebellar 
column  degenerates  upward  after  a  transverse  lesion  of  the  cord. 
Such  ascending  degeneration  is  not  evident,  however,  if  the  lesion  lies 
in  the  lumbar  or  sacral  region,  inasmuch  as  this  column  begins  at  the 
first  lumbar  segment.  The  higher  the  level  of  the  lesion  the  greater 
the  area  of  degeneration  in  this  column.  The  column  of  Gowers 
(antero-lateral  ascending  tract)  degenerates  upward  in  its  entire  length 
after  a  transverse  lesion  of  the  cord,  and  the  area  of  this  degeneration 
is  greater  the  higher  the  level  of  the  transverse  lesion.  These  de- 
generations are  well  shown  by  the  Marchi  stain  in  Plate  XXI. 

Secondary  degeneration  occurs  after  compression  or  lesion  of  the 
cord  at  a  single  segment  from  whate^v^er  cause ;  thus,  the  compression 
of  the  cord  by  tumors,  or.  by  caries  of  the  spinal  column,  or  injuries 
of  the  cord  by  fracture  of  the  spine,  or  by  hemorrhages  outside  or 
within  the  cord,  or  by  wounds  of  the  cord  are  followed  by  these 
secondary  degenerations. 

Symptoms.  —  The  symptoms  occurring  in  payelitis  and  myeloma- 
lacia depend  chiefly  upon  the  location  of  the  lesion.  Two  kinds  of 
symptoms  develop  which  can  be  sharply  differentiated  from  one  another. 
The  first  class  of  symptoms  may  be  termed  direct  symptoms,  and  are 
due  to  destruction  of  the  elements  of  the  spinal  cord  at  the  point  of 
lesion.  These  consist  of  (a)  paralysis,  atrophy,  and  reaction  of  degen- 
eration in  the  muscles  which  are  supplied  by  the  cells  of  the  anterior 
horns  destroyed  ;  (6)  loss  of  the  reflex  action  controlled  by  the  segment 
which  is  affected ;  (c)  parsesthesia  and  anaesthesia  in  the  area  of  skin 
related  to  the  segment  which  is  destroyed.  The  region  of  anaesthesia, 
as  outlined  upon  the  body  of  the  patient  when  compared  with  Plate 
VII.,  page  46,  is  the  best  guide  to  the  exact  level  of  the  lesion. 
There  may  be  a  condition  of  hypersesthesia  just  above  the  level  of 
anesthesia  corresponding  to  the  two  segments  of  the  cord  just  above 
the  level  of  the  lesion.  There  is  usually  pain,  and  tenderness  to  pres- 
sure in  the  back.  Hot  applications  are  painful  opposite  the  segment 
of  the  cord  which  is  invaded.  This  is  due  to  the  hypersensitive  con- 
diti(m  of  the  segments  just  above  the  lesion. 

The  second  class  of  symptoms  may  be  termed  indirect  symptoms, 
and  are  produced  by  the  cutting  off  of  impulses  to  and  from  the  parts 


404  MYELITIS    AND    MYELOMALACIA. 

of  the  cord  below  the  point  of  injury.  It  will  be  remembered  that  the 
cord  transmits  many  impulses  upward  and  downward  to  and  from  the 
brain.  In  transverse  myelitis  the  tracts  conveying  these  impulses  are 
damaged  ;  hence  a  certain  number  of  symptoms  develop,  due  to  inter- 
ruption of  the  motor  tracts  to  the  parts  below  the  lesion  and  to  inter- 
ruption of  the  sensory  tracts  from  the  parts  below  the  lesion.  These 
indirect  symptoms  are  associated  with  the  secondary  degenerations 
already  mentioned  as  a  result  of  the  lesion.  Such  indirect  symptoms 
are  (a)  motor,  consisting  of  paralysis  of  voluntary  motion  in  the  legs 
with  an  increase  of  muscular  tone  in  the  paralyzed  muscles.  These 
become  rigid,  have  a  tendency  to  contraction,  and  produce  spasms  and 
contractures,  with  deformities  in  the  joints.  Such  paralyzed  muscles 
are  not  atrophied  and  have  no  reaction  of  degeneration  ;  (b)  the  reflexes 
are  markedly  increased  in  the  parts  below  the  lesion,  so  that  an  exag- 
gerated knee-jerk,  a  crossed  adductor  reflex  (tapping  on  the  knee  of 
one  side  produces  adduction  of  the  opposite  thigh),  ankle  clonus,  and 
Babinski's  reflex  are  present ;  (c)  there  is  a  loss  of  the  control  of  the 
bladder  and  rectum,  attended  by  retention  of  urine  and  involuntary 
evacuations  of  the  bowel ;  (rf)  sensations  in  the  parts  below  the  level 
of  the  lesion  are  uniformly  impaired  in  all  qualities  :  touch,  tempera- 
ture, pain,  and  the  muscular  sense  being  affected ;  (e)  trophic  symp- 
toms, especially  bedsores  over  the  sacrum,  trochanters,  and  heels  and 
ankles.  These  develop  early,  on  account  of  venous  congestion  in  the 
skin,  intensified  by  too  long-continued  pressure,  the  position  not  being 
changed  and  the  skin  being  irritated  by  fine  particles  of  dirt  or  infected 
through  small  abrasions  ;  they  often  become  extensive  and  deep,  secrete 
an  ichorous,  offensive  discharge,  expose  the  bone,  which  becomes  ne- 
crotic, and  cause  a  general  septic  condition  which  may  be  fatal ;  cystitis 
is  usually  to  be  ascribed  to  infection  from  dirty  catheters  or  by  an 
ascending  urethritis,  and  is  rarely,  if  ever,  a  trophic  lesion  of  the  blad- 
der ;  (/)  vasomotor  disturbances,  consisting  of  blueness  and  coldness 
of  the  skin,  distention  of  the  veins,  and-  increase  of  sweat.  The  sweat 
may  be  offensive. 

The  onset  of  these  symptoms  may  be  sudden,  or  they  may  develop 
gradually  in  the  course  of  a  week  or  ten  days.  In  the  acute  cases, 
which  come  on  within  a  few  hours,  the  probable  condition  is  one  of 
myelomalacia  or  a  primary  vascular  lesion.  In  the  subacute  cases, 
which  develop  more  slowly,  an  infectious  or  inflammatory  origin  is 
more  likely.  The  occurrence  of  a  rise  of  temperature  and  marked 
febrile  symptoms  point  to  the  latter  condition. 

The  course  of  the  case  is  a  slow  one,  and  the  gradual  recovery  from 
the  severe  paralysis  to  the  state  of  partial  incapacity  takes  several 
months  or  years.  The  patients  are  said  to  be  in  a  state  of  chronic 
myelitis  during  their  life  of  invalidism.  Occasionally  in  the  lighter 
cases  complete  recovery  ensues.  But  the  damage  to  the  cord,  if  serious, 
cannot  be  repaired,  and  hence  no  complete  or  permanent  recovery  is 
possible.  On  the  other  hand,  if  the  causes  of  death,  which  are  cystitis, 
inflammation  of  the  rectum,  sepsis  from  bed-sores,  and  pneumonia,  can 


SYMPTOMS    OF   MYELOMALACIA.  405 

be  avoided  by  care  and  attention  to  the  patient,  the  prospect  of  recovery 
from  the  attack  is  good.  A  permanent  condition  of  chronic  myelitis, 
the  result  of  secondary  degenerations,  remains  for  the  rest  of  the 
patient's  life,  and  this  is  manifest  by  a  greater  or  less  degree  of  spastic 
paraplegia  in  which  the  limbs  are  stiff,  the  knees  overlap  in  walking, 
there  is  trepidation  of  the  feet  when  the  weight  rests  on  the  ball  of  the 
foot ;  there  are  spasmodic  contractions  and  cramps  in  the  legs,  there  is 
more  or  less  loss  of  sensibility  in  the  limbs,  and  an  inactivity  of  the 
bladder  and  rectum,  so  that  the  catheter  has  to  be  used  regularly  and 
the  rectum  washed  out  regularly.  Hence  a  state  of  invalidism  remains, 
from  which,  however,  the  patients  do  not  die  unless  some  complication 
arises.  The  chief  danger  to  which  they  are  subject  is  an  infection  by 
the  catheter,  consequent  cystitis,  pyelonephritis,  and  uraemia. 

These  various  symptoms  of  myelitis  occur  in  varying  combinations. 
The  most  common  type  is  transverse  dorsal  myelitis,  of  which  the  fol- 
lowing case  is  a  fair  example  and  demonstrates  the  course  of  the  disease 
as  well  as  the  various  symptoms  which  appear. 

Male,  aged  fifty-six  years,  of  good  habits,  not  syphilitic  and  per- 
fectly healthy,  after  a  long  walk  perceived  sensations  of  numbness 
and  tingling  in  the  feet  and  legs  as  high  as  the  knees.  Instead  of 
resting,  he  attempted  by  further  walking  to  wear  off"  this  numbness, 
but  before  he  had  proceeded  one-half  a  mile  was  conscious  of  great 
weakness  in  the  feet  and  legs,  and  within  one-half  hour  was  con- 
siderably paralyzed,  so  as  to  be  unable  to  stand.  The  paralysis  was 
total  in  the  legs  below  the  knees.  It  was  impossible  to  pull  the  knees 
up  in  bed,  though  they  could  be  extended  and  slightly  adducted. 
Within  two  hours  all  sensation  was  lost  up  to  the  level  of  an  inch 
below  the  umbilicus,  and  at  this  point  a  distinct  girdle  sensation  was 
perceived.  Examination  showed  loss  of  sense  of  touch,  temperature, 
and  pain  up  to  this  level,  and  for  two  inches  above  this  level  a  con- 
dition of  extreme  hypersesthesia  to  touch,  pain,  and  temperature.  The 
muscular  sense  was  not  lost  in  the  legs,  and  all  changes  of  position 
in  the  feet  and  toes  was  acutely  perceived.  Retention  of  urine  was 
present,  and  there  were  no  sensations  in  the  rectum  and  no  control 
over  its  action.  There  were  no  symptoms  above  the  level  of  the  um- 
bilicus. There  was  no  fever.  This  condition  remained  stationary  for 
about  four  months,  during  which  time,  by  care  in  the  emptying  of  the 
bladder  and  of  the  rectum,  and  by  frequent  change  in  the  position  of 
the  patient  in  bed,  complicating  cystitis,  proctitis,  and  bed-sores,  which 
were  constantly  threatened,  were  avoided.  The  patient  then  began  to 
recover  slightly  the  power  in  the  legs,  but  was  much  troubled  by 
twitching  of  the  muscles  and  sudden  spasmodic  contractions  of  the 
legs,  sometimes  in  the  form  of  flexion  of  the  knees,  sometimes  in  the 
form  of  extension  of  the  foot.  The  paralyzed  muscles  did  not  atrophy, 
and  the  electrical  reaction  remained  normal.  Knee-jerks,  which  were 
completely  lost  during  the  first  week,  then  returned  and  gradually 
became  exaggerated,  and  after  two  weeks  ankle  clonus,  Babinski's 
reflex,  and  crossed  reflex  of  tlie  knees  appeared  and  have  continued. 


406  MYELITIS    AND    MYELOMALACIA. 

A  gradual  progressive  recovery  of  power  and  of  sensation  ensued 
during  the  following  two  years,  but  was  attended  by  an  increasing 
rigidity  of  the  legs.  His  sexual  power  returned.  A  stationary  con- 
dition has  remained  for  four  years  in  which  the  patient  has  a  spastic 
rigidity  of  both  legs,  and,  though  able  to  walk,  does  so  with  stiffness 
and  great  effort  with  the  aid  of  two  canes,  there  being  tendency  of  the 
knees  to  overlap,  a  dragging  of  the  feet,  and  a  frequent  trepidation  of 
the  foot  when  the  weight  happens  to  rest  upon  the  ball  of  the  toe.  It 
is  impossible  for  him  to  rise  from  the  chair  or  to  sit  down  without 
assistance.  There  has  never  been  a  return  of  the  control  of  the 
bladder  and  rectum,  there  being  retention  of  urine,  which  is  relieved 
by  the  catheter.  Sensations  have  returned  in  the  previously  anaesthetic 
area,  but  are  by  no  means  as  keen  as  in  the  hands  and  arms.  The 
girdle  sensation  remained  for  two  years  and  then  subsided.  There  is 
no  longer  a  zone  of  hyperaesthesia. 

It  is  evident  from  this  case  that  there  was  an  acute  onset,  then  a 
stationary  period  lasting  several  months,  and  then  a  gradual  improve- 
ment, which  progressed  for  six  years,  leaving  the  man  at  the  end  of 
that  time  in  a  stationary  condition  from  which  no  recovery  is  likely. 
The  level  of  the  lesion  was  the  tenth  dorsal  segment,  as  was  shown  by 
the  level  of  the  anaesthesia.  Inasmuch  as  the  functions  of  the  tenth 
dorsal  segment  of  the  cord  are  very  few,  the  direct  symptoms  in  this 
case  were  quite  subordinate  to  the  indirect  symptoms  due  to  a  cutting 
off  of  the  tracts  passing  through  this  segment. 

I  have  seen  a  case  in  which  a  rapidly  advancing  transverse  myelitis 
at  the  sixth  dorsal  segment  left  the  patient  totally  paralyzed  and  abso- 
lutely anaesthetic  below  this  level.  This  condition  had  been  present 
four  years  when  I  saw  him,  and  during  all  this  time  bed-sores  had  been 
prevented  by  having  him  turned  in  bed  every  half-hour  day  and  night. 
The  pressure  of  his  body  weight  caused  a  redness,  followed  by  cyanosis 
in  the  skin,  which  would  surely  have  gone  on  to  a  bed-sore  had  this 
change  of  posture  not  been  made.  In  this  patient,  although  all  sensa- 
tion was  abolished  below  the  level  of  the  eighth  intercostal  nerve,  any 
irritation  of  the  skin  in  the  anaesthetic  region  produced  a  sensation 
which  was  referred  to  the  skin  in  the  hyperaesthetic  band  at  the  level 
of  the  sixth  intercostal  nerve.  Such  erroneous  location  of  sensations 
I  have  observed  in  several  cases  of  myelitis,  but  have  not  seen  it 
described. 

Transverse  lumbar  myelitis  is  the  next  most  common  form  of  mye- 
litis, and  here  the  lesion  may  be  limited  to  the  lumbar  segments  or  may 
involve  both  lumbar  and  sacral  segments  of  the  spinal  cord.  As  an 
example,  the  following  case  may  be  cited  : 

Male,  aged  sixty  years,  of  fairly  good  habits  and  without  any  ascer- 
tainable cause,  was  suddenly  attacked  by  a  tingling  and  numbness  in 
the  legs  as  high  as  the  kness  and  in  the  gluteal  region.  This  numb- 
ness was  followed  in  the  course  of  twenty-four  hours  by  a  condition  of 
marked  weakness  in  the  feet  and  legs,  with  drop-foot  and  with  reten- 
tion of  urine  and  inability  to  control  the  rectum.     He  had  considerable 


SYMPTOMS    OF   MYELOMALACIA.  407 

dull  pain  in  the  back  over  the  sacrum  and  down  the  back  of  the  thighs 
and  legs.  Within  four  hours  of  the  onset  he  was  completely  unable  to 
move  his  legs  in  bed  in  any  direction,  tliough  when  the  knees  were 
pulled  up  slight  adductor  movements  of  the  thighs  could  be  produced 
voluntarily.  An  extensive  area  of  anaesthesia  was  found  in  the  legs 
and  in  the  back  and  outer  part  of  the  thighs,  but  a  funnel-shaped  zone 
on  the  front  of  the  thigh  was  found  hypersensitive  rather  than  anaes- 
thetic, and  there  was  no  disturbance  of  sensation  above  the  level  of 
Poupart's  ligament  in  front.  The  knee-jerks  were  lost,  the  muscles 
that  were  paralyzed  in  the  legs  were  flaccid,  soft,  and  soon  began  to 
atrophy,  later  on  showing  partial  reaction  of  degeneration.  He  had  a 
slight  fever,  the  temperature  varying  from  99.5°  to  101°  F.  for  six 
days,  when  it  became  normal.  At  the  end  of  two  weeks  all  the  symp- 
toms began  slowly  to  improve.  In  the  course  of  three  mouths  he  had 
begun  to  move  his  limbs,  though  by  this  time  they  were  markedly 
atrophied.  He  had  developed  a  cystitis  due  to  imperfect  catheteriza- 
tion, and  was  still  unable  to  control  the  action  of  his  rectum.  The 
anaesthesia,  though  present,  was  markedly  diminished  in  intensity,  and 
there  was  no  point  upon  the  limbs  where  sensations  of  touch  and  pain 
could  not  be  located.  A  subjective  difference,  however,  between  the 
sensations  of  touch,  temperature,  and  pain,  as  felt  in  the  legs  and  hands, 
was  present.  During  the  following  three  months  the  improvement 
gradually  went  on,  and  seven  months  after  the  onset  he  was  able  to 
walk  with  a  cane,  but  was  still  unable  to  go  up  and  down  stairs.  He 
could  rise  from  a  chair  by  the  aid  of  his  hands,  but  in  walking  there 
was  a  marked  weakness  of  all  the  muscles  and  a  tendency  to  drop- 
foot.  Numbness  was  present  over  the  legs  and  back  of  the  thighs,  and 
it  was  still  necessary  to  use  a  catheter.  This  condition  has  persisted 
for  the  past  five  years,  the  legs  being  weak,  thin,  and  somewhat  insen- 
sitive. The  knee-jerks  have  never  returned,  and  the  action  of  the 
bladder  has  still  to  be  assisted..  He  is  impotent.  The  entire  lumbo- 
sacral enlargement  up  to  the  second  lumbar  segment  was  evidently 
diseased,  but  the  lesion  was  nowhere  so  completely  destructive  as  to 
suspend  motor  and  sensory  functions. 

I 'have  seen  a  similar  case,  developing  after  a  confinement  that  had 
been  attended  by  profuse  hemorrhage  but  no  apparent  sepsis.  This 
patient  went  on  slowly  to  complete  recovery  in  six  months. 

When  the  cervical  region  of  the  cord  is  invaded  by  transverse 
myelitis  the  direct  symptoms  appear  in  the  arms  and  hands,  the  hands 
and  arms  below  the  elbow  being  aflFected  if  the  lesion  is  in  the  lower 
part  of  the  cervical  region,  and  the  arms  and  shoulders  if  the  lesion  is 
as  high  as  the  fifth  cervical  segment.  A  rapid  onset  of  total  paralysis 
with  anaesthesia  and  reaction  of  degeneration  in  the  muscles  aflFected 
and  loss  of  their  reflex  activity  occurs,  the  ansesthesia  on  the  body, 
however,  being  often  lower  down  than  might  be  expected  in  a  cervical 
lesion  of  the  cord.  Thus  in  a  case  of  sudden  onset  of  paralysis  aflFect- 
ing  both  hands  and  muscles  of  the  forearms,  excepting  the  supinator 
longus,  and  attended  by  anaesthesia  in  the  inner  surface  of  the  arm  and 


408  MtELiTIS    AND    3IYEL0MALAC1A. 

in  the  greater  part  of  the  forearm,  excepting  the  radial  side,  the 
anaesthesia  upon  the  trunk,  together  with  the  zone  of  hypersesthesia 
above  it,  was  below  the  level  of  the  nipple.  Paralysis  of  the  sympa- 
thetic in  the  neck,  causing  a  narrowing  of  the  palpebral  fissure,  retrac- 
tion of  the  eyeball  and  a  loss  of  dilatation  of  the  pupil  on  irritation  of 
the  neck  were  present. 

The  indirect  symptoms  of  spastic  paralysis  in  the  legs  develop  early. 
The  knee-jerks  are  exaggerated,  ankle  clonus  appears,  the  legs  are  stiff 
and  rigid,  and  subject  to  quick  sharp  spasms  of  a  painful  character,  and 
cramps.  There  is  usually  retention  of  urine,  though  occasionally  the 
bladder  may  empty  itself  suddenly  without  the  control  of  the  patient. 
There  is  no  control  of  the  rectum,  which  may  act  in  the  same  manner 
as  the  bladder  when  full.  In  these  cases  it  is  not  uncommon  for 
priapism  to  occur.  These  cases  are  more  dangerous  than  those  of 
transverse  myelitis  of  the  dorsal  and  lumbar  region,  because  of  the 
possibility  of  an  ascending  myelitis  which  may  invade  the  cells  govern- 
ing the  phrenic  nerve  and  cause  death  by  respiratory  paralysis. 

In  disseminated  myelitis  the  symptoms  of  paralysis  with  atrophy  and 
disturbances  of  sensation  are  more  extensive  than  in  cases  of  transverse 
lesion,  and  symptoms  appear  both  in  the  legs,  body,  and  arms  simul- 
taneously or  in  rapid  succession.  A  few  muscles  in  each  limb  may  be 
picked  out  by  the  paralysis,  adjacent  muscles  being  weak,  but  showing 
no  tendency  to  atrophy  or  reaction  of  degeneration.  A  disturbance  of 
sensibility  is  usually  marked,  but  does  not  go  on  to  a  total  anaesthesia. 
There  is  commonly  a  loss  of  bladder  and  rectal  control. 

E.  W.  Taylor  has  recorded  the  interesting  fact  that  the  course  of 
labor  with  successful  delivery  and  firm  uterine  contractions,  was  in  no 
way  interfered  with  in  a  case  of  lumbar  myelitis  with  destruction  of 
the  entire  lower  cord  as  shown  by  autopsy.  There  was  paralysis  of 
the  abdominal  muscles  and  no  power  of  aiding  the  expulsion  of  the 
child  by  them,  but  the  uterus  contracted  rhythmically  during  labor. 

Prognosis.  —  In  the  early  stage  of  myelitis  a  very  guarded  prognosis 
must  be  given.  It  is  impossible  to  tell  to  what  extent  or  severity 
the  symptoms  may  develop,  and  until  the  progress  is  arrested  the 
outlook  is  always  serious.  Infectious  cases,  especially  those  that*  are 
secondary,  have  a  better  chance  of  recovery  than  cases  due  to  disease 
of  the  bloodvessels,  and  the  slower  the  onset  of  the  symptoms  the 
better  the  chance  of  their  arrest.  The  more  absolute  the  ane&sthesia 
in  any  case  the  worse  is  the  prognosis.  The  greater  the  pain  in  the 
back,  especially  if  it  is  attended  by  rigidity  of  the  spine,  or  spasms  in 
the  legs,  the  worse  the  prognosis.  After  the  acute  onset  the  prognosis 
will  depend  on  the  severity  of  the  symptoms.  Bed-sores  and  cystitis 
make  the  outlook  less  hopeful.  After  very  complete  paralysis  I  have 
seen  a  slow  but  progressive  improvement  and  a  return  of  power  to 
walk.  It  is  always  well  to  hold  out  the  hope  of  some  improvement 
for  two  years.  After  that  time  no  change  can  be  expected.  The 
j^ounger  the  patient  the  more  vigorous  his  general  condition,  and  the 
better  his  habits  the  better  his  chances. 


Symptoms  of  myelomalacia.  400 

Treatment.  —  Absolute  rest  in  bed  must  be  enforced  from  the  out- 
set. The4)atient  should  lie  in  a  prone  position,  if  possible,  and  great 
care  should  be  taken  to  change  the  position  at  least  every  half-hour 
and  to  so  distribute  the  weight,  by  means  of  pillows  or  by  an  air-bed 
or  water-bed,  as  to  prevent  pressure  of  the  body  coming  upon  the 
bony  points.  Frequent  sponging  of  the  skin  and  careful  attention  to 
cleanliness,  especially  if  there  is  an  incontinence  of  urine  or  feces,  are 
necessary  in  order  to  prevent  the  development  of  bed-sores.  Under 
all  circumstances  the  use  of  the  bed-pan  or  urinal  should  be  enforced, 
as  the  effort  of  sitting  up  may  increase  the  symptoms.  If  the  skin  is 
very  red  it  should  be  bathed  with  an  antiseptic  solution  and  with  a 
solution  of  alum  and  covered  with  a  powder,  and  no  pressure  on  it 
permitted.  If  a  bed-sore  forms  it  is  to  be  dressed  like  any  ulcer  and 
kept  as  aseptic  as  possible.  Stimulants  to  healing,  such  as  balsam  of 
Peru  or  a  galvanic  current  directly  applied,  have  never  seemed  to  me 
to  be  effective.  The  most  important  thing  is  to  remove  all  pressure 
from  the  ulcerated  surface,  and  thus  allow  it  to  heal.  The  greatest 
care  should  be  taken  in  catheterization,  all  catheters  should  be  kept  in 
antiseptic  solutions  and  carefully  washed,  and  coated  with  carbolized 
vaseline  before  being  used.  It  is  too  commonly  the  custom  to  limit 
the  washing  of  the  catheter  to  the  time  succeeding  instead  of  preceding 
its  use.  If  cystitis  develops  the  bladder  should  be  washed  out  twice 
or  three  times  a  day  with  a  10  per  cent,  solution  of  boric  acid  at 
98°  F.,  and  the  patient  should  be  given  salol,  five  grains,  or  urotropin, 
ten  grains,  every  four  hours  by  the  mouth.  The  action  of  the  bowels 
should  be  regulated  by  means  of  laxatives  daily,  which  should  be  as- 
sisted by  an  injection  given  at  a  regular  time  of  the  day,  a  pad  being 
constantly  applied  to  the  anus  to  prevent  leakage.  The  spasmodic 
contractions  and  cramps  in  the  muscles  may  be  relieved  by  the  use  of 
warm  cloths  to  the  back  or  of  warm  bottles,  care  being  taken  not  to 
burn  a  blister  in  the  anaesthetic  skin.  Bromides  are  sometimes  of 
much  service  for  this  symptom.  Any  active  medicinal  treatment  for 
the  disease  during  the  period  of  onset  or  during  the  period  of  maximum 
intensity  is  usually  futile,  though  salicylate  of  soda  and  salicin  have 
been  supposed  to  be  of  use  in  infectious  cases.  When  the  active  proc- 
ess has  come  to  a  standstill  and  a  period  of  improvement  sets  in, 
massage  and  electrical  applications  to  the  muscles,  gradually  increasing 
voluntary  movements  and  exercises,  tending  to  reestablish  the  volun- 
tary control  of  the  limbs,  are  to  be  used.  Baths  are  also  of  service, 
either  warm  and  cool  sponging  of  the  back,  or  warm  douches  under 
moderate  pressure  (ten  pounds),  or  tepid  sitz  baths  with  sponging  of 
the  back.  Cold  (60°  F.)  or  hot  (100°  F.)  baths  are  to  be  avoided. 
When  the  patient  begins  to  walk  great  care  should  be  taken  to  pre- 
vent over-fatigue  and  to  prevent  falls.  Rubber  heels  or  soles  help 
these  paralytics.  In  some  cases  apparatus  may  be  used  to  facilitate 
the  act  of  walking,  such  as  braces  to  the  ankles  or  knees  or  a  stiff 
corset  to  the  back. 


410  MYELITIS    AND    MYELOMALACIA. 

CHRONIC  MYELITIS. 

Etiology.  —  Chronic  myelitis  may  develop  as  the  result  of  an  acute 
myelitis  when  this  does  not  go  on  to  a  fatal  termination  but  leaves  a 
certain  amount  of  damage  to  the  spinal  cord.  A  myelitis,  however, 
may  be  from  its  onset  a  slow  process,  and  thus  the  disease  may  be 
chronic  from  the  start.  Any  of  the  causes  capable  of  causing  acute 
myelitis  are  equally  capable  of  producing  chronic  myelitis.  Distur- 
bances in  the  circulation  of  the  cord  are  probably  the  primary  cause  in 
the  majority  of  cases,  atheroma  of  the  bloodvessels  or  syphilitic  en- 
darteritis being  among  the  most  common  conditions  found  in  this 
disease.  Chronic  myelitis  may  also  develop  as  the  result  of  chronic 
meningitis,  and  is  usually  associated  with  more  or  less  implication  of 
the  spinal  meninges.  The  term  chronic  myelitis  may  be  applied  to  any 
or  all  of  the  processes  of  degeneration  developing  in  the  cord  in  con- 
nection with  other  spinal-cord  affections.  Thus  the  terminal  stage 
of  lateral  and  combined  sclerosis,  of  amyotrophic  lateral  sclerosis,  of 
acute  or  chronic  poliomyelitis,  or  of  disseminated  sclerosis  may  be 
diagnosticated  as  chronic  myelitis  in  case  no  exact  history  of  the  onset 
of  the  disease  can  be  obtained.  Chronic  myelitis  may  also  occur 
without  any  ascertainable  cause,  a  chronic  irregular  degeneration,  both 
in  the  white  columns  and  in  the  gray  matter  being  found  after  death 
in  cases  where  the  disease  has  been  one  of  slow  and  irregular  type. 
Chronic  myelitis  may  develop  in  the  course  of  very  many  constitu- 
tional diseases  that  produce  disturbance  of  general  nutrition,  and  it  may 
also  develop  secondarily  to  many  of  the  acute  infectious  diseases,  being 
in  these  cases  due  rather  to  the  toxic  agents  produced  by  the  infection 
than  to  any  direct  bacterial  action.  Any  of  the  various  forms  of  injury 
of  the  spinal  cord  or  of  its  bony  covering,  or  of  its  meninges,  may  be 
followed  by  a  chronic  degeneration  in  the  spinal  cord.  Very  great 
and  long-continued  physical  exercise  is  also  supposed  to  be  a  cause. 
Chronic  alcoholism  may  produce  a  chronic  myelitis  as  well  as  a  periph- 
eral neuritis,  and  in  some  cases  of  very  severe  peripheral  neuritis 
which  failed  to  recover,  changes  have  been  found  of  a  degenerative 
nature  in  the  spinal  cord.  Thus  chronic  myelitis  may  be  the  result  of 
a  state  of  poisoning  which  produced  first  a  multiple  neuritis.  Chronic 
myelitis  develops  occasionally  as  a  sequel  of  gout  with  or  without  an 
attending  multiple  neuritis,  the  exact  nature  of  the  process  being  still  a 
matter  of  uncertainty.  Chronic  myelitis  is  in  some  cases  due  to 
syphilis,  and  then  it  may  be  either  a  diffuse  process  advancing  into  the 
cord  from  the  periphery,  attended  by  degeneration  which  appears  first 
in  the  lateral  columns  of  the  cord  and  gives  rise  to  the  symptoms  of 
spastic  paraplegia  (the  spinal  syphilis  of  Erb),  or  it  may  be  a  trans- 
verse process  due  to  syphilitic  endarteritis  in  the  vessels  at  a  certain 
level,  or  to  the  appearance  of  syphilitic  indurations  with  infiltration  of 
round  cells  in  both  white  and  gray  matter.  Such  syphilitic  infiltra- 
tions may  be  very  irregular  in  their  distribution ;  may  be  confined  to 
one  segment  of  the  cord,  causing  a  chronic  transverse  myelitis ;  may 


CEBONIC  MYELITIS. 


411 


be  limited  to  one  or  more  columns  of  the  cord,  causing  symptoms 
resembling  focomotor  ataxia  or  lateral  sclerosis  of  one  or  both  sides ; 
may  cause  a  unilateral  lesion,  producing  symptoms  of  Brown-S6quard 
paralysis,   or  may  be  irregularly  disseminated   throughout  the  cord. 


Fig.  181. 


Chronic  myelitis.  Swelling,  degeneration,  and  sclerosis  in  the  cord,  a,  degenerated  tissue  ;  6,  cell 
in  state  of  chromatolysis ;  c,  c',  swollen  axis  cylinders;  d,  sclerotic  meshes  of  glia;  g,  vessels  with 
thickened  walls ;  n,  enlarged  meshes  of  glia.     (Schmaus-Sacki. ) 

Chronic  myelitis  is  sometimes  due  to  pernicious  anaemia,  the  changes 
in  the  cord  which  occur  in  the  course  of  this  disease  being  diffuse  in 
location  and  slow  in  development^  (see  page  393).  Slow  poisoning  by 
ergot  or  lathyrus  may  cause  chronic  myelitis. 

Chronic  myelitis  is  usually  a  disease  of  adult  life,  persons  of  the  ages 

from  twenty-five  to  forty-five  years  being  more  liable  to  the  affection ; 

it  is  about  equally  prevalent  in  males  and  females.     It  is  possibly 

traceable  in  some  cases  to  an  inherited  neuropathic  tendency.     It  is,  in 

'  F.  Billings,  The  Shattuck  Lecture,  1902. 


412  MYELITIS    AND    MYELOMALACIA. 

my  experience,  a  very  rare  form  of  affection,  and  it  is  probable  that 
the  descriptions  found  in  the  older  text-books,  which  rarely  correspond 
to  cases  now  seen  in  the  hospitals,  were  written  before  the  time  when 
careful  distinction  was  made  between  the  various  forms  of  spinal-cord 
disease.  As  the  knowledge  of  spinal -cord  disease  has  increased  it  has 
been  possible  to  separate  accurately  the  different  types,  and  the  more 
this  is  done  the  rarer  the  diagnosis  of  simple  chronic  myelitis  is  made. 
In  fact,  some  authors  have  denied  that  as  a  special  disease  it  can  occur, 
and  affirm  that  it  is  always  to  be  regarded  as  the  terminal  stage  of 
some  one  of  the  diseases  of  the  cord  already  considered. 

Pathology.  —  The  pathology  of  chronic  myelitis  cannot  be  traced 
accurately  from  the  onset,  inasmuch  as  the  terminal  stage  of  the  disease 
is  the  only  one  which  is  open  to  direct  observation.  In  patients  dying 
from  chronic  myelitis  the  spinal  cord  may  present  slight  deformities  in 
its  contour,  but  the  changes  are  only  visible  in  microscopic  section. 
These  changes  are  of  two  varieties :  first,  the  appearance  of  sclerotic 
patches  irregularly  distributed  throughout  the  white  and  gray  matter 
of  the  spinal  cord.  These  sclerotic  patches  consist  of  a  thickening  of 
the  glia  and  of  the  connective  tissue  about  the  bloodvessels,  usually 
attended  by  a  distention  of  the  bloodvessels  and  by  changes  in  their 
walls  of  the  nature  of  endarteritis  —  both  obliterating  or  atheromatous. 
As  a  rule,  this  sclerotic  tissue  is  thicker  and  more  dense  than  in  the 
ordinary  forms  of  secondary  degeneration,  or  in  tabes,  or  in  lateral 
sclerosis.  Occasionally  in  the  mass  of  sclerotic  tissue  a  distended  axis 
cylinder  has  produced  a  cavity,  and  the  cord  presents  the  cribriform 
appearance,  or  Swiss  cheese  appearance,  which  has  been  described  as 
occurring  in  acute  myelitis.  Usually  the  sclerosis  is  thicker  at  the 
periphery  of  the  cord,  and  is  associated  with  adhesions  of  the  pia  and 
with  chronic  meningitis.  It  has  a  tendency  to  be  thicker  around  the 
bloodvessels  that  enter  the  cord  from  the  periphery,  and  very  often 
wedge-shaped  patches  of  sclerosis  are  seen  with  the  apex  pointing 
inward.  It  is  evident  that  the  neuroglia  of  the  cord  is  increased  in 
density  and  also  that  the  connective  tissue  in  the  vessel  walls  is 
thickened.  All  throughout  this  sclerotic  tissue  a  fine  cell  infiltration 
is  found,  more  intense  about  the  bloodvessels,  but  everywhere  present 
in  the  cord.  If  this  irregular  sclerotic  process  has  gone  on  chiefly  in 
the  surface  of  the  cord  the  so-called  annular  sclerosis  is  found,  a  ring 
of  sclerotic  tissue  surrounding  the  fairly  normal  fibres  of  the  cord,  the 
gray  matter  not  being  greatly  affected.     This  condition  is  rare. 

The  second  pathological  change  present  in  chronic  myelitis  is  an 
advanced  degeneration  of  the  nerve  fibres  and  of  the  nerve  cells  through- 
out the  cord.  This  may  be  very  irregular  in  its  distribution ;  and  its 
extent  at  the  time  of  the  autopsy  is  very  often  much  greater  than  the 
extent  of  the  initial  sclerosis  to  which  it  is  secondary.  Secondary 
degeneration  follows  each  nerve  fibre  to  the  end  where  it  is  interrupted 
at  any  point  in  its  connection  with  its  proper  neurone  body.  Thus  a 
small  patch  of  sclerosis  at  one  level  of  the  lateral  column  may  be  fol- 
lowed by  a  long  descending  degeneration  in  that  column.     And  a  small 


CHBONIC  MYELITIS.  413 

patch  of  scl^osis  in  the  posterior  column  may  give  rise  to  ascending 
degeneration  as  high  as  the  medulla. 

It  is  not  always  possible  to  determine  from  a  microscopic  examina- 
tion whether  the  primary  process  in  a  chronic  myelitis  is  due  to  a 
degeneration  of  the  nerve  fibres  with  secondary  sclerosis,  or  whether 
it  is  due  to  a  primary  sclerosis  with  compression  and  secondary  degen- 
eration of  the  nerve  fibres.  Both  conditions  are  found  in  the  terminal 
stage  of  the  disease.  In  some  sections  it  is  evident  that  the  thickening 
of  connective  tissue  is  greater  than  that  which  occurs  in  an  ordinary 
replacement  hyperplasia.  In  other  regions,  however,  the  sclerosis  is 
quite  comparable  to  that  ordinarily  found  in  secondary  degenerations. 
It  is  this  mingling  of  the  two  processes  in  chronic  myelitis  which  gives 
rise  to  the  irregular  appearance  found.  Enormous  swelling  of  the  axis 
cylinders,  such  as  is  found  in  acute  myelitis,  is  occasionally  observed 
in  chronic  myelitis  ;  and  granular  corpuscles,  the  products  of  degenera- 
tion, are  scattered  everywhere  through  the  spinal  cord.  The  distribu- 
tion of  these  lesions  through  the  cord  varies  in  different  cases,  both 
transverse  and  disseminated  chronic  myelitis  being  found. 

Symptoms.  —  The  symptoms  of  chronic  myelitis  are  such  as  might 
be  expected  from  a  chronic  implication  of  the  various  columns  of  the 
spinal  cord  and  of  the  various  segments  of  the  cord  in  their  gray  mat- 
ter. The  process  is  such  a  slow  one,  however,  and  so  diflFerent  in  dif- 
ferent cases  that  no  typical  course  can  be  described  in  this  disease.  A 
general  feeling  of  weakness  in  the  legs,  undue  fatigue  on  exertion,  going 
on  gradually  to  a  state  of  paralysis  of  greater  or  less  degree,  perhaps 
not  sufficient  to  incapacitate  the  patient  for  many  years,  is  the  usual 
history.  The  paralysis  is  usually  of  the  spastic  type,  with  rigidity  in 
the  muscles,  but  it  may  be  confined  to  a  few  muscles  in  the  limb  affected, 
and  these  muscles  may  show  atrophy  and  reaction  of  degeneration.  As 
a  rule,  there  is  in  the  early  stage  an  increase  in  the  tendon  reflexes, 
with  ankle  clonus,  and  this  may  persist  for  several  years  and  finally 
subside,  and  in  the  later  stages  the  reflexes  may  be  lost.  Occasionally, 
if  the  lesion  happens  to  afi'ect  the  reflex  arc,  the  knee-jerks  are  lost  at 
the  outset.  The  legs  may  be  the  only  parts  paralyzed  for  many  years, 
or  the  paralysis  may  extend  within  the  first  year  to  the  arms,  or  it  may 
begin  in  the  arms,  but  usually  after  several  years  some  symptoms  of 
paralysis  are  present  in  all  the  extremities.  The  paralysis  rarely  is  as 
extreme  as  it  is  in  the  forms  of  muscular  atrophy,  and  therefore  rarely 
gives  rise  to  permanent  deformities  or  contractures.  These,  however, 
are  occasionally  seen. 

Disturbances  in  the  sensory  sphere  are  very  common  in  myelitis. 
These  usually  consist  of  irregular  parsesthesise  which  may  be  followed 
by  plaques  of  ansesthesia  and  analgesia.  Pain  is  not  a  common  symp- 
tom, excepting  in  so  far  as  the  general  stiffness  and  weakness  give  rise 
to  extreme  sensations  of  fatigue  or  painful  affections  of  the  muscles 
paralyzed,  l)ut  pain  in  the  back  is  not  uncommon  when  the  myelitis  is 
associated  with  a  chronic  meningitis.  In  these  cases  also  some  shoot- 
ing pains  in  the  limbs  and  in  the  body  may  develop. 


414  MYELITIS    ANT)    MYELOMALACIA. 

Trophic  disturbances  are  quite  common  in  the  course  of  chronic 
myelitis.  Urticaria,  irregular  eruptions  U})on  the  skin,  vasomotor 
paralysis,  giving  rise  to  flushes  or  streaks  of  pallor,  irregular  growth 
of  the  hair  and  of  the  nails,  trophic  disturbances  of  the  joints  and  of 
the  bones  are  not  uncommonly  observed  in  the  course  of  chronic  mye- 
litis. In  the  last  stage  of  the  disease  bed-sores  are  a  distinct  danger. 
But  all  these  symptoms  may  vary  in  intensity,  and  many  of  them  may 
be  recovered  from  with  proper  care. 

In  the  majority  of  cases  as  the  result  of  a  long-continued  chronic 
myelitis  the  patient  is  reduced  to  a  state  of  helpless  paralysis.  He  is 
confined  to  the  bed,  the  limbs  are  wasted  and  very  often  drawn  up  in 
a  condition  of  contracture,  extreme  flexion  of  the  thighs  and  legs  with 
adduction  of  the  thighs  being  present.  In  these  cases  the  hands,  too, 
become  helpless,  and  the  patient  is  reduced  to  the  most  abject  state  of 
paralysis.  A  complicating  cystitis  not  infrequently  develops,  quite 
early  in  the  course  of  chronic  myelitis,  as  the  irregular  action  of  the 
bladder  may  necessitate  the  constant  use  of  catheters  which  convey 
infection.  Cystitis  and  bed-sores  are  the  common  cause  of  death  in 
this  disease,  though,  if  the  sclerosis  invades  the  upper  portion  of  the 
cord,  affections  of  respiration  and  deglutition  may  be  the  active  cause 
of  death.  Many  symptoms  referable  to  an  implication  of  the  sympa- 
thetic nervous  system  are  usually  observed  in  the  course  of  chronic 
myelitis,  crises  such  as  occur  in  locomotor  ataxia  having  been  observed 
frequently.  If  the  first  dorsal  segment  of  the  cord  happens  to  be 
affected  irritation  or  paralysis  of  the  sympathetic  in  the  neck  will  give 
rise  to  pupillary  symptoms. 

Diagnosis.  —  The  diagnosis  of  chronic  myelitis  is  to  be  made  entirely 
by  exclusion.  When  all  other  forms  of  spinal-cord  disease  can  be 
eliminated,  and  yet  objective  spinal  symptoms  of  irregular  distribution 
are  present,  chronic  myelitis  may  be  diagnosticated.  When  the  typical 
symptoms  of  the  various  spinal-cord  affections  are  considered,  it  will 
be  evident  that  they  are  none  of  them  likely  to  be  confounded  with 
chronic  myelitis.  The  most  difficult  diagnosis  is  from  disseminated 
sclerosis.  In  this  disease,  however,  it  is  to  be  noted  that  nystagmus 
and  other  cerebral  symptoms  appear  early,  of  which  the  most  notice- 
able are  the  scanning  speech  and  the  intention  tremor.  The  develop- 
ment, therefore,  of  cerebral  symptoms  in  the  course  of  the  case  of  sup- 
posed chronic  myelitis  will  indicate  that  the  original  diagnosis  should 
have  been  disseminated  sclerosis.  In  the  early  stage  of  primary  lateral 
sclerosis  or  of  amyotrophic  lateral  sclerosis,  where  the  only  symptoms 
are  spastic  paraplegia  and  where  no  ostensible  cause  can  be  found,  the 
supposition  of  a  transverse  chronic  myelitis  of  the  dorsal  region  may 
be  entertained ;  but  this  diagnosis  can  only  be  substantiated  when  the 
development  of  sensory  symptoms  and  disturbance  in  the  action  of  the 
bladder  and  rectum  make  it  probable  that  the  lesion  is  more  extensive 
than  one  affecting  the  pyramidal  tracts  of  the  spinal  cord  alone. 

Prognosis.  —  The  prognosis  in  chronic  myelitis  is  uniformly  unfay^ 
orable,  and  the  patients  are  destined  to  a  life  of  invalidism. 


SENILE  PABAPLEGIA.  415 

Treatment.  —  The  treatment  of  chronic  myeUtis  is  one  of  care  of 
the  general  health,  the  various  symptoms  of  the  disease  being  coun- 
teracted as  they  arise,  so  far  as  possible,  and  the  patient  kept  in  a  state 
of  comfort.  All  the  measures  already  recommended  in  connection  with 
the  treatment  of  locomotor  ataxia  may  be  carried  out  in  the  course  of 
chronic  myelitis,  and  are  not  infrequently  attended  by  temporary 
improvement.  This  is  particularly  true  of  hydrotherapeutic  treatment 
and  the  careful .  application  of  massage.  A  prevention  of  any  of  the 
complicating  diseases  which  cause  death  will  be  a  duty  in  the  latter 
stages. 

SENILE  PARAPLEGIA. 

People  of  advanced  years,  especially  those  who  are  rather  feeble,  are 
occasionally  afflicted  by  a  slowly  progressive  weakness  of  the  legs, 
attended  by  pain  and  sensations  of  numbness,  undue  fatigue  upon  exer- 
tion, and  possibly  with  a  slight  degree  of  difficulty  in  the  retention  of 
urine.  These  symptoms  give  rise  to  the  suspicion  that  they  are  about 
to  develop  chronic  myelitis.  They  are  of  very  slow  progress  and  pro- 
duce disturbance  of  walking  which  results  in  a  slight  dragging  of  the 
feet,  or  rather  shuffling  gait,  difficulty  in  getting  up  stairs  or  stepping 
up  into  a  carriage,  but  not  attended  by  any  true  ataxia  of  movement. 
A  great  sense  of  weariness  is  usually  experienced  in  these  patients  after 
any  exertion,  and  they  may  be  the  subjects  of  senile  tremors  of  the 
hands  or  of  the  head.  Very  many  patients  afflicted  in  this  way  are 
the  subjects  of  chronic  arterial  disease  or  of  chronic  affections  of  the 
heart.  The  condition  may  develop  occasionally  quite  rapidly,  all  the 
symptoms  appearing  within  a  few  days,  and  making  it  difficult  for  the 
patient  to  move  about  at  all.  In  my  own  experience  the  affection  is 
more  common  in  women  than  in  men.  It  is  probably  due  to  some  dis- 
turbance of  circulation  in  the  lower  part  of  the  spinal  cord,  either  of 
the  nature  of  an  arterial  anaemia  or  of  a  venous  congestion.  This  seems 
likely  because  of  the  fact  that  in  the  majority  of  cases  absolute  rest  in 
bed,  skilful  massage,  the  use  of  hot  applications  to  the  spine,  followed 
by  cool  douching,  heart  stimulants,  and  general  tonics  result  in  a  pro- 
gressive recovery. 

Autopsies  are  wanting  to  establish  the  pathology,  as  no  fatal  cases 
have  been  recorded. 

Disseminated  or  insular  sclerosis  of  the  spinal  cord  is  so  commonly 
associated  with  similar  lesions  in  the  brain  that  it  will  be  discussed  in 
connection  with  brain  diseases. 


CHAPTER   XXII. 


INJUEIES  OF  THE  SPINAL  COED.     H^MATOMYELIA. 

Injueies  and  wounds  of  the  spinal  cord,  producing  a  laceration  of 
the  cord  and  hemorrhages  within  it  or  in  the  spinal  canal,  are  of  fre- 
quent occurrence.  The  usual  cause  of  such  injuries  to  the  cord  are 
fractures  and  dislocations  of  the  vertebrse.  It  is  natural  from  the 
length  of  the  organ  and  from  its  location  within  a  narrow  cylindrical 
bony  canal  that  any  injury  to  the  wall  surrounding  it  should  have  a 
direct  or  indirect  effect  upon  it.  And  it  is  rare  for  a  fracture  or  a  dis- 
location of  the  vertebra  to  occur  without  producing  immediate  damage 
of  the  spinal  cord. 

Fig.  182. 


\ 


Hemorrhage  in  the  central  gray  matter  on  the  left  side,  due  to  fracture  of  the  spine. 
First  dorsal  segment. 

Pathology. — Such  an  injury  is  usually  attended  by  a  compression 
and  bruising,  or  by  a  laceration  of  the  cord,  a  disintegration  of  its 
substance,  and  a  considerable  hemorrhage  that  perforates  the  cord  and 
is  followed  by  an  inflammatory  process  that  may  intensify  the  disinte- 
gration or  may  go  on  to  an  attempt  at  repair  by  the  formation  of  an 
extensive  cicatrix.  Fig.  182  shows  the  appearance  of  a  spinal  cord 
after  a  fracture  of  the  sixth  cervical  vertebra.  The  eighth  cervical 
segment  was  crushed  and  disintegrated,  the  typical  form  of  the  gray 
matter  being  obliterated  and  the  tracts  in  the  white  matter  torn  across. 

416 


PATHOLOGY.  417 

A  large  hemorrhage  occurred  which  penetrated  the  cord  and  destroyed 
the  gray  master  for  some  distance  above  and  below  the  eighth  cervical 
segment,  being  largely  confined  to  the  gray  matter,  especially  of  the 
posterior  horn.  The  clot  followed  the  line  of  least  resistance,  which  is 
always  within  the  gray  matter.  (Fig.  183.)  lu  another  case,  where 
the  fracture  occurred  in  the  dorsal  region,  the  hemorrhage  surrounded 
the  cord  above  and  below  the  destroyed  segment,  filling  the  spinal 
canal  within  the  dura. 

When  the  cord  is  exposed  at  the  autopsy  or  in  a  surgical  operation 
for  the  repair  of  the  fracture,  its  external  appearance  may  not  be 
changed,  and  if  the  pia  and  sheath  be  not  ruptured   there  may  be 

Fig.  183. 


Clot  in  the  posterior  horn  of  the  left  side,  three  segments  above  the  preceding  figure. 
Sixth  cervical  segment. 

very  little  evidence  of  the  extreme  destruction  present.  It  is  only 
upon  section  that  the  lesion  is  evident.  Thus,  in  the  case  from  which 
the  figures  are  taken  external  inspection  showed  no  lesion  of  the  cord. 
But  on  cutting  the  cord  the  general  disintegration  and  hemorrhage  were 
visible.  Bullet  wounds  and  stab  wounds  of  the  cord  cause  a  similar 
disintegration  of  the  spinal  elements  and  a  hemorrhage  without  or 
within  the  cord.  These  are  more  likely  to  be  followed  by  purulent  in- 
flammation, the  result  of  septic  material  brought  in  with  the  bullet  or 
knife.  This  inflammatory  condition  involves  the  meninges  as  well  as 
the  cord  itself,  and  may  go  on  to  purulent  myelitis  or  to  abscess  of  the 
cord  with  total  destruction  in  a  transverse  direction.  Hemorrhages 
within  the  cord  cause  a  long,  narrow,  cylindrical  clot  usually  within 
the  gray  matter,  sometimes  in  the  posterior  columns,  very  rarely  in  the 

27 


418  INJURIES    OF    THE    SPINAL    CORD.     H^MATOMYELIA. 

lateral  columns.  The  maximum  destruction  is  limited  to  one  or  two 
segments,  but  the  clot  may  perforate  a  number  of  segments,  even  to 
one-half  the  lengtli  of  the  cord.  Sometimes  many  little  clots  are 
found  at  diflFerent  levels.  Occasionally  the  capsule  of  the  cord  is 
broken  and  the  hemorrhage  reaches  the  pia  and  infiltrates  it.  If  the 
patient  dies  at  once  the  clot  is  found  surrounded  by  broken-down  cord 
tissue  filled  with  small  cells  and  granular  corpuscles  —  pigment  and 
hsematin  crystals.  If  the  patient  survives  a  few  weeks  the  clot  may 
be  somewhat  absorbed  and  contracted,  the  tissue  about  it  may  be 
yellowish-red  from  fatty  degeneration  of  the  nerve  elements,  and  the 
cells  and  fibres  will  be  found  in  all  stages  of  disintegration.  If  the 
patient  lives  for  some  years  the  clot  may  be  entirely  absorbed,  leaving 
either  a  long  scar  of  connective  tissue  or  a  long  cavity  lined  with  con- 
nective tissue — the  hemato-myelo-porus  of  Van  Gieson.  Hemor- 
rhage from  the  vessels  of  the  pia  mater,  causing  compression  of  the 
cord  by  a  clot,  may  occur.  Janeway  has  described  such  a  condition 
occurring  in  a  patient,  the  subject  of  hemophilia.  I  have  seen  one 
case  which  came  on  suddenly  after  unusual  exertion. 

In  the  patients  who  survive  secondary  degenerations  develop  in  the 
cord,  as  in  cases  of  transverse  myelitis,  above  and  below  the  injured 
segments ;  secondary  degeneration  also  occurs  in  the  motor  nerves  from 
the  segment  injured,  to  the  muscles,  and  atrophy  of  the  muscles,  as  in 
anterior  poliomyelitis. 

Etiology.  —  Hemorrhage  within  the  spinal  canal  or  within  the  cord 
may  be  the  result  of  severe  blows  or  falls  or  of  direct  concussion. 
Thus  I  have  seen  multiple  hemorrhages  in  the  pia  with  a  number  of 
small  clots  outside  the  cord  following  a  fall  through  an  elevator  shaft 
down  five  stories.  I  have  also  seen  a  hemorrhage  in  the  conns  ter- 
minalis  due  to  the  patient  sitting  down  where  there  was  no  seat  and 
injuring  the  buttocks.  Hemorrhages  may  also  occur  as  the  result  of 
spontaneous  rupture  of  bloodvessels,  either  after  great  effort  or  after 
long-continued  arterial  disease.  In  one  case  a  severe  sudden  bending 
forward  of  the  neck  without  dislocation  or  fracture  was  followed  by  a 
hemorrhage  within  the  cord  which  penetrated  through  the  cervical  and 
half  the  dorsal  segments,  causing  a  long  tubular  clot.  In  another  case 
the  lifting  of  an  unusual  weight  was  immediately  followed  by  a  hemor- 
rhage in  the  lower  lumbar  region  which  destroyed  the  lumbar  and 
sacral  enlargement  and  infiltrated  the  cauda  equina,  the  latter  being 
found  embedded  in  the  clot  at  the  autopsy. 

Symptoms.  —  When  a  vertebra  is  dislocated  or  fractured  a  displace- 
ment either  of  the  spinous  process  or  of  the  body  of  the  vertebra  can 
be  felt,  causing  a  double  deformity.  The  muscles  about  the  fractured 
bone  are  thrown  into  a  state  of  rigidity,  and  a  fixation  of  the  back  or 
neck  in  an  abnormal  position  occurs.  Any  motion  causes  great  pain 
both  at  the  point  of  injury  and  in  the  domain  of  nerves  which  are 
compressed  or  lacerated.  The  exact  character  of  the  injury  can  only 
be  determined  by  an  examination  with  the  Roentgen  rays.     There  are 


SYMPTOMS.  419 

some  cases  on  record  where  slight  fractures  or  partial  dislocations 
«jaused  no  •Spinal  symptoms.  There  are  many  cases  where  the  spinal 
symptoms  exceed  in  severity  any  apparent  surgical  injury,  for  disloca- 
tion may  be  temporary  and  spontaneously  reduced.  The  surgical 
aspect  of  the  case,  however,  is  of  less  interest  than  the  symptoms  of 
spinal  lesion.  These  depend  wholly  upon  the  position  of  the  injury; 
the  higher  the  fracture  the  more  extensive  the  symptoms,  for  the  injury 
usually  arrests  all  voluntary  motion  and  conscious  sensation  in  the  parts 
below  the  level  of  the  lesion.  Hence  the  level  of  the  symptoms  indi- 
cates the  level  of  the  lesion.  The  facts  of  the  localization  of  spinal 
functions  already  presented  in  detail  need  not  be  reproduced  here ;  but 
if  carefully  studied  they  will  permit  an  accurate  diagnosis  of  the  posi- 
tion of  the  lesion  to  be  reached.  Particular  attention  should  be  paid 
to  the  posture  involuntarily  assumed  by  the  patient  in  bed  (see  page 
66),  to  the  extent  'of  the  paralysis  (see  page  48),  and  to  the  distri- 
bution of  the  anaesthesia  (see  page  73). 

In  any  case  of  injury  of  the  cord  a  state  of  paraplegia  is  produced 
at  once.  The  paralysis  is  complete,  the  limbs  are  relaxed  and  flaccid, 
the  reflex  action  is  much  diminished,  and,  in  cases  of  transverse  lesion 
with  entire  division  of  the  cord,  is  lost ;  there  is  incontinence  of  urine 
with  retention  and  a  paralysis  of  the  rectum ;  there  is  often  a  state  of 
priapism ;  there  are  pains  in  the  back  at  the  level  of  the  fracture  and 
in  the  region  of  the  body  to  which  the-  nerves  near  the  fracture  go ; 
these  pains  are  often  attended  by  twitchings  of  the  muscles  which  cause 
much  discomfort ;  there  is  a  condition  of  anaesthesia  more  or  less  com- 
plete whose  upper  limit  is  easily  defined  and  corresponds  to  the  level 
of  the  lesion ;  there  is  a  zone  of  hyperaesthesia  just  above  the  anaesthetic 
level.  The  segment  of  the  cord  which  is  affected  in  any  particular 
fracture  is  the  one  that  is  opposite  the  fractured  vertebra.  The 
researches  of  Reid^  have  demonstrated  that  there  is  not  a  uniform 
relation  between  the  vertebrae  and  the  segments  of  the  cord.  The 
diagram  (Fig.  184)  of  Dejerine  shows  the  usual  relation  between 
the  various  segments  of  the  cord  -and  the  spines  and  bodies  of  the 
vertebrae. 

Chipault  has  given  a  practical  set  of  rules  for  determining  the  rela- 
tion of  the  segments  to  the  spinous  processes  of  the  vertebrae.  He  says : 
"In  the  cervical  region  add  one  to  the  number  of  the  vertebra,  and 
this  will  give  the  segment  opposite  to  it.  In  the  upper  dorsal  region 
add  two;  from  the  sixth  to  the  eleventh  dorsal  vertebra  add  three. 
The  lower  part  of  the  eleventh  dorsal  spinous  process  and  the  space 
below  it  are  opposite  the  lower  three  lumbar  segments.  The  twelfth 
dorsal  spinous  process  and  the  space  below  it  are  opposite  the  sacral 
segments."  The  spinal  cord  ends  opposite  the  body  of  the  first  lumbar 
vertebra  or  opposite  the  cartilage  in  the  space  below  it.  The  lower 
half  of  the  spinal  canal  is,  therefore;  occupied  by  the  cauda  equina 
only.     In  infants  the  cord  reaches  as  low  down  as  the  body  of  the 

'Journal  of  Anatomy  and  Physiology,  vol.  xxiii.,  p.  312. 


420        INJURIES  OF  THE  SPINAL  COBD.    IIJi:MATOMYELTA. 


Fig.  184. 


N.  to  rectus  lateralis 

.'^to  rectus  antic,  minor 
Aiuisloniobis  with  hypoglossal 

Anastomosis  with  pneutnogastric 
N.  to  rectus  antic.major. 
N.  to  mastoid  region. 
.Great  auricular  n. 
■Transverse  cervical  n. 
'-.Ei^\N.  to  Trapezius,  Ang.  Scap.  and  Rhomboid . 

Supra  clavicular  n. 
_Supra-acromiaL  n. 
_  Phrenic  n. 

A',  to  levator  ang.  scap, 

N.  to  rhomboid 

Subscapular  n. 

Subclavicular  n. 


jV.  to  peetoralis  major. 


Circumflex  n. 

Blusculo-cutaneous  n. 

Median  n. 

Radial  n. 

Ulnar  n. 

Internal  cutaneous  «. 

Small  internal  cutaneous  ». 


Ilio-hijpogaslric  n. 
.llio-inguinal  n. 


—External  cutantous  n, 
Genito-crural  ;i. 


Anterior  crural  n. 
' Obturator  n. 


JV.  to  levator  ani. 

If.  to  obturator 

N.  to  ophincter  ani..^ 

Coccygeal  ?i, 


Superior  gluteal  n. 


JV.  to  pyriformis 

lY.  to  gemellus  super. 


^ N.  to  gemellus  infer. 

df.  to  guadratus 

.Small  sciatic  n, 
-Ssiatic  n. 

The  relation  of  the  segments  of  the  spinal  cord  and  their  nerve  roots  to  the  bodies  and  spines  of 
the  vertebr*.     (Dejerine  et  Thomas,  Mai.  d.  1.  Moelle  Epinifere,  Paris,  1902.) 


INJURIES  OF  THE  SPINAL  CORD.    H^MATOMYELIA.        421 

third  lumbar  vertebra,  but  by  the  age  of  twelve  years  the  lower  limit 
is  opposite  the  body  of  the  second  vertebra. 

Certain  special  symptoms  attend  fractures  at  different  levels  : 

I.  Fractures  of  the  upper  four  cervical  vertebrae  are  usually  attended 
by  sudden  death  either  from  involvement  of  the  vital  centres  in  the 
adjacent  medulla  or  by  a  lesion  of  the  centres  of  the  phrenic  nerves 
and  respiratory  paralysis.  In  a  few  patients  who  have  survived  for  a 
few  days  pain  has  been  felt  in  the  great  occipital  nerves  ;  the  head  has 
been  held  rigid,  and  any  motion  of  it  has  been  very  painful ;  a  deformity 
has  been  felt  in  the  pharynx  ;  a  total  paralysis  of  the  entire  body  below 
the  neck  has  occurred,  and  high  or  subnormal  temperature  with  very 
rapid  pulse  have  been  present.     Death  always  follows  soon. 

II.  Fractures  of  the  lower  three  cervical  and  first  dorsal  vertebrae 
are  quite  common,  over  one-quarter  of  spinal  fractures  being  in  this 
location.  Divers  into  shallow  water,  laborers  whose  heads  are  bent 
forward  upon  their  bodies,  and  persons  struck  by  heavy  objects  falling 
Qn  the  shoulders,  or  who  fall  from  a  height  in  such  a  manner  that  the 
body  is  bent  upon  the  head  usually  fracture  one  or  more  of  these  cervi- 
cal vertebra.  In  such  fractures  either  the  upper  or  the  lower  half  of 
the  cervical  enlargement  of  the  cord  is  injured. 

(a)  When  the  upper  part  of  the  cervical  enlargement  is  injured  the 
paralysis  of  the  arms  is  complete  and  the  arms  lie  relaxed  at  the  side 
of  the  patient ;  pain  is  felt  in  the  neck  and  shoulders,  and  spasms  of 
the  arm  muscles  are  frequent ;  anaesthesia  is  complete  below  the  deltoid 
area  over  the  shoulder-joint  (Fig.  185) ;  there  is  no  reflex  activity  in 
the  upper  extremities,  but  there  may  be  some  reflex  activity  in  the 
legs  after  the  shock  passes  off,  and  this  soon  becomes  exaggerated ; 
there  is  usually  constant  priapism,  and  retention  of  urine  which  must 
be  relieved  by  catheter ;  there  is  paralysis  of  the  rectum ;  there  is  a 
marked  tendency  to  bed-sores,  respiration  is  wholly  diaphragmatic ; 
coughing  and  expiratory  efforts  are  impossible  ;  a  high  fever  and  rapid 
pulse  develop ;  unusual  sweating  of  the  entire  body  occurs,  and,  as  a 
rule,  death  follows  during  the  first  or  second  week.  If  the  patient 
survives  a  state  of  nearly  complete  paralysis  remains,  with  atrophic 
paralysis  of  the  arms  and  spastic  paralysis  of  the  legs. 

(6)  When  the  lower  part  of  the  cervical  enlargement  is  injured  the 
paralysis  of  the  arms  is  partial,  the  shoulder  muscles  and  the  flexors 
of  the  forearms  escaping,  and  sometimes  some  of  the  muscles  of  the 
forearms  also  escape.  In  this  form  the  patient  lies  with  the  arms 
abducted  from  the  sides  and  the  forearms  flexed,  the  hands  resting  on 
the  chest ;  the  body  and  legs  are  paralyzed  and  flaccid,  pain  is  felt  in 
the  hands  and  on  the  inner  side  of  the  forearms  and  often  about  the 
body ;  the  anaesthesia  is  more  marked  on  the  inner  side  of  the  arms  and 
forearms  and  is  total  in  the  hands  (Figs.  186  to  188) ;  it  affects  the 
body  also,  but  the  upper  limit  of  anaesthesia  on  the  body  may  be  as 
low  as  the  ni[)plc  ;  there  is  a  region  of  hyperaesthesia  on  the  outer  side 
of  the  arm  and  about  the  chest  above  the  nipple  ;  there  may  be  a 
temporary  suspension  of  reflex  action  in  the  legs,  followed  later  by 


422       INJURIES  OF  THE  SPINAL  CORD.     H^MATOMYELIA. 


exaggeration  of  all  the  reflexes ;  there  is  priapism,  retention  of  urine, 
paralysis  of  the  bladder  and  rectum ;  there  is  an  oculo-pupillary  par- 
alysis with  contraction  of  the  pupil,  failure  to  dilate  when  the  neck  is 
scratched,  and  a  narrowing  of  the  palpebral  fissure,  and  retraction  of 


Fig.  185. 


Fig.  186. 


Anaesthesia  caused  by  a  lesion  of 
the  fifth  cervical  segment. 


Anaesthesia  caused  by  a  lesion  of  the  sixth  cervical 
segment  on  the  right  side,  and  involving  the  fifth  seg- 
ment of  the  left  side. 


the  eyeball,  these  symptoms  indicating  a  lesion  of  the  first  dorsal  seg- 
ment, but  being  frequently  present  with  any  injury  of  the  lower  cervi- 
cal enlargement.  The  paralysis  is  not  always  symmetrical  in  the  arms^ 
and  many  muscles  may  escape  if  the  lesion  is  low  down  in  the  en- 


FiG.  187. 


Fig.  188. 


Anaesthesia  caused  by  a  lesion  of  the  seventh  Anaesthesia  caused  by   a  lesion   of  the  first 

cervical  segment,  almost  symmetrical.  Dotted  dorsal  segment.  Dotted  area  hyperaesthetic. 
area  hyperaesthetic.     (Herter.)  (Bruns.) 

largement.  (Fig.  189.)  If  the  patient  recovers  a  state  of  atrophic 
paralysis  in  the  upper  extremities  of  greater  or  less  extent  remains  and 
a  condition  of  spastic  paralysis  in  the  legs. 

III.  Fractures  of  the  dorsal  vertebrae  are  rather  infrequent.     They 


INJURIES  OF  THE  SPINAL  CORD.    H^MATOMYELIA.        423 

cause  a  paralysis  of  the  legs  and  abdominal  muscles  with  loss  of  con- 
trol of  bladder  and  rectum.  There  is  a  distinct  girdle  sensation  often 
attended  by  pain.     There  is  a  line  of  anaesthesia  about  the  trunk  above 


189. 


Anaesthesia  of  the  right  side  due  to  lesion  of  the  fifth  cervical  segment ;  of  the  left  side  due  to 
lesion  of  the  first  dorsal  segment ;  tongue-like  extension  of  ansesthesia  in  axilla  and  under  the  arm. 
(Wiohmann.) 

which  is  a  zone  of  hypersesthesia.  This  line  of  anaesthesia  is  usually 
the  most  important  guide  to  the  level  of  the  lesion,  but  it  is  not  on  a 
line  with  that  level.  (Figs.  190  and  191.)  It  is  from  three  to  four 
inches  below  it,  for  there    is  an  overlapping  or  anastomosis  of  the 


Fig.  190. 


Fig.  191. 


Ansesthesia  from  injury  of  the  seventh  dorsal 
segment,  showing  the  anjesthesia  curve  about  the 
body  and  extending  higher  behind  than  in  front. 
(Wichmann.) 


Anaesthesia  due  to  lesion  of  the  eleventh 
dorsal  segment.     (Wichmann.) 


sensory  filaments  of  nerves,  and  the  intercostal  nerves  of  one  segment 
supply  the  skin  directly  related  to  two  or  even  three  adjacent  segments, 
as  Sherrington^  has  shown.      (Fig.  192.)     Furthermore,  the  sensory 

^Transactions  of  the  Royal  Philosophical  Society,  London,  1893,  vol.  clxxxiv. 


424       INJURIES  OF  THE  SPINAL  CORD.     H^MATOMYELIA. 

fibres  entering  the  cord  ascend  to  the  two  higher  segments  before 
terminating,  and  hence  a  transverse  lesion  of  the  cord  does  not  cut  off 
all  the  sensations  from  the  nerve  roots  at  its  level.  This  will  be 
understood  by  reference  to  Fig.  22.  Thus  in  a  case  of  fracture  of  the 
sixth  dorsal  vertebra  the  eighth  dorsal  segment  was  injured,  and  the 
line  of  anaesthesia  corresponded  to  the  distribution  of  the  tenth  dorsal 
nerve,  being  three  inches  below  the  level  of  the  injury.  The  zone  of 
hypersesthesia  which  lies  above  the  level  of  the  ansesthesia  is  due  to 
the  fact  that  sensations  received  in  segments  of  the  cord  just  above  the 
lesion  which  are  in  a  state  of  inflammatory  irritation  are  felt  to  an 
exaggerated  degree.  The  girdle  sensation  is  a  consciousness  of  this 
hypersensitive  skin.     If  the  cord  is  not  severed  and  is  capable  of 

Fig.  192. 


^ 


Tth  thoracic 
sensory 
spinal 
SKin-Jield 


wwww 


'II//IIIIIII/I 
\%\\\\\\\\\\\ 


mil 


WW 


\W\\\\\\\\\\\\\\\\\\\\\ 


\\w\\\\ 


LLUlUlLiL 


w 


Illrd  thoracic 


Vth  thoracic 


Diagram  showing  tlie  nerve  supply  of  the  slcin  of  the  thorax  ;  every  part  is  supplied 
Dy  two  nerves.     (Sherrington. ) 


transmitting  some  sensations  through  the  lesion,  patients  often  refer  to 
this  hypersensitive  zone,  all  sensations  coming  up  from  the  legs.  Thus 
in  one  of  my  patients  a  pin-prick,  or  pinch,  or  touch,  or  a  thermal 
sensation  produced  in  the  paralyzed  and  anaesthetic  legs  or  trunk,  was 
always  felt  about  the  level  of  the  sixth  dorsal  nerve.  If  on  the  back 
of  the  body  it  was  referred  to  the  back ;  if  in  front,  to  the  front,  and 
it  was  always  felt  on  the  side  which  was  irritated.  This  man  had  a 
transverse  injury  of  the  cord  due  to  fracture  at  the  fifth  dorsal  vertebra 
and  had  been  paraplegic  four  years  when  examined.  I  have  seen  this 
symptom  in  many  cases,  but  have  not  seen  it  described.  It  is  of 
service  in  distinguishing  total  from  partial  lesions  of  the  cord.  In 
cases  of  dorsal  injury  the  initial  symptoms  may  slowly  subside  in  part. 
The  patient  remains,  however,  in  a  state  of  spastic  paraplegia  with  all 
the  symptoms  of  lateral  sclerosis  due  to  the  descending  degeneration 
in  the  motor  tracts  of  the  cord.  The  danger  of  bed-sores  and  cystitis 
is  directly  proportionate  to  the  degree  of  anaesthesia  remaining.  Some- 
times these  patients  live  for  many  years. 

It  is  in  cases  of  dorsal  injury  that  the  syndrome  due  to  a  lesion  of 
one  lateral  half  of  the  cord,  known  as  Brown-Sequard  paralysis,  is 
most  commonly  seen,  although  it  may  result  from  a  lesion  at  any  level. 
This  combination  of  symptoms  is  as  follows : 

1.  On  the  side  of  the  lesion  there  is  paralysis  with  rigidity;  an 


INJURIES  OF  THE  SPINAL  GOBD.    H^MATOMYELIA. 


425 


Fig.  193. 


.X 


increase  of  tendon  reflexes;  vasomotor  paresis  causing  a  temporary- 
elevation  of  temperature ;  a  diminution  in  muscular  sense  and  of  the 
sense  of  position  of  the  leg;  a  general  hypersensitive  state  to  touch, 
temperature,  and  pain  up  to  the  level  of  the  lesion,  which  may  subside 
after  a  time ;  there  is  a  narrow  zone  of  anaesthesia  about  the  body  to 
touch,  temperature,  and  pain,  the  latter  two  being  lost  a  little  lower 
than  touch. 

2.  On  the  side  opposite  the  lesion  there  is  no  paralysis ;  there  is  a 
slight  increase  of  tendon  reflexes;  there  is  anaesthesia  to  touch,  tem- 
perature, and  pain  up  to  a  line  passing  about  the  body  about  an  inch 
lower  than  the  zone  of  anaesthesia  on  the  side  of  the  lesion.  The 
accompanying  diagram  (Fig.  193),  illustrating  the  course  of  motor  and 
sensory  fibres  through  a  cord,  which 
is  supposed  to  have  a  lesion  involv- 
ing one  segment  on  the  left  side 
only,  demonstrates  the  manner  in 
which  these  symptoms  are  pro- 
duced. The  lesion  interrupts  the 
transmission  of  nervous  impulses  of 
various  kinds.  In  a  patient  of  my 
own  a  fracture  of  the  seventh  dor- 
sal vertebra  caused  this  syndrome, 
which  was  partly  relieved  by  oper- 
ation. The  fractured  lamina  that 
compressed  the  cord  was  removed, 
the  symptoms  gradually  subsided, 
but  even  at  the  end  of  eight  years 
there  remains  some  stiffness  of  the 
paralyzed  leg  and  an  appreciable 
difference  of  sensation  on  the  two 
sides  in  the  legs. 

IV.  Fractures  at  the  lower  two 
dorsal  and  upper  lumbar  vertebrae 
are  the  most  common  fractures  of 
the  spine,  over  one-half  of  the  cases 
being  in  this  locality.  Such  frac- 
tures injure  the  lumbar  enlargement 
of  the  cord. 

(a)  When  the  upper  part  of  the 
enlargement  is  destroyed  there  is 
total  paralysis  with  flaccid  condition 
in  the  abdominal  muscles  and  in 
the  muscles  of  the  thighs,  with  in- 
creasing atrophy  and  reaction  of  de- 
generation and  a  loss  of  reflex  action  ;   SSquard  paralysis. 

there  is  paralysis  without  atrophy  or 

change  in  electric  reaction  in  the  muscles  of  the  legs  and  feet;    the 

legs  lie  extended  in  bed  and  cannot  be  drawn  up;  there  is  loss  of 


AncEsthesiu, 
Analgesia 


Diagram  showing  the  eflfect  of  a  unilateral 
lesion   of   the   spinal    cord,    causing   "Brown- 


426       INJURIES  OF  THE  SPINAL  CORD.     HMMATOMYELIA. 


voluntary  control  of  the  bladder  and  rectum,  with  retention  of  urine ; 
compression  of  the  testicle  does  not  cause  a  contraction  of  the  abdomi- 
nal muscle  of  the  same  side  (Kocher's  reflex) ;  there  is  anaesthesia  as 
high  as  Poupart's  ligament  about  the  body,  and  the  girdle  sensation  is 
located  here.  Sometimes,  however,  when  the  upper  two  lumbar  seg- 
ments escape,  the  funnel-shaped  area  on  the  front  of  the  thigh  is  hyper- 
sensitive (Fig.  194),  a  sense  of  pain  is  felt  there,  and  the  anaesthesia  is 


Fig.  194. 


Fig.  195. 


Area  of  anaesthesia  in  a  lesion  of  the  lower  two  lum- 
bar segments  on  the  left  side  and  entire  lumbar  enlarge- 
ment on  the  right  side.     (Starr  and  McBurney.) 


Ansesthesia  after  a  lesion  of  the  fifth  lumbar 
segment.     (Eulenberg.) 


confined  to  the  area  corresponding  to  the  third  lumbar  segment  and  the 
segments  below  it.  If  these  patients  recover  in  part,  as  they  often  do, 
a  great  degree  of  inability  remains,  as  the  thighs  are  paralyzed. 

(6)  When  the  lower  part  only  of  the  lumbar  enlargement  is  de- 
stroyed, the  upper  portion  escaping,  the  paralysis  is  limited  to  the  legs 
below  the  knees,  or  even  to  the  peronei  and  feet,  and  these  muscles  are 
soon  atrophied  and  show  a  reaction  of  degeneration ;  the  thighs  are 
drawn  up  in  bed  and  the  legs  are  flexed ;  the  knee-jerks  are  sometimes 
present,  but  there  is  never  any  ankle  clonus  or  toe  reflex ;  there  is 
paralysis  of  the  bladder  and  rectum,  with  complete  relaxation  of  both 
sphincters ;  pain  is  felt  in  the  back  and  in  the  feet  or  legs ;  the  anses- 
thesia is  confined  to  the  back  and  outer  part  of  the  thigh,  and  is  great- 
est on  the  outer  part  of  the  leg  and  foot,  some  one  of  the  peculiar  areas 
of  ansesthesia  shown  in  Figs.  195  and  196  being  present.  Sensation 
in  the  testicles  is  preserved.  In  these  cases  a  recovery  leaves  the 
patient  with  some  power  of  getting  about  on  crutches,  as  apparatus 
may  hold  the  knees  and  ankles  firm,  while  the  thighs  are  under  vol- 
untary control. 

(c)  Lesions  are  sometimes  limited  to  the  conus  terminalis  or  lower 


INJURIES  OF  THE  SPINAL  CORD.    H^MATOMYELIA.       427 


three  sacral  segments.  The  most  common  lesion  is  a  hemorrhage. 
The  symptoms  produced  are  a  loss  of  control  of  the  bladder  and 
rectum,  with  complete  relaxation  of  the  sphincters,  a  total  loss  of 


Fig.  196. 


Fig.  197. 


Anaesthesia  after  a  lesion  of  the  first 
sacral  segment.     (Starr. ) 

Fig.  198. 


t  III     i„  i 


Anaesthesia  from  a  lesion  of  the  fourth  lumbar 
segment.     (Wichmann.) 

Fig.  199. 


Anaesthesia  after  lesions  of  the  second,  third,  and  fourth  sacral  segments. 
(Oppenheim,  Huber,  and  Starr.) 

sexual  power,  and  an  area  of  anaesthesia  either  limited  to  the  anal  re- 
gion, mcluding  the  posterior  part  of  the  scrotum  or  posterior  half  of 
the  labia  and  perineum,  or  extending  into  a  heart-shaped  area  over  the 
sacral  region.     (Figs.  198  and  199.) 


428       INJURIES  OF  THE  SPINAL  CORD.    HjEMATOMYELIA. 


Fig.  200. 


^ 


<?; 


Ncr^ 


DKI 


DXIT 


LIl 


V.  Fractures  of  the  lower  four  lumbar  vertebrae  or  of  the  sacrum 
cause  compression  of  the  cauda  equina,  but  no  injury  of  the  cord.  The 
Cauda  equina  is  shown  in  Fig.  200.  Compression  of  it  by  fracture 
produces  very  much  the  same  kind  of  symptoms  as  injury  of  the  spinal 
cord,  since  it  is  made  up  of  the  nerve  roots 
coming  off  from  the  lumbar  enlargement. 
When  this  mass  of  nerves  is  compressed  it  is 
found  that  the  innermost  ones  suffer  chiefly, 
and  as  these  arise  from  the  sacral  segments  the 
symptoms  of  injury  to  the  cauda  equina  are 
often  those  of  sacral  lesions,  viz.,  paralysis  of 
the  feet  and  peronei,  loss  of  control  of  the 
bladder  and  rectum,  with  relaxation  of  the 
sphincters  and  incontinence  of  urine,  and  anaes- 
thesia in  the  saddle-shaped  area  on  the  but- 
tocks, about  the  anus,  and  on  the  posterior  part 
of  the  genitals.  The  symptoms  may  not  be 
equally  distributed  in  the  two  legs.  In  one 
case  of  fracture  of  the  fourth  lumbar  vertebra 
the  symptoms  were  unilateral.  Fig.  201  shows 
the  drop-foot  present  in  this  patient,  and  Fig. 
202  shows  the  area  of  anaesthesia  present. 
No  Brown-Sequard  syndrome  can  be  caused 
by  a  partial  lesion  of  the  cauda  equina.  The 
power  of  regeneration  of  the  nerves  is  well 
known.  Hence  complete  recovery  is  possible 
after  cauda  lesions.  In  the  patient  shown  in 
Fig.  202,  operation  for  the  elevation  of  bone 
and  removal  of  pressure  resulted  in  a  complete 
cure. 

The  diagnosis  of  a  cauda  equina  lesion  from 
a  lesion  of  the  lower  part  of  the  cord  is  not 
always  possible  from  the  nervous  symptoms 
alone.  The  diagram  of  Schultze  (Fig.  200) 
shows  that  two  lesions  may  cause  the  same 
symptoms.  In  fractures  or  injuries  the  loca- 
tion of  the  lesion  is,  however,  not  so  uncertain 
as  it  is  in  hemorrhage  within  the  cord  or  in 
tumors  within  the  spinal  canal.  It  is  to  be 
remembered  that  at  the  site  of  the  fracture 
or  injury  the  nerves  are  often  lacerated  or  com- 
pressed as  they  issue  from  the  fractured  verte- 
bra. This  causes  intense  pain  or  a  zone  of 
anaesthesia.  The  location  of  this  pain  or  zone 
may  be  at  a  higher  level  than  the  anaesthesia  produced  by  com- 
pression of  the  cauda  or  cord,  and  may  often  be  a  guide  to  the 
level  of  the  lesion.  Thus  in  a  case  of  injury  to  the  back  the  pain 
was  felt  on  the  outer  side  of  the  thigh  on  one  side,  and  in  this  region 


kLIII 


LIV 


Diagram  of  the  lumbar  en- 
largement and  the  cauda  equina^ 
A,  lesion  of  the  lower  half  of  the 
enlargement,  and  B,  lesion  com- 
pressing the  Cauda  equina  pro- 
duce identical  nervous  symp- 
toms. Ncr,  anterior  crural  nerve. 
(Schultze.) 


INJURIES  OF  THE  SPINAL  CORD.    HMMATOMYELIA.       429 


the  skin  was  anaesthetic,  the  paralysis  was  limited  to  the  feet,  bladder, 
and  rectum^  and  there  was  fair  sensation  in  the  areas  belonging  to  the 
fourth  and  fifth  lumbar  segments.  In  this  case  the  diagnosis  of  a 
lesion  of  the  third  lumbar  segment  could  be  excluded  and  a  diagnosis 
made  of  compression  of  the  cauda  equina  and  laceration  of  the  third 


Fig.  201. 


Fig.  202. 


Drop-foot  after  compression  of  the  right  half  of  the 
Cauda  equina,  relieved  by  operation. 


Area  of  ansesthesia  after  compres- 
sion of  the  Cauda  equina.  (Starr  and 
Lloyd.) 


lumbar  nerve.  Symptoms  of  cauda  equina  lesions  are  less  likely  to  be 
exactly  symmetrical  in  their  distribution  than  symptoms  of  lesion  of 
the  conus  terminalis.  In  cauda  equina  lesions  severe  pain  in  the 
back,  perineum,  and  genitals  is  more  common  than  in  lesions  of  the 
cord.     Bed-sores  are  more  likely  to  develop  in  lesions  of  the  cord. 

In  the  description  of  symptoms  here  given  the  lesion  has  been  de- 
scribed as  a  transverse  one.  This  is  the  rule.  But  in  many  cases  the 
lesion  is  somewhat  irregular,  one  side  or  part  of  the  cord  suffering  more 
than  the  other.  And  in  other  cases  there  occur  perforating  hemorrhages 
in  the  gray  matter  above  the  transverse  lesion,  which  also  give  rise 
to  symptoms.  In  both  these  conditions  the  symptoms  are  not  as  sym- 
metrical as  in  a  transverse  lesion — the  paralysis  may  be  greater  in  one 
leg  or  arm  than  in  the  other,  and  the  area  of  ansesthesia  on  the  two 
sides  may  differ.  The  asymmetry  of  symptoms  is  even  more  marked 
in  spontaneous  hemorrhage  in  the  cord  without  external   injury.^     In 

*  For  a  full  history  of  the  cases  here  cited  the  reader  is  referred  to  the  American 
Journal  of  the  Medical  Sciences,  July,  1892,  and  Brain,  1894. 


430       INJURIES  OF  THE  SPINAL  CORD.    HJiMATOMYELIA. 

discussing  syringomyelia  the  fact  has  already  been  stated  that  the 
symptoms  of  that  disease  may  be  produced  by  hemorrhage  within  the 
spinal  cord;  hence  in  any  case  where  these  symptoms  develop  rapidly 
after  an  injury  spinal  hemorrhage  may  be  suspected.     (See  page  323.) 

Another  symptom  which  requires  special  mention  is  the  extreme 
lancinating  pain  felt  either  in  the  trunk  or  in  the  extremities  in  the 
domain  of  the  nerves  which  are  compressed  or  lacerated  at  the  point 
of  fracture.  These  pains  are  referred  to  the  peripheral  termination  of 
the  compressed  nerves ;  but,  like  the  pains  of  locomotor  ataxia,  are  of 
central  origin.  They  often  aid  in  the  location  of  the  lesion,  as  they 
are  referred  not  to  nerve  districts  but  to  segmental  areas. 

Diagnosis.  —  The  most  important  point  to  settle  in  any  case  of 
injury  of  the  spinal  cord  by  dislocation  or  fracture  of  the  spine  or  by 
internal  hemorrhage  is  the  question  whether  the  cord  has  been  com- 
pletely destroyed  at  any  level,  whether  the  transverse  lesion  is  total 
and  the  cord  below  it  is  cut  off  entirely  from  the  cord  above.  If  any 
sensations  whatever  are  felt  by  the  patient  in  the  parts  below  the 
lesion,  such  as  pains,  numbness,  cramps,  distention  of  the  bladder  or 
rectum,  abdominal  pain,  or  if  changes  of  position  given  to  the  limbs 
are  perceived,  or  if  any  motion  in  the  legs  is  possible  the  lesion  is  not 
total.  Even  when  there  is  total  anaesthesia  it  is  possible  in  some  cases 
to  cause  sensations  by  severe  irritation  by  needles  in  the  paralyzed 
parts,  and  these  are  felt  in  the  hypersesthetic  zone.  This  never  occurs 
if  the  lesion  is  total.  The  tendon  reflexes  return  soon,  if  lost  at  first, 
and  become  exaggerated  in  partial  lesions.  If  the  lesion  is  total  the 
patellar  tendon  reflex  is  permanently  lost  (Miles,  Bastian,  Kocher),  the 
paralysis  of  the  legs  is  flaccid,  and  they  show  no  rigidity  on  passive 
motion;  the  line  of  anaesthesia  is  more  sharply  defined  and  is  abso- 
lute ;  skin  reflexes  are  lost ;  there  is  greater  vaso-motor  paralysis  in  the 
parts  below  the  lesion ;  the  limbs  are  hot,  the  veins  distended,  priapism 
is  present  or  easily  produced  by  irritation  of  the  genitals,  groins  or 
thighs,  the  urine  is  retained,  and  the  rectum  paralyzed. 

Prognosis.  —  The  prognosis  in  cases  of  injury  of  the  spinal  cord 
depends  very  largely  upon  the  degree  and  severity  of  the  symptoms 
presented.  The  higher  the  lesion  the  worse  the  prognosis  both  as  to 
life  and  as  to  the  degree  of  recovery. 

Inasmuch  as  a  repair  of  the  spinal  cord  does  not  occur,  and  although 
scar  tissue  may  form  at  the  seat  of  the  lesion,  no  restoration  of  con- 
tinuity of  the  nerve  fibres  is  possible,  and  no  regeneration  of  nerve 
tissue  within  the  cord  has  ever  been  known  to  occur ;  the  prognosis  in 
spinal  injuries  is  a  very  bad  one.  This  fact  should  be  carefully  con- 
sidered before  any  attempt  at  surgical  interference  after  such  injuries 
is  undertaken.  When  the  examination  with  Roentgen  rays  demon- 
strates the  existence  of  pressure  it  is  a  matter  of  comparative  ease  to 
the  surgeon  to  remove  it.  Such  operations,  however,  are  uniformly 
disappointing,  for,  although  the  surgical  end  desired  may  be  attained, 
it  is  rarely  followed  by  any  improvement  in  the  condition  of  the 
patient.     Hence  the  prognosis  in  these  affections  is  extremely  unfavor- 


INJURIES  OF  THE  SPINAL  COBD.    HjEMATOMYELIA.        431 

able  under  all  circumstances,  and  the  local  diagnosis  of  the  seat  of  the 
injury  is  tl^refore  a  matter  more  of  medical  interest  than  of  practical 
importance  to  the  patient.  In  minor  injuries  where  the  spinal  cord  is 
only  slightly  disintegrated,  and  where  but  one  or  two  of  the  main  motor 
or  sensory  tracts  are  involved  in  the  injury,  some  restoration  of  func- 
tion may  spontaneously  ensue.  Hence  the  importance  of  guarding 
against  such  complicating  conditions  as  bedsores  and  cystitis  which  are 
the  immediate  cause  of  death  in  so  many  cases.  Rest  in  time  may 
result  in  the  gradual  improvement  of  the  symptoms,  and  the  patients 
may  even,  after  medium  or  slight  injury,  be  able  to  get  about  in  a 
state  of  spastic  paraplegia  of  a  more  or  less  complete  degree.  Gurlts 
has  affirmed  that  when  paralysis  of  the  bladder  and  rectum  continues 
after  eight  weeks  a  spontaneous  recovery  never  occurs,  but  to  this  state- 
ment I  do  not  agree  as  I  have  seen  recovery  of  control  after  a  year  of 
incontinence  of  urine. 

Treatment.  —  The  treatment  of  injuries  of  the  spinal  cord  consists, 
first,  of  the  treatment  of  the  surgical  condition  which  is  the  cause  of 
the  injury,  by  giving  proper  support  to  the  back,  by  reduction  of  the 
dislocation,  by  correction  of  the  fracture,  if  possible,  and  by  removal 
of  bone  which  is  causing  pressure.  The  question  of  surgical  inter- 
ference is  often  difficult  to  decide.  No  operation  is  justifiable  during 
a  condition  of  shock  such  as  usually  follows  a  dislocation  or  fracture, 
or  when  high  or  subnormal  temperature,  rapid  pulse,  or  labored  res- 
piration are  present.  When,  after  a  few  days,  the  shock  has  passed 
off,  the  general  symptoms  may  assist  the  decision.  If  there  is  evidence 
of  a  total  transverse  division  of  the  cord  an  operation  is  useless.  If 
the  cord  is  only  partially  injured  an  operation  may  do  good  when  it  is 
evident  that  the  symptoms  are  kept  up  by  a  permanent  compression. 

A  complete  anaesthesia  below  the  level  of  the  fracture  is  an  indi- 
cation that  the  cord  is  destroyed  and  that  an  operation  would  be  use- 
less. If  there  is,  however,  some  sensation  in  the  body  or  legs  below 
the  level  of  the  fracture  the  cord  cannot  be  destroyed.  The  greater 
the  degree  of  sensation  in  the  legs,  the  better  the  prospect  of  doing 
good  by  an  operation.  The  sensory  condition  is  a  better  guide  than 
the  degree  of  paralysis. 

Time  may  show  that  the  compression  is  partly  due  to  hemorrhage,  and 
as  this  is  absorbed  some  functions  may  return.  The  majority  of  sur- 
geons advise  postponing  an  operation  until  after  the  second  week,  in 
order  to  distinguish  temporary  symptoms  due  to  concussion  and  to 
shock  or  to  extradural  hemorrhage  from  the  permanent  symptoms  of 
cord  lesion.  To  put  it  off  much  longer,  however,  is  to  wait  needlessly. 
If  there  is  evidence,  by  X-ray  examination,  of  an  existing  compression 
of  the  cord  by  displaced  bone,  this  should  always  be  removed.  If, 
however,  there  is  no  such  evidence  an  operation  should  be  refused. 
The  statistics  gathered  by  Chipault  and  Hahn  support  this  view.  In 
one  hundred  and  sixty-seven  cases  Chipault  found  that  twelve  were 
cured,  twenty-four  were  improved  and  sixty-five  died.  In  sixty-four 
cases  Hahn  found  that  nineteen  were  cured  or  improved,  twelve  were 


432  INJURIES    OF    THE   SPINAL    CORD.     HMMATOMYELIA. 

slightly  helped,  and  twenty-five  died.  Kocher  agrees  with  these  sur- 
geons in  advising  strongly  against  any  operation  unless  there  is  distinct 
evidence  that  pressure  can  be  removed.  To  lay  bare  the  cord  without 
the  prospect  of  relieving  pressure  is  to  expose  the  patient  to  the  risk 
of  a  surgical  operation  which  can  have  no  result,  for  the  nervous  symp- 
toms are  due  to  actual  permanent  destruction  of  spinal-cord  tissues  inca- 
pable of  repair.  Hence,  in  the  majority  of  cases,  it  is  necessary  to 
refuse  operation,  to  keep  the  back  perfectly  quiet,  and  by  mechanical 
devices  which  prevent  motion  of  the  vertebrae,  such  as  cushions,  plaster- 
of-Paris  jacket,  braces,  extension  apparatus,  etc.,  to  assist  nature  in 
uniting  the  broken  bones  in  as  perfect  a  position  as  is  possible. 

Secondly,  the  treatment  of  the  nervous  symptoms  is  by  complete 
and  absolute  rest  in  bed  for  several  weeks  or  mouths  after  the  injury ; 
by  the  prevention,  if  possible,  of  bed-sores,  by  keeping  the  skin  in 
perfect  order  and  changing  the  position  of  the  patient  or  changing  the 
points  of  greatest  pressure  upon  the  body  frequently;  by  clean  cath- 
eterization to  prevent  cystitis,  and  careful  regulation  of  the  action  of 
the  rectum  by  enemata.  If  spasmodic  contractions  and  cramps  in  the 
muscles  of  the  limbs  at  some  distance  below  the  level  of  the  lesion  is 
an  annoying  symptom,  it  may  sometimes  be  controlled  by  the  use  of 
bromide  or  by  the  use  of  bromide  and  chloral,  or  by  phenacetin.  Pain 
at  the  level  of  the  injury,  especially  in  nerves  that  are  crushed,  will 
require  the  free  use  of  morphine,  which  may  have  to  be  continued  for 
a  long  period.  The  paralysis  must  be  treated  by  massage  and  electricity 
if  it  is  of  the  flaccid  type,  but  where  the  paralysis  is  of  the  spastic  type 
electricity  will  only  irritate  and  excite  the  muscles  to  spasmodic  cramps. 
Warm  applications,  packs,  and  massage  will  then  be  of  much  more 
service.  The  general  health  of  the  patient  is  to  be  regarded  as  well 
as  the  local  condition,  and  is  more  amenable  to  medical  treatment  by 
drugs  than  is  the  spinal  lesion. 


CHAPTER  XXIII. 

COMPRESSION  OF  THE  SPINAL  CORD. 

The  spinal  cord  may  be  compressed  by  : 

I.  Caries  of  the  vertebrae. 

II.  Carcinomatous  growths  of  the  vertebrae  or  meninges. 

III.  Syphilitic  exudations  into  the  spinal  canal. 

ly .  Tumors  within  the  spinal  canal,  either  extradural  or  intradural ; 
extraspinal  or  intraspinal. 

V.  Aneurisms  of  the  aorta  causing  absorption  of  the  vertebral 
bodies. 

YI.  Pachymeningitis  cervicalis  sen  lumbalis  hypertrophica. 

Any  one  of  these  causes,  acting  as  it  does  in  a  gradually  progressing 
manner,  and  usually  localized  at  one  level,  produces  a  slowly  increas- 
ing compression  of  the  dura,  of  the  pia,  and  of  the  bloodvessels  of 
the  cord,  and  of  the  spinal  cord  and  of  its  nerve  roots,  both  outside 
and  at  their  point  of  entrance  into  the  cord. 

I.    CARIES  OF  THE  SPINE. 

Pathology.  —  The  most  common  cause  of  compression  is  caries  of 
the  spine.  One  of  the  favorite  sites  of  tuberculous  disease  is  the  body 
of  the  vertebra,  and  caries  starting  here  may  extend  into  the  laminae 
as  well.  This  caries  may  be  and  usually  is  attended  by  the  formation 
of  pus,  which  collects  as  an  abscess  outside  of  the  spine  and  perforates, 
or  which  may  press  on  the  dura  and  spinal  cord  within  the  canal. 
The  result  of  a  tuberculous  degeneration  of  a  vertebra  is  a  gradual 
softening  of  its  bony  mass,  which  thus  removes  the  support  of  the 
body  and  allows  a  sinking  of  all  the  vertebra  above  it.  As  the 
diseased  vertebra  gives  way  a  displacement  forward  and  downward 
occurs.  The  spinous  process  is  thus  projected  backward,  producing 
the  characteristic  deformity  of  Pott's  disease,  viz.,  kyphosis.  Less 
commonly  a  lateral  deviation  occurs.  Usually  in  connection  with  such 
disease  of  the  vertebra  tuberculous  deposits  are  formed  between  the 
bone  and  the  dura  mater,  producing  a  pachymeningitis.  These  masses 
are  of  a  caseous  nature  with  much  connective-tissue  formation,  but 
occasionally  they  break  down  into  purulent  debris.  In  very  many 
cases  of  tuberculous  disease  of  the  spinal  column  the  dura  appears  to 
offer  a  protection  to  the  cord  from  any  direct  infection.  However 
great  the  formation  of  caseous  material  or  of  pus  outside  of  the  dura 
the  inner  surface  of  the  dura  remains  smooth  and  clean.  Occasionally, 
however,  this  is  not  the  case ;  the  dura  becomes  eroded  or  its  inner 
28  433 


434  COMPRESSION    OF    THE    SPINAL    CORD. 

surface  may  be  studded  with  tubercles,  or  a  caseous  mass  may  form, 
involving  both  the  arachnoid  and  pia.  Thus  the  membranes  may  be 
fused  into  a  dense  mass  which  impinges  directly  upon  the  spinal  cord. 
Pressure  upon  the  cord  primarily  affects  its  bloodvessels  and  produces 
a  condition  of  ischsemia  which  may  be  sufficiently  intense  to  go  on  to 
necrosis.  Where  the  pressure  is  not  very  severe,  this  ischsemic  con- 
dition, while  sufficient  to  cause  a  lack  of  general  nutrition  of  the  cord 
at  the  point  of  pressure,  is  not  enough  to  set  up  true  destructive  or 
degenerative  processes ;  and  while  symptoms,  such  as  increase  of  the 
reflexes  below  the  level  of  the  lesion,  some  degree  of  motor  weakness 
and  stiffness  of  the  limbs,  slight  parsesthesise,  and  possibly  severe  pain 
of  a  girdle  nature  in  the  domain  of  the  spinal  nerves  whose  roots  are 
compressed,  may  remain  for  some  time,  yet,  if  the  pressure  is  relieved 
by  healing  of  the  process  or  by  removal  of  the  mass,  they  may  subside 
and  a  restoration  of  function  in  the  cord  may  ensue.  In  some  cases, 
however,  the  pressure  produces  a  true  constriction  of  the  cord  which 
is  visible  at  the  autopsy. 

Fig.  203. 


Epidural  tuberculosis.    The  cord  is  tightly  surrounded  by  the  thickened  dura. 
Magnified  with  a  hand-glass.     (Schmaus-Sacki.) 

Microscopic  examinaticjn  of  the  cord  opposite  the  point  of  such 
pressure  does  not,  as  a  rule,  show  necrosis  rather  than  inflammation. 
There  is  a  swelling  of  the  axis  cylinders,  a  segmentation  of  the  myelin 
sheath  with  fatty  drops,  a  disappearance  of  the  nerve  fibres,  with 
secondary  hyperplasia  of  the  glia,  and  a  production  of  sclerotic 
patches.  The  cells  of  the  cord  at  this  point  are  often  atrophied  and 
found  in  various  stages  of  degeneration,  chromatolysis,  and  vacuoliza- 
tion. In  more  advanced  stages  of  the  disease  this  results  in  a  disap- 
pearance of  the  nerve  fibres  and  cells,  whose  place  is  taken  by  small 
cells,  small  nuclei,  and  thickened  gliomatous  tissue,  with  an  increase 
of  connective  tissue  about  the  blood-vessels.  Corpora  amylacea  and 
Deiters'  cells  are  often  found  in  the  mass.  In  the  terminal  stage  of 
the  process  the  spinal  cord  at  the  point  of  compression  is  reduced  to  a 
mass  of  connective  tissue,  the  nervous  elements  being  completely  de- 
generated. Occasionally  tubercles  are  found  along  the  bloodvessels 
extending  into  the  spinal  cord.  It  is  evident,  therefore,  that  the  degree 
of  the  pressure  will  determine  the  degree  of  the  degeneration.  (Fig. 
181,  page  411.) 


CARIES    OF    THE    SPINE.  435 

There  is  considerable  discussion  among  pathologists  as  to  whether  the 
origin  of  the  pathological  process  is  a  pressure  upon  the  bloodvessels, 
producing  ischsemia  and  necrosis,  or  upon  the  lymph  channels,  pro- 
ducing a  stasis  and  serous  effusion  with  oedema  of  the  cord  and  secon- 
dary degeneration,  or  whether  the  mechanical  compression  of  the  ner- 
vous elements  results  in  a  primary  degeneration  without  any  vascular 
or  lymphatic  stasis.  The  evidence  offered  by  different  authors  for  the 
existence  of  all  three  conditions  is  so  overwhelming  as  to  make  it 
apparent  that  all  three  conditions  may  occur.  We  therefore  assume 
that  the  pathological  process  in  a  compression  myelitis  varies  in  dif- 
ferent individuals.  But  in  all  the  terminal  lesion  may  be  considered 
the  same,  namely,  a  destruction  of  the  nervous  elements  and  the  pro- 
duction of  gliomatous  tissue  of  a  scar-like  nature  at  the  point  of  com- 
pression Fig.  181  (page  411)  shows  the  terminal  result  when  scar 
tissue  only  is  found. 

After  compression  of  the  cord  of  sufficient  degree  to  cause  a  disin- 
tegration of  the  tracts  passing  through  it,  secondary  degenerations  de- 
velop, and  as  the  compression  of  the  cord  increases  and  the  tracts  in 
their  entirety  are  cut  off  from  their  respective  neurones,  this  degen- 
eration becomes  well  marked.  The  location  of  these  secondary  degen- 
erations has  already  been  described  on  page  402,  with  the  pathology 
of  acute  myelitis.  The  nerve  roots  lying  adjacent  to  the  cord  or  pass- 
ing out  through  the  caseous  mass  are  degenerated. 

Symptoms.  —  The  symptoms  of  caries  of  the  spine  are,  first,  pain, 
in  the  back  in  the  bones  that  are  affected,  and  this,  pain  is  increased 
by  any  motion  or  by  such  acts  as  coughing  or  sneezing  or  by  sudden 
muscular  efforts.  Walking  rapidly  or  bringing  the  weight  of  the  body 
down  suddenly  upon  the  heels  usually  causes  pain  in  the  affected  ver- 
tebrae. Tenderness  to  pressure  and  to  heat  or  to  electricity  over  the 
diseased  vertebrae  is  an  early  symptom.  Stiffness  of  the  back  soon 
follows.  It  is  due  to  an  involuntary  spasmodic  contraction  of  the  deep 
muscles  of  the  back,  reflex  in  character,  the  object  of  which  is  to  pre- 
vent any  movement  of  the  diseased  bones  upon  one  another.  The  stiff- 
ness is  also  partly  voluntary,  the  object  being  to  prevent  any  additional 
pain  upon  movement.  Later  on,  the  development  of  deformities  of  the 
spine  and  a  marked  kyphosis  confirms  the  suspicion  of  disease  of  the 
bone.  Sometimes  purulent  collections  originating  in  the  bone  find  their 
exit  outward  through  the  skin  of  the  back  or,  burrowing  widely,  appear 
in  some  other  point  of  the  body,  as  a  retropharyngeal  abscess,  as  abscess 
of  the  mediastinum,  or  as  psoas  abscess.  As  the  bony  disease  increases 
the  patient  may  grow  shorter,  the  bodies  of  the  vertebrae  being  gradually 
absorbed. 

Pain  is  probably  the  most  valuable  and  earliest  of  all  the  symptoms 
of  caries  of  the  spine.  It  is  limited  to  the  distribution  of  the  nerves 
whose  trunks  or  roots  are  involved  in  the  pathological  process,  and  it 
is  felt  in  the  peripheral  distribution  of  these  nerves.  Thus  in  the 
common  type  of  caries  of  the  dorsal  vertebra  pain  is  felt  in  some  one 


436  COMPRESSION    OF    THE    SPINAL    CORD. 

or  two  of  the  intercostal  nerves  shooting  around  the  body  and  causing 
a  sensation  of  a  girdle,  a  feeling  as  if  a  band  were  tightly  drawn  around 
the  trunk.  If  the  caries  is  in  the  cervical  region  the  pain  is  felt  in  the 
arms  or  hands.;  if  in  the  lumbar,  in  the  thighs.  This  pain,  together 
with  the  increase  of  knee-jerks,  may  be  the  only  evidence  of  compres- 
sion of  the  cord  until  the  development  of  a  deformity  in  the  spine.  As 
the  disease  advances  the  symptoms  of  transverse  myelitis  one  by  one 
appear,  the  motor  symptoms,  however,  being  much  more  common  than 
the  sensory.  Thus  localized  atrophic  paralysis  may  develop  as  the 
result  of  pressure  on  the  nerve  roots  or  from  disease  of  the  segment. 
Rigidity  and  weakness  of  the  legs  with  increase  of  the  reflexes  develops 
early.  The  control  of  the  sphincters  may  be  impaired.  Anaesthesia 
very  rarely  develops  in  compression  myelitis  until  the  cord  is  pretty 
completely  destroyed,  though  tingling  and  numbness  and  shooting  pains 
below  the  level  of  the  compression  may  be  present  for  a  long  time 
before  the  development  of  a  true  anaesthesia.  The  skin  reflexes  are 
often  exaggerated.     Later  bed-sores  may  develop. 

The  exact  symptoms  of  compression  of  the  cord  will  depend  entirely 
upon  the  level  of  the  lesion.  It  is  not  necessary  to  rehearse  these 
symptoms,  as  they  are  fully  described  in  Chapter  lY.,  upon  the  diag- 
nosis of  spinal  lesions,  and  have  been  mentioned  concisely  in  the  chap- 
ter upon  injuries  of  the  spinal  cord  (pages  418-430). 

Course.  —  The  course  of  the  disease  in  caries  of  the  spine  is  very 
slow.  The  early  symptoms  may  develop  and  continue  for  many 
months  without  going  on  to  a  true  compression  myelitis.  In  fact,  if 
treatment  by  orthopedic  measures  is  successful  paralysis  may  never 
develop,  the  spine  may  become  fixed  in  its  deformed  position,  and  all 
nervous  symptoms  may  subside.  This  is  the  rule  in  childhood.  When 
symptoms  of  compression  of  the  cord  develop  they  may  progress  very 
slowly,  so  that  the  spastic  paraplegia  may  be  present  for  years  with- 
out incapacitating  the  patient.  Occasionally,  however,  they  come  on 
rapidly.  I  have  known  a  patient  to  be  completely  paralyzed  in  the 
legs  within  three  months  of  the  beginning  of  pain.  A  sudden  giving 
way  of  a  vertebral  body  has  been  known  to  produce  immediate  paralysis 
when  the  patient  had  been  free  from  nervous  symptoms  for  many 
months.  Even  when  spastic  paraplegia  has  developed  fully  the  symp- 
toms may  gradually  subside,  as  in  a  patient  of  Oppenheim  who  went 
on  to  recovery  after  seven  years.  This  is  a  rare  result.  As  a  rule, 
the  condition  of  spastic  paraplegia  of  greater  or  less  intensity  remains 
throughout  life,  death  occurring  from  some  complication.  The  occur- 
rence of  caries  in  childhood,  though  followed  by  a  long  life  of  freedom 
from  the  symptoms,  may  predispose  a  person  to  the  development  late 
in  life  of  a  lateral  sclerosis.  Gowers  and  Oppenheim  report  such  cases, 
and  I  also  have  seen  one.  When  caries  develops  in  middle  life  the 
probability  of  serious  spinal  symptoms  is  greater  than  in  youth,  and 
the  course  of  the  case  is  usually  more  rapid. 

Prognosis. — The  prognosis  of  compression  myelitis  due  to  caries 
will  depend  upon  the  possibility  of  removal  of  the  cause.     When  the 


CARCINOMA    AND    SARCOMA    OF    THE    VERTEBRA.  437 

carious  process  is  not  far  advanced  fixation  of  the  spine  with  extension 
is  not  infrequently  followed  by  a  gradual  recovery,  and  iu  these  cases 
it  is  not  uncommon  for  all  of  the  nervous  symptoms  to  disappear. 
Under  these  circumstances  it  must  be  supposed  that  the  pressure  was 
not  sufficient  to  produce  any  degeneration  in  the  spinal  cord,  but  the 
nervous  symptoms  were  wholly  due  to  an  anaemia  or  to  an  oedema.  In 
other  cases  where  the  disease  makes  further  progress  the  prognosis  is 
much  less  favorable.  One  has  to  think  not  only  of  the  nervous 
symptoms  but  of  the  original  disease,  its  progress,  and  of  its  likelihood 
to  go  on  to  the  formation  of  abscesses  and  to  a  destruction  of  the 
vertebrae  as  well  as  to  complete  paralysis.  The  prognosis,  therefore, 
in  these  cases  is  much  less  favorable  than  in  ordinary  chronic  myelitis, 
and  the  cases  go  on  to  a  fatal  termination  within  two  or  three  years. 

Treatment.  —  The  treatment  of  compression  myelitis  from  caries 
consists  in,  first,  the  removal  of  the  cause  of  the  condition,  and 
second,  in  alleviation  of  the  symptoms.  The  devices  of  orthopedic 
surgery  are  so  numerous  for  the  treatment  of  Pott's  disease  that  they 
cannot  be  enumerated  here,  but  the  successful  treatment  of  this  con- 
dition is  quite  possible  under  proper  apparatus  and  fixation,  and  in  the 
early  stage  it  is  easy  to  arrest  the  progress  of  the  disease,  to  remove 
the  pressure  from  the  cord,  and  thus  to  secure  recovery.  The  treat- 
ment of  the  cause  will  often  remove  many  of  the  nervous  symptoms, 
notably  the  neuritic  pain,  the  spasmodic  rigidity  and  spasms  of  the 
legs,  the  cramps  of  the  various  muscles,  and  the  parsesthesia.  Pain, 
however,  may  require  treatment  by  morphine.  Spasms  of  the  legs  may 
be  alleviated  by  bromide  or  chloral.  I  prefer  to  give  ten  grains  of 
bromide  of  sodium  every  two  hours  than  larger  doses  at  longer  inter- 
vals. If  the  apparatus  worn  will  permit  it,  hot  and  cold  douches  and 
baths  and  packs  to  the  spine,  by  increasing  the  circulation  and  nutri- 
tion in  the  spinal  cord,  are  sure  to  be  followed  by  a  certain  amount  of 
benefit.  The  care  of  the  patient's  general  condition  must  never  be 
neglected.  Fresh  air,  a  healthy  climate,  the  most  nutritious  food, 
supplemented  by  cod-liver  oil  and  all  kinds  of  general  tonics,  are  fully 
as  important  as  the  local  treatment,  and  should  always  be  prescribed  in 
addition  to  symptomatic  remedies. 

Operative  measures  in  caries  are  almost  invariably  unsuccessful,  the 
conditions  found  being  such  as  to  preclude  any  permanent  relief. 

II.    CARCINOMA  AND  SARCOMA  OF  THE  VERTEBRAE. 

Carcinoma  of  the  vertebrae  is  almost  always  secondary  to  carcinoma 
in  other  parts  of  the  body,  especially  of  the  breast.  It  develops  very 
gradually  in  persons  beyond  middle  life,  women  being  more  frequently 
affected  than  men.  Sarcoma  and  osteosarcoma  of  the  spine  may  de- 
velop at  any  age.  While  these  tumors  begin  in  the  body  of  the  verte- 
bra, they  extend  rapidly,  involving  the  laminae,  the  ligaments,  and 
adjacent  vertebrae,  and  soon  invade  the  spinal  canal.  Here  they 
cause  either  a  direct  pressure  upon  the  dura  and  spinal  cord,  or  more 


438 


COMPRESSION    OF    THE    SPINAL    CORD. 


Fig.  204. 


commonly  they  erode  the  dura,  and  both  the  membranes  and  cord  are 
directly  infiltrated  by  the  new-growth. 

Symptoms.  —  The  symptoms  are  first  those  referable  to  the  bony 
disease,  pain  in  the  spine,  tenderness  on  pressure,  stiffness  and  rigidity 
of  the  neck  or  back,  and  deformity.  The  kyphosis  is  usually  more 
diffuse  than  in  caries,  as  several  vertebrae  are  affected,  and  the  develop- 
ment of  the  symptoms  is  more  rapid.  Pain  is  an  early  symptom  in 
this  disease  and  is  most  severe  and  constant.  It  occurs  in  paroxysms 
of  intense  burning,  shooting  character,  its  situation  depending  upon 
the  level  of  the  tumor.  It  is  increased  by  motion,  by  jarring,  or  by 
pressure  on  the  spine,  so  that  these  patients  instinctively  avoid  any 
movement.  The  nerves  at  their  exit  through  the  vertebra  or  the  nerve 
roots  within  the  spinal  canal  are  directly  involved  in  the  tumor,  and 
hence  the  pain  persists  in  spite  of  treatment.  The  pain  may  be  at 
first  unilateral,  but  soon  becomes  symmetrical,  and  is  attended  by 
hyperesthesia  in  the  painful  parts  and  by  muscular  twitchiugs  and 
spasms.  Herpes  zoster  often  develops  early.  Symptoms  of  compres- 
sion of  the  cord  follow  the  pain.     A  rapidly  advancing  paraplegia  is 

the  rule,  and  as  it  is  so  uniformly 
attended  by  pain  it  has  been  termed 
"paraplegia  dolorosa."  At  the  level 
of  the  lesion  an  atrophic  paralysis  is 
produced  and  a  spastic  paraplegia  in 
the  parts  below  it.  The  level  of  the 
lesion  is  indicated  by  the  extent  of  the 
ansesthesia.  If  it  is  in  the  lower  cer- 
vical region  there  may  be  a  paralysis 
of  the  cervical  sympathetic  nerve,  as 
shown  in  Fig.  204.  A  loss  of  control 
of  bladder  and  rectum  develops  soon, 
and  the  tendency  to  bed-sores  is  very 
marked.  The  intense  pain  may  cause 
a  neurotic  or  hysterical  condition,  but 
this  should  never  mislead,  as  objective 
symptoms  are  always  present.  The 
course  of  the  case  is  usually  rapid, 
and,  as  these  cases  are  not.  open  to 
surgical  treatment,  a  fatal  termination 
always  follows.  The  duration  is  from 
three  to  six  months.  Very  rarely  a  longer  course  has  been  observed. 
In  one  case  of  cervical  carcinoma  the  symptoms  of  compression  of 
the  cord,  with  great  pain  in  the  arms,  were  present  for  three  months 
without  any  local  signs  of  the  disease.  It  was  supposed  to  be  pachy- 
meningitis cervicalis.  This  case  was  secondary  to  cancer  of  the  liver. 
In  another  case  the  carcinomatous  character  of  the  lesion  was  not 
discovered  until  after  death,  though  the  signs  of  compression  of  the 
cervical  cord  and  intense  pain  in  the  occipital  nerves  and  brachial 
plexus  had  indicated  the  probable  nature  of  the  disease,  which  was 


_>/ 


Paralysis  of  the  left  cervical  sympathetic 
nerve  from  carcinoma  of  the  spine.  Ptosis, 
retraction  of  the  eyeball,  and  flattening  of 
the  face  are  seen. 


SYPHILITIC  EXUDATION   ABOUT   VERTEBRA. 


439 


secondary  to  cancer  of  the  breast.  This  patient  for  a  month  never 
made  any  I'^ovemeut  without  holding  her  head  firmly  with  her  hands. 
In  the  third  case  the  symptoms  were  those  of  subacute  myelitis  of  the 
lumbar  region,  with  great  pain  in  both  sciatic  nerves,  but  there  was  no 
local  tenderness  of  the  spine,  or  deformity.  Here  the  autopsy  showed 
multiple  carcinomata  in  various  abdominal  viscera  and  a  small  deposit 
within  the  spinal  canal  involving  the  cord  and  membranes,  but  not 
eroding  the  bone. 

In  a  case  of  osteosarcoma  of  the  lower  lumbar  region  there  was  no 
external  evidence  of  the  disease  of  the  bone,  and  the  diagnosis  of  a 
tumor  of  the  cauda  equina  led  to  an  operation  being  undertaken.  The 
softened  bone  was  found,  also  an  erosion  of  the  dura,  and  a  mass  of 
sarcomatous  tissue  surrounding  and  compressing  the  cauda  equina. 

In  a  case  of  carcinoma  of  the  stomach  a  secondary  cancer  of  the 
mid-dorsal  region  developed  and  the  deformity  of  the  spine  became 
evident  two  mouths  before  paraplegia  occurred.  In  this  case  the  pain 
was  referred  chiefly  to  the  front  and  sides  of  the  body  until  the 
paralysis  became  complete. 

It  is  thus  evident  that  the  local  signs  of  the  disease  may  not  precede 
the  nervous  symptoms. 

Treatment.  —  The  only  treatment  for  this  condition  is  palliative, 
and  the  use  of  hypodermic  injections  of  morphine  to  give  the  patient 
relief  from  the  pain  is  imperative. 

III.  SYPHILITIC  EXUDATION  ABOUT  THE  VERTEBRA  AND  CORD. 

While  gummy  exostoses  are  not  uncommon  on  the  long  bones,  a 
syphilitic  disease  of  the  vertebrae  is  a  rare  occurrence.  It  does  occur, 
however,  and  requires  mention.  I  have  seen  it  as  a  manifestation  of 
inherited  syphilis  both  in  an  infant  and  in  a  young  man.     In  both 


Fig.  205. 


Gummy  exudation  about  the  cord  and  nerve  roots,  a,  gummy  masses  ;  6,  thickened  capsule  of  tlie 
cord;  c,  nerve  roots;  d,  pia;  e,  ascending  degeneration  in  columns  of  Goll.  (Buttersach,  Arch.  f. 
Psych.,  xvii.,  603.) 

cases  a  diffuse  thickening  of  the  tissues  about  the  spine,  in  the  one  case 
in  the  dorsal,  in  the  other  in  the  cervical  region,  occurred,  and  symp- 


440  COMPRESSION    OF    THE    SPINAL    CORD. 

toms  of  slowly  advancing  compression  of  the  spinal  cord  followed.  In 
the  latter  patient  these  advanced  to  well-developed  paraplegia,  with 
pains  and  weakness  in  the  hands  and  a  mild  degree  of  spastic  para- 
plegia in  the  legs.  In  both  cases  inunctions  of  mercury  and  large 
doses  of  iodide  of  potassium  produced  a  gradual  subsidence  of  all  the 
symptoms,  though  in  the  latter  case  a  permanent  increase  of  knee- 
jerks  and  a  slight  trace  of  spastic  gait  remains. 

A  condition  has  been  described  of  a  gummy  exudation  encasing  the 
spinal  cord  (Fig.  205),  the  exudation  being  within  the  dura  and  causing 
compression  of  the  nerve  roots  and  of  the  cord  itself.  This  was  not 
limited  to  one  level,  and  therefore  the  symptoms  produced  were  those 
of  chronic  myelitis.  It  was  due  to  inherited  syphilis.  Syphilitic 
exudations  have  been  observed  in  the  tertiary  stage  as  a  result  of  ac- 
quired syphilis.  The  symptoms  produced  were  localized  swelling  and 
signs  of  tumor  of  the  vertebrae  without  much  pain,  and  symptoms  of 
compression  of  the  spinal  cord.  The  course  was  a  slow  one,  and 
recovery  followed  treatment. 

IV.     TUMORS  AFFECTING  THE  SPINAL  CORD. 

Tumors  of  the  vertebra  that  press  upon  the  cord  are  twice  as  com- 
mon as  tumors  of  the  membranes  and  of  the  cord  combined.  Statistics 
show  that  tumors  affecting  the  cord  are  relatively  rare.  Schlesinger 
found  only  147  in  35,000  autopsies  and  151  in  6,540  tumors.  The 
ratio  to  tumors  of  the  brain  is  one  to  thirteen.  Yet  an  added  impor- 
tance to  tumors  of  the  cord  has  been  recently  given  by  their  success- 
ful removal,  and  hence  every  fact  which  aids  an  early  diagnosis  is  of 
interest. 

Table  IX.  —  Table  of  Spinal  Cord  Tumors. 


Intradural. 

Both. 

Extradural. 

Single. 

Multiple. 

Variety. 

Medullary. 

Meningeal. 

Meningeal. 

Not 
meningeal. 

Total. 

Sarcoma, 

14 

53 

9 

17 

11 

80 

27 

107 

Tubercle, 

62 

2 

55 

9 

64 

Echinococcus, 

5 

39 

8 

36 

44 

Fibroma, 

20 

"i 

5 

15 

18 

33 

Gumma, 

"i 

4 

15 

2 

19 

9 

28 

Glioma, 

20 

20 

20 

Psammoma, 

18 

18 

18 

Myxoma, 

7 

"4 

11 

11 

Lipoma, 

"i 

8 

"i 

8 

"3 

11 

Cysticercus, 

2 

5 

1 

4 

4 

8 

Gliosarcoma, 

3 

"'4 

7 

7 

Endothelioma, 

5 

i 

"4 

2 

6 

Melanosarcoma, 

i 

"3 

1 

3 

4 

Neuroma, 

4 

3 

1 

4 

Lymphangioma, 

i 

i 

1 

1 

2 

Cysts, 

1 

1 

1 

1 

2 

Cholesteatoma, 

"i 

1 

1 

Uncertain, 

13 

12 

"2 

"3 

24 

"q 

30 

125  142  35  75  13         273         127         400 

Pathology.  —  The  preceding  table  of  Schlesinger  shows  the  varieties 
of  tumor  affecting  the  spinal  cord  and  their  relative  frequency,  their 


TUMORS   AFFECTING    TEE    SPINAL    CORD. 


441 


situation,  the  fact  that  tumors  are  in 
many  cases  i^ultiple,  the  fact  that  they 
often  involve  both  membranes  and  cord 
together,  and  the  fact  that  meningeal 
tumors  are  more  common  than  medul- 
lary tumors. 

Sarcoma  about  the  cord  has  a  ten- 
dency to  extend  rapidly,  invading  the 
membranes  and  the  posterior  surface  of 
the  cord,  though  more  commonly  com- 
pressing than  destroying  the  cord.  It 
is  rarely  encapsulated,  but  often  sur- 
rounds the  cord  and  can  be  stripped 
from  it.  But  sometimes  it  is  closely 
adherent,  and  in  other  cases  infiltrates 
the  cord,  following  the  vessels  and 
compressing  and  destroying  the  nervous 
elements.  But  the  fact  that  such 
destruction  is  the  exception  rather  than 
the  rule  increases  the  chance  of  success 
in  its  removal.  Sarcoma  is,  however, 
often  multiple,  and  not  infrequently 
sarcoma  of  the  cerebellum  or  of  other 
organs  is  associated  with  sarcoma  of  the 
cord.  Sarcoma  often  starts  from  a 
nerve  root  and  invades  both  meninges 
and  cord.     (Fig.  207.) 

Tubercle  is  more  likely  to  develop 
within  the  cord  than  in  the  membranes 
when  it  occurs  as  a  solitary  tumor, 
though  a  tuberculous  meningitis  is  rela- 
tively more  frequent  than  tumor  and 
may  develop  as  a  complication  of 
it.  Tubercles  are  occasionally  found 
throughout  the  cord,  as  Raymond  has 
shown,  but  the  usual  form  of  tumor  is 
a  caseous  mass  within  the  cord.  In 
some  cases  it  appears  to  erode  the  cord, 
not  increasing  its  diameter  or  changing 
its  contour,  and  it  usually  begins  in 
the  gray  matter,  showing  that  the  in- 
fection reaches  the  cord  by  the  vessels 
rather  than  from  the  meninges.  In  a 
few  cases,  as  in  one  of  my  own,  a 
tuberculous  mass  inay  lie  upon  the 
cord  and  compress  it,  and  hence  be 
easily  removed. 

But  the  tendency  to  recurrence  is 
more  marked  in  tubercle  than  in  other 


Fig.  206. 


Sarcoma  surrounding  the  spinal  cord  and  in- 
vading the  nerve  roots.     (Schulz.) 


442 


COMPRESSION    OF    THE    SPINAL    CORD. 


forms  of  tumor,  and  the  difficulty  of  removal,  together  with  the 
danger  of  infection  of  the  meninges,  makes  an  operation  much  more 
unfavorable  in  this  form  of  tumor.  In  my  case  the  patient  developed 
a  general  tuberculous  spinal  meningitis  two  months  after  the  removal 
of  the  tumor,  and  died.  Tubercles  grow  rapidly ;  hence  the  course  of 
the  case  is  one  of  fast  progress. 

Echinococcus  and  cysticercus  cysts  are  much  more  common  in  Ger- 
many than  in  this  country,  and  hence  require  little  notice  here.    They 


Fig.  207. 


Gliosarcoma  of  the  cord.    The  cord  is  invaded  and  destroyed  on  the  left  side. 
(Specimen  furnished  by  E.  W.  Taylor  and  M.  Prince.) 

grow  rapidly,  causing  a  progressive  compression  of  the  cord,  but  do  not 
invade  it  as  a  rule.  Hence  the  symptoms  are  those  of  compression 
myelitis. 

Fibroma  springs  from  the  meninges,  usually  from  the  dura  on  its 
inner  surface.  It  lies  upon  and  compresses  the  cord,  but  does  not 
invade  it.  It  is  encapsulated  and  single.  It  grows  very  slowly,  and 
is  the  most  favorable  tumor  for  operation  because  both  diagnosis  and 
surgical  treatment  are  comparatively  simple.     (Fig.  208.) 

Gumma  usually  originates  in  the  meninges,  infiltrates  them  and  also 
the  cord,  gluing  both  together  in  a  soft  mass  and  destroying  the  cord 
tissue  completely.  It  is  diffuse,  not  encapsulated,  and  grows  very 
rapidly.  It  is  often  difficult  to  distinguish  gumma  from  tubercle  in 
the  cord  on  inspection.  It  is  a  most  unfavorable  tumor  for  operation, 
but  may  yield  to  treatment.  The  treatment,  however,  is  not  followed 
by  restoration  of  the  spinal  functions. 


TUMORS    AFFECTING    THE    SPINAL    CORD. 


443 


Glioma  of  the  cord  is  not  to  be  confounded  with  gliomatosis,  although 
it  occasionalty  complicates  this  disease.      (See  Syringomyelia,  Chapter 
XVII.)     Glioma  is  an  infiltrating  tumor  of  the  cord  tissue  not  sharply 
defined  or   encapsulated,   but  caus- 
ing a  marked  enlargement  of  one  or  Fig.  208. 
two    segments.       It    may    be    more 
extensive  and  involve  the  cord  in  a 
great  part  of  its  entire  length.     It 
does  not  involve  the  meninges.     It 
is  very  vascular,  but  rarely  contains 
cysts  as  do  gliomata   in  the  brain. 
It  grows  very  rapidly  and  is  im- 
possible of  removal.     It  is  not  in- 
frequently associated  with  sarcoma, 
but  gliosarcoma  often   involves  the 
meninges  as  well  as  the  cord  itself. 

Psammona  is  a  hard  oval  tumor 
originating  in  the  arachnoid  or  dura 
and   lying   upon    the    cord.     It   is 

Fig.  209. 


Fibroma  lying  ou  the  Bpinul  cord: 
removed  by  McCosh. 


Fibrosarcoma  of  the  lower  half  of  the  spinal  cord 
and  Cauda  equina.     (Bruns. ) 


444  COMPRESSION    OF    THE    SPINAL    CORD. 

sharply  defined  and  chalky.  It  is  not  improbable  that  the  small  chalky 
plaques  that  are  so  common  in  the  arachnoid  on  the  posterior  surface 
of  the  cord  are  the  beginning  of  psammoma,  but  they  rarely  go  on  to  a 
size  that  can  compress  the  cord.  A  lai'ge  tumor,  four  centimetres  long 
and  two  centimetres  thick,  was  found  by  Pal.  This  had  given  rise  to 
symptoms  for  six  years.  These  tumors  grow  slowly  and,  like  fibroma, 
are  easily  diagnosed  and  removed. 

Myxoma  is  usually  intradural,  sometimes  extradural,  but  never  infil- 
trates the  spinal  cord.  It  is  single,  encapsulated,  vascular,  and  may  be 
cystic,  of  slow  growth,  and  is  easily  removed. 

Lipoma  is  often  multiple,  is  intradural,  more  frequently  than  extra- 
dural, though  occasionally  masses  of  fat  are  found  about  the  back  and 
between  the  vertebrae.  It  is  a  soft,  encapsulated,  fatty  tumor  of  slow 
growth  and  easily  removed.  The  other  forms  of  tumor  given  in  the 
table  are  so  rare  as  to  be  curiosities  of  pathology.  Endothelioma  is  a 
very  small  tumor,  always  meningeal  and  often  multiple.  Melanosar- 
coma  occurs  in  the  cord  as  a  part  of  a  general  aifection  only.  Neuroma 
is  usually  a  fibroma  located  on  a  nerve  root. 

The  diagnosis  of  the  variety  of  tumor  present  in  any  case  during  life 
is  rarely  possible,  though  the  facts  already  stated  in  regard  to  the 
rapidity  of  growth  and  the  tendency  to  the  apppearance  of  tumors  in 
other  organs,  or  of  multiple  tumors  of  the  cord  may  afford  some  hint 
as  to  the  probable  nature  of  the  tumor. 

Malignant  tumors  are  ten  times  as  frequent  as  benign  tumors.  The 
situation  of  intramedullary  tumors  is  usually  in  the  cervical  or  lumbar 
enlargement  of  the  cord ;  that  of  extramedullary  or  meningeal  tumors 
is  chiefly  in  the  dorsal  region.  If  the  two  are  taken  together  it 
appears  that  no  part  of  the  cord  is  particularly  liable  to  tumor. 
Hence  the  situation  of  a  tumor  does  not  throw  much  light  upon  either 
diagnosis  or  prognosis  regarding  operation.  Tumors  are  much  more 
commonly  situated  on  the  posterior  and  lateral  surfaces  of  the  cord 
than  upon  the  anterior  surface,  hence  they  are  not  difficult  of  access  to 
the  surgeon. 

Etiolog'y .  —  Children  are  subject  to  tumors  of  the  cord  as  well  as  of 
the  brain.  Of  one  hundred  cases  thirty  occurred  under  the  age  of 
fifteen  years,  seventy  above  fifteen  years.  The  oldest  case  recorded 
was  sixty-six  years  of  age.  No  age  is  peculiarly  liable,  but  children 
are  more  liable  to  tuberculous  tumors  than  to  any  other  form.  Glioma 
is  next  in  frequency  in  childhood.  Gumma  is  a  disease  of  middle 
life.  Other  forms  are  equally  frequent  at  different  ages.  The  sexes 
are  equally  liable. 

Traumatism  precedes  the  development  of  tumors  in  so  many  cases 
as  to  be  justly  regarded  as  a  cause.     No  other  cause  is  known. 

Symptoms.  —  The  clinical  aspect  of  intramedullary  and  extramed- 
ullary tumors  is  so  similar  that  no  absolute  distinction  can  be  made  in 
any  particular  case.  The  important  thing  to  determine  is  that  a  tumor 
is  affecting  the  cord.  This  is  first  indicated  by  pain  of  an  intense 
kind,  neuralgic,  sharp,  shooting,  burning,  and  agonizing,  and  persistent 


TUMORS   AFFECTING    THE    SPINAL    CORD.  445 

in  one  locality.  It  is  not,  as  a  rule,  increased  by  pressure  on  the  nerve 
or  attended  Ky  tender  spots.  There  is  no  other  disease  which  causes 
such  definitely  localized  recurring  and  persistent  pain  as  tumor  of  the 
spinal  cord,  and  in  any  case  in  which  pain  is  continued  for  over  a 
month  in  one  particular  part  of  the  body,  there  being  no  local  disease 
to  explain  it  and  no  evidence  of  spinal  caries  or  carcinoma,  a  tumor 
may  be  suspected.  The  situation  of  the  pain  will  depend  on  the 
position  of  the  tumor,  it  being  felt  in  the  part  of  the  body  (not  in  the 
spine)  related  to  the  segment  or  nerve  root  first  affected.  Thus  in  a 
tumor  of  the  lower  cervical  region  the  pain  was  felt  for  weeks  in  the 
hand  and  forearm;  in  a  tumor  of  the  upper  cervical  region,  in  the 
shoulder  and  neck ;  in  a  tumor  of  the  sixth  dorsal  segment,  in  the 
nipple  and  chest;  in  a  tumor  of  the  tenth  dorsal  segment,  in  the 
abdomen  and  groin ;  in  a  tumor  of  the  cauda  equina,  in  the  sciatic 
nerve.  The  pain  becomes  bilateral  after  it  has  persisted  for  a  time ; 
but  this  may  not  occur  until  spinal  symptoms  develop.  Such  extension 
to  the  other  side  is,  however,  a  very  important  diagnostic  symptom. 
There  is  rarely  any  rigidity  of  the  spine,  and  bending  the  back  does 
not  increase  the  pain.  The  painful  area  is  often  hypersesthetic.  I 
have  known  two  cases  of  spinal  tumor  which  for  some  months  were 
erroneously  diagnosed  as  intercostal  neuralgia,  and  one  case  was  sup- 
posed to  be  angina  pectoris. 

The  direct  spinal  symptoms  are  those  of  compression  and  destruction 
of  the  segments  on  which  or  in  which  the  tumor  lies.  They  are 
atrophy  and  paralysis  of  the  muscles,  with  reaction  of  degeneration, 
loss  of  reflex  activity  in  their  tendons,  anaesthesia  in  the  skin,  which 
may  be  attended  by  analgesia  and  thermic  ansesthesia,  although  these 
affections  of  sensation  may  occur  independently  and  without  loss  of 
touch ;  and  herpes  zoster  and  trophic  changes  in  the  domain  of  the 
affected  nerve.  The  existence  of  dissociated  anaesthesia  is  not  always 
due  to  intraspinal  tumors,  as  it  has  been  found  in  cases  of  extradural 
tumor,  but  in  such  cases  it  is  usually  temporary. 

The  indirect  spinal  symptoms  are  those  of  spastic  paraplegia  of  the 
legs,  due  to  pressure  upon  the  long  tracts  traversing  the  segment 
affected.  They  are  rigidity  and  weakness  with  exaggerated  reflexes 
in  the  legs,  an  imperfect  control  of  bladder  and  rectum,  anaesthesia  up 
to  the  level  of  the  lesion,  and  a  tendency  to  the  development  of  bed- 
sores. They  do  not  occur  in  tumors  of  the  lumbar  region,  as  tumors 
there  cause  atrophic  flaccid  paralysis  of  the  lower  extremities. 

Tumors  of  the  cauda  equina  cause  great  pain  in  the  sacral  region  as 
well  as  in  all  the  nerves  of  the  legs ;  hence  the  pain  is  more  widespread 
in  these  tumors  than  in  tumors  of  the  cord.  The  paralysis  which 
follows  is  an  atrophic  one  and  is  attended  by  reaction  of  degeneration. 
It  may  be  limited  to  some  one  of  the  lumbar  or  sacral  nerves  and  is 
rarely  bilateral  and  symmetrical.  Paralysis  of  the  bladder  and  rectum 
are  early  symptoms.  The  facts  presented  in  the  chapters  upon  the 
diagnosis  of  local  lesions  and  myelitis  and  injuries  of  the  cord  may  be 
referred  to,  as  they  explain  more  fully  these  various  symptoms. 


446  COMPRESSION    OF    THE    SPINAL    CORD. 

It  is  to  be  remembered,  however,  that  in  no  other  form  of  disease  of 
the  cord  do  the  symptoms  of  transverse  myelitis  of  gradual  onset  coin- 
cide with  the  symptom  of  persistent  pain.  When  the  symptoms  are 
fully  developed  it  is  possible  in  some  cases  to  elicit  tenderness  on  pres- 
sure upon  the  spine  over  the  tumor,  and  the  patient  is  often  extremely 
sensitive  also  to  heat  or  to  electricity  in  the  region  of  the  tumor. 
Occasionally  the  symptoms  of  Brown -Sequard  paralysis  appear,  as  in 
a  case  of  gumma  under  my  care  which  was  gradually  absorbed  by 
treatment. 

Course.  —  The  course  is  progressive.  The  duration  varies  some- 
what according  to  the  nature  of  the  tumor.  It  is  on  an  average  about 
sixteen  months  from  onset  of  symptoms  to  death.  Death  occurs  from 
complicating  bed-sores  or  cystitis. 

Diagnosis.  —  The  diagnosis  of  a  spinal  tumor  presents  no  difficulty, 
and  should  be  made  very  early;  in  fact,  as  soon  as  spinal  symptoms 
are  associated  with  intense  persistent  pain  in  one  locality.  The  first 
question  which  arises  is  whether  the  tumor  is  single  or  multiple.  This 
can  be  determined  by  the  location  of  the  severe  pain,  for  it  will  appear 
in  several  regions  if  there  are  several  tumors,  and  by  the  development 
of  mixed  types  of  paralysis;  atrophic  rather  than  spastic  paralysis  being 
the  type  produced  by  each  tumor  in  the  muscles  related  to  the  segment 
involved. 

The  second  question  is  whether  the  tumor  is  intraspinal  or  extra- 
spinal.  The  best  summary  which  can  be  given  at  present  of  the  points 
of  differentiation  is  as  follows  :  ^  In  favor  of  extramedullary  tumors 
are  long  continued  root  pains,  long  continued  pain  and  tenderness  in 
the  vertebrae,  paralysis  of  root  distribution  rather  than  nerve  distri- 
bution, Brown-Sequard  syndrome,  and  slow  development  of  the  symp- 
toms. In  favor  of  intramedullary  tumor  are  an  irregular  course  with 
sudden  appearance  of  symptoms  due  to  hemorrhage  in  the  tumor, 
remissions,  an  absence  of  neuralgic  pains  at  the  outset,  the  develop- 
ment of  dissociated  anaesthesia  as  in  syringomyelia  and  the  absence 
of  tenderness  of  the  vertebrae.  If  the  growth  is  very  slow  in  its 
progress  it  is  most  likely  to  be  a  fibroma,  a  psammoma,  or  a  lipoma, 
and  in  any  of  these  cases  extraspinal.  All  malignant  tumors  are 
more  rapid  in  the  development  of  all  symptoms  than  are  benign 
tumors  and  are  attended  by  general  cachexia  and  constitutional 
disturbance,  which  are  absent  in  benign  tumprs.  The  fact  of  the 
previous  occurrence  of  a  tumor  elsewhere  and  of  an  operation  for 
it,  which  had  been  overlooked  by  the  patient  as  having  no  bearing 
upon  the  case,  has  several  times  been  elicited  by  me  and  thrown  light 
upon  the  nature  of  the  tumor  and  its  secondary  occurrence  in  the  spinal 
cord.  I  have  seen  three  sarcomata  in  which  this  history  was  elicited. 
It  is  evident,  then,  that  the  nature  and  the  location  of  the  tumor, 
whether  extraspinal  or  intraspinal,  is  always  uncertain. 

The  differentiation  between  a  tumor  and  caries  of  the  spine  may  be 
difficult  in  the  early  stage  of  the  latter  disease ;  but,  as  a  rule,  stiffness 
of  the  spine,  great  tenderness  over  the  spinous  process  on  pressure^  a 
iJVIalaise,  Deut,  Arch.  Klin.  Med.,  Bd  Ixxx.,  S.  143,  1908, 


TUMORS    AFFECTING    TEE    SPINAL    CORD.  447 

marked  increase  of  pain  on  motion  or  in  walking,  and  general  consti- 
tutional evidences  of  tuberculous  disease,  swollen  glands,  febrile  move- 
ment, etc.,  are  present  in  caries  and  not  in  tumor.  The  appearance  of 
kyphosis  decides  the  question.  Peptonuria  is  present  in  cases  of  tuber- 
culous abscess. 

The  differentiation  from  carcinoma  of  the  vertebra  is  more  difficult ; 
in  fact  in  many  cases  it  is  impossible  until  the  disease  of  the  vertebra 
is  palpable  and  deformity  is  evident,  though  the  previous  history  of 
the  patient  and  the  rapidly  advancing  cachexia  may  point  to  that  dis- 
ease. I  cannot  agree  with  the  statement  of  Gowers  that  the  pain  in 
carcinoma  is  greater  than  in  spinal  tumor,  but  in  carcinoma  it  is  more 
commonly  felt  in  the  spine  itself  as  well  as  in  the  nerve  distribution. 
Herpes  zoster  is  rarely  seen  except  in  tumor.  The  examination  of  the 
blood  gives  little  information,  as  leucocytosis  may  be  present  in  both 
conditions.     But  examination  by  Roentgen  rays  may  be  of  much  service. 

Treatment.  —  In  the  early  stage  of  pain  analgesics  of  all  kinds  are 
to  be  used,  but  morphine  will  eventually  have  to  be  resorted  to,  as  the 
new  analgesics  rarely  quiet  the  pain  sufficiently.  Rest  in  bed  cannot 
always  be  enforced,  as  patients  may  find  that  an  upright  position  gives 
relief  to  the  pain.  Thus  in  one  of  my  patients,  who  was  found  to  have 
fibroma  of  the  dorsal  region  on  the  posterior  surface  of  the  cord,  a 
sitting  posture  leaning  forward  over  the  back  of  another  chair  was  the 
only  one  in  which  the  pain  was  relieved.  In  the  majority  of  cases  a 
series  of  inunctions  of  mercury  combined  with  the  administration  of 
large  doses  of  iodide  of  potassium  has  been  tried.  In  cases  of  gumma 
this  is  curative,  as  I  have  seen  in  three  cases,  but  it  is  to  be  remem- 
bered that  gumma  is  rare  (twenty-eight  in  four  hundred  cases  of 
Schlesinger),  and  unless  there  is  a  previous  history  of  syphilis  no  im- 
provement should  be  looked  for.  In  two  of  my  cases  precious  time  has 
been  lost  by  such  treatment. 

An  operation  is  to  be  attempted  as  early  as  possible  after  the  diag- 
nosis is  reached.  It  should  be  undertaken  with  the  understanding  that 
it  is  exploratory,  but  it  should  be  urged  as  affording  the  only  hope. 
It  is  a  bloody  operation  and  one  which  takes  much  time  and  no  small 
amount  of  skill ;  but  it  is  not  a  dangerous  operation  if  done  aseptically, 
as  shock  is  less  frequent  than  in  cerebral  operations.  The  higher  the 
tumor  in  the  cord  the  greater  the  danger  of  cessation  of  respiration 
during  the  operation.  This  has  occurred  in  two  of  ray  operations  for 
cervical  tumors,  and  caused  much  trouble,  but  did  not  prevent  com- 
pletion of  the  operation.  The  most  important  preliminary  to  the 
operation  is  the  determination  of  the  exact  level  of  the  tumor  in  the 
spine.  It  may  be  clear  to  the  examiner  that  the  tumor  affects  a  cer- 
tain segment  of  the  spinal  cord  or  rather  that  the  symptoms  indicate 
the  upper  level  of  the  lesion.  The  level  of  the  pain  and  of  the  anaes- 
thesia are  the  most  reliable  indications.  But  it  is  to  be  remembered 
that  the  symptoms  will  he  the  same  from  a  lesion  of  a  segment  or  a 
lesion  of  its  nerve  roots  at  a  point  somewhat  lower  down  within  the 
spinal  canal.  Thus  a  tumor  pressing  on  the  roots  of  the  sixth  dorsal 
nerve  opposite  the  eighth  dorsal  segment,  where  they  issue  from  the 


448  COMPRESSION    OF    THE    SPINAL    CORD. 

cord,  will  give  the  same  symptoms  as  a  tumor  in  the  sixth  dorsal  seg- 
ment an  inch  higher.  It  is  also  to  be  remembered  that  the  relative 
position  of  the  segments  and  the  vertebra  vary,  as  already  stated,  page 
420.  Hence  in  any  operation  after  the  diagnosis  of  the  level  of  the 
segment  affected  is  reached  and  the  probable  relation  of  that  segment 
to  the  spinous  processes  is  determined  it  is  well  to  make  the  initial 
incision  a  long  one  and  to  expose  at  least  three  vertebrae,  removing 
their  spines  before  exposing  the  cord.  In  one  case  of  my  own  a  con- 
genital maldevelopment  of  one  spinous  process  led  to  the  count  of  the 
dorsal  vertebrae  being  erroneous,  and  the  cord  was  therefore  exposed 
one  inch  lower  than  was  intended.  In  the  majority  of  patients  here- 
tofore operated  upon  the  tumor  has  been  found  somewhat  higher  than 
was  expected.  It  is  safe  to  say  that  in  the  dorsal  region  it  is  four 
inches  higher  than  the  level  of  the  anaesthesia  on  the  back.  When  the 
dura  is  exposed  over  a  region  at  least  two  inches  long,  if  palpation  does 
not  reveal  the  tumor,  it  is  well  to  lay  bare  another  inch  before  opening 
it,  and  it  is  better  to  go  higher  than  lower.  The  dura  should  pulsate, 
but  does  not  do  so  near  a  tumor,  especially  below  it.  I  have  seen  the 
contrast  between  pulsation  above  and  no  pulsation  below  a  tumor  when 
the  dura  was  exposed.  In  all  cases,  even  in  those  in  which  an  extra- 
dural tumor  is  found,  it  is  well  to  open  the  dura.  In  some  cases  a 
second  tumor  lies  within.  There  is  usually  much  oedema  of  the  arach- 
noid about  a  tumor.  This  subsides  on  incision.  When  the  tumor  is 
found  it  can  sometimes  be  easily  lifted  off  the  cord.  In  other  cases  it 
must  be  dissected  away.  In  some  cases  it  must  be  shelled  out  of  the 
cord  or  even  cut  out  if  it  is  diffuse.  Division  of  the  cord  should  always 
be  in  a  longitudinal  direction,  and  it  is  better  to  cut  wedge-shaped  pieces 
out  of  the  tumor  than  to  tear  it  out.  Intraspinal  tumors  usually  recur. 
In  regard  to  the  technique  of  the  operation,  the  measures  of  asepsis,  the 
control  of  hemorrhage  by  compresses,  the  careful  division  of  dura  and 
pia,  and  ligation  of  bleeding  spinal  vessels,  the  proper  means  of  stitch- 
ing the  dura  and  closing  the  wound,  reference  must  be  made  to  surgical 
text-books.  It  is  an  important  thing  to  keep  the  patient  in  the  prone 
position  or  on  the  side  as  long  as  possible  after  the  operation  to  prevent 
undue  drainage  of  spinal  fluid.  The  loss  of  spinal  fluid  after  an  opera- 
tion may  be  considerable  without  any  permanent  ill  effect. 

Recent  records^  show  that  136  tumors  have  been  successfully  re- 
moved from  the  spinal  canal,  29  of  which  sprang  from  the  bone.  In  all 
of  these  cases  the  tumor  was  found,  although  in  several  of  them  it  lay 
at  a  level  somewhat  higher  than  that  at  which  it  had  been  originally 
located,  and  in  all  but  three  of  these  cases  it  was  possible  to  remove 
the  tumor.  Many  of  these  cases  were  successful,  as  the  patients 
recovered  not  only  from  the  operation  but  were  very  much  improved 
some  months  afterward  when  the  cases  were  reported.  In  some  of  these 
cases  operation  was  partially  successful,  the  patients  recovering  from 
the  operation  but  not  being  materially  improved  by  the  removal  of  the 
tumor.  In  all  of  these  cases  degenerations  had  occurred  in  the  spinal 
cord.  These  remained  after  the  tumor  was  taken  away.  Had  the 
iFlatau.  Deut.  Zejtspjj.  t  Nervenh.,  Oct.  15,  190B. 


ANEURISM    OF    THE    AORTA.  449 

operation  been  performed  at  an  earlier  date  in  the  course  of  the  disease 
there  is  no  d'dubt  that  these  cases,  too,  would  have  been  successful.  In 
the  remainder  of  the  cases  the  operation  failed  to  relieve,  and  the  patients 
died.  In  several  of  these  cases  the  death  was  due  to  shock,  the  opera- 
tion being  greatly  prolonged  and  hemorrhage  being  excessive.  With 
present  methods  of  technique  this  appears  to  be  avoidable.  The  chief 
danger  in  the  operation  lies  in  the  hemorrhage  from  large  veins  in  the 
muscles  and  about  the  bone.  Such  hemorrhages  cannot  be  arrested  by 
tying  the  vessels,  and  must  be  treated  by  pressure.  If,  therefore,  in 
the  course  of  the  operation  as  tissues  are  divided,  pads  held  in  place  by 
retractors  are  pressed  into  the  wound,  such  hemorrhage  may  be  to  some 
extent  avoided.  Another  cause  of  death  has  been  meningitis  of  a  septic 
nature,  and  this,  too,  may  be  avoided  by  care  in  the  preservation  of 
perfect  asepsis. 

The  terminal  result  of  an  operation  will  depend  wholly  on  the  degree 
to  which  the  cord  has  been  compressed  or  destroyed.  If  degeneration 
has  set  in  no  permanent  recovery  can  be  expected ;  but  if  the  opera- 
tion is  done  in  an  early  stage  a  great  degree  of  benefit  is  obtained  and 
a  slight  degree  of  spastic  paraplegia  only  may  remain.  In  any  case  a 
successful  operation  saves  life  and  arrests  the  progress  of  the  disease, 
even  if  the  patient  remains  a  paralytic.  In  the  majority  of  the  suc- 
cessful and  unsuccessful  operations  hitherto  reported  the  result  would 
have  been  better  had  the  operation  been  done  sooner.  A  prompt 
surgical  interference  as  soon  as  the  diagnosis  is  made  cannot  be  too 
strongly  urged.  In  case  the  tumor  cannot  be  removed  a  division  of 
the  posterior  nerve  roots  is  to  be  made  above  and  at  the  level  of  the 
tumor  for  the  relief  of  the  pain. 

Carcinoma  of  the  spinal  cord  is  inoperable.  In  sarcoma  the  chances 
are  about  even  that  the  tumor  can  be  removed.  Tubercles  can  some- 
times be  taken  away,  even  from  the  anterior  surface  of  the  cord,  as  in 
a  patient  operated  on  for  me  by  McBurney ;  but  the  danger  of  a  gen- 
eral tuberculous  affection  of  the  meninges  subsequent  to  the  operation, 
which  occurred  in  this  case,  or  of  a  recurrence,  or  of  development  of 
tubercles  elsewhere,  is  to  be  remembered.  Fibroma,  psammoma,  lipoma, 
myxoma,  osteoma,  and  cysts  are  easily  removed,  and  do  not  leave  any 
damage  to  the  cord  if  taken  away  early. 

The  treatment  of  complicating  cystitis  and  bed-sores  is  described  in 
the  chapter  on  myelitis. 

V.    ANEURISM  OF  THE  AORTA. 

An  aneurism  of  the  aorta  may  erode  the  vertebrse  and  produce  a 
gradually  increasing  pressure  upon  the  spinal  cord.  In  some  cases 
the  physical  signs  of  such  an  aneurism  are  present,  and  there  is  no 
difficulty  in  the  diagnosis.  In  other  cases,  however,  as  in  one  of  my 
patients,  the  aneurism  lying  deep  in  the  mediastinum  upon  the  dia- 
phragm cannot  be  detected  until  the  pressure  symptoms  appear.  These 
resemble  the  symptoms  of  spinal  tumor,  the  compression  of  the  nerves 
and  of  the  cord  producing  similar  pain  and  paraplegia.  There  is 
29 


450  COMPRESSION    OF    THE    SPINAL    CORD. 

sometimes,  however,  a  difficulty  of  movement,  especially  of  rotation  of 
the  spinal  column,  which  does  not  occur  in  spinal  tumors,  and  the 
symptoms  may  suggest  carcinoma  of  the  spine  or  caries  of  the  spine 
rather  than  tumor,  although  no  deformity  appears  until  the  body  of  the 
vertebra  is  eroded.  The  appearance  of  kyphosis  iu  such  a  case  is  not 
accompanied  by  any  thickening  of  the  tissues  about  the  spinous  process 
of  the  vertebra  or  about  its  laminae,  and  there  is  less  tenderness  of  the 
spine  than  in  these  diseases.  In  any  case,  however,  in  which  symp- 
toms of  compression  myelitis  appear  the  possibility  of  an  aneurism  is 
to  be  considered  and  its  physical  signs  looked  for. 

VI.    PACHYMENINGITIS  CERVICALIS  HYPERTROPHICA. 

Pathology.  —  This  disease,  first  described  by  Charcot,  presents  all 
the  symptoms  of  a  compression  myelitis  of  the  cervical  region  of  the 
spinal  cord  of  rather  rapid  progress.  The  lesion  consists  of  a  fibrous 
thickening  of  the  dura  mater  with  consequent  compression  of  the  cord 
and  especially  of  the  nerve  roots  in  their  passage  through  the  dura. 
As  a  result  of  this  thickening  of  the  meninges  there  is  usually  a 
secondary  annular  myelitis  in  the  periphery  of  the  cord  at  the  point 
of  implication ;  hence  the  affection  is  really  a  meningomyelitis  of  the 
cervical  enlargement.  In  chronic  fibrous  pachymeningitis  hemorrhages 
within  the  dura  or  in  the  newly-formed  layers  of  tissue  upon  its  inner 
surface,  such  as  occur  in  pachymeningitis  of  the  brain,  are  not  un- 
common. The  thickened  dura  not  uncommonly  becomes  adherent  to 
the  pia,  layers  of  connective  tissue  uniting  both,  and  attendant  upon 
these  changes  there  is  usually  thickening  of  the  bloodvessels.  The 
pressure  of  the  newly-formed  tissue  is  exerted  chiefly  upon  the  nerve 
roots  as  they  pass  through  the  dura,  and  these  are  found  embedded  in 
the  fibrous  mass. 

If  the  process  does  not  cease  with  the  mere  thickening  of  the  dura, 
connective  tissue  is  produced  along  the  lines  of  the  bloodvessels  into 
the  cord.  The  result  of  the  compression  of  the  spinal  cord  and  of  its 
vessels  is  a  degeneration  of  its  tissue.  There  is  also  frequently  a  for- 
mation of  gliomatous  masses  and  the  occurrence  of  secondary  de- 
generations. In  the  majority  of  the  cases  the  disease  is  of  syphilitic 
origin.  Changes  similar  to  those  described  in  compression  myelitis 
follow. 

Symptoms.  —  The  symptoms  of  this  disease  are  identical  with  the 
symptoms  of  chronic  myelitis  of  the  cervical  region  of  the  cord.  They 
differ  somewhat  in  their  distribution,  according  as  the  upper  half  or 
lower  half  of  the  cervical  enlargement  is  first  involved,  and  they  have 
been  divided  by  Charcot  into  several  stages :  first  the  stage  of  pain ; 
second,  the  stage  of  paralysis  with  contractures;  and  third,  the  stage 
of  spastic  paraplegia  secondary  to  the  compression. 

The  pain  in  the  affection  is  felt  in  the  back  of  the  neck,  possibly  iu 
the  back  of  the  head,  and  is  attended  by  great  stiffness  of  the  neck 
with  a  tendency  to  bending  forward  of  the  head  and  difficulty  on  any 


PACHYMENINGITIS    CEBVICALIS    HYPEBTBOPHICA. 


451 


lateral  motiou.  Pain  is  also  felt  in  the  hands  or  in  the  arms.  Such 
pains  are  of  an  intense  neuralgic  character,  and  are  attended  by  anaes- 
thesia and  parsesthesia  and  sometimes  by  muscular  spasms  or  tremor. 
.  After  a  period  of  two  to  five  months  symptoms  of  paralysis  appear  in 
the  muscles,  with  atrophy  and  reaction  of  degeneration.  These  may 
be  limited  to  the  ulnar  and  median  distribution  if  the  lower  half  of 
the  cervical  enlargement  is  invaded,  or  to  the  musculo-spiral  distribu- 
tion if  the  upper  half  is  involved.  Charcot  describes  certain  defor- 
mities of  the  hand  and  wrist  due  to  paralysis  (main  en  griffe),  but  these 
may  be  present  in  any  affection  producing  paralysis  of  either  the  ex- 
tensor or  flexor  muscles  upon  the  arras  and  forearms,  with  consequent 
contracture  of  their  opponents.  Thus  drop-wrist  or  claw-hand  may 
be  symptoms  appearing  in  this  disease.     If  the  lower  part  of  the  cer- 

FiG.  210. 


Ossification  of  the  vertebrae  in  spondylose  rhizomelique.     Icon,  de  la  Salpetri&re,  June,  1911. 
An  ossified  mass  welding  tlie  vertebrse  together. 

vical  enlargement  is  affected  there  may  be  paralysis  of  the  sympathetic 
nerve.  If  the  pressure  affects  the  spinal  cord  to  a  degree  sufficient  to 
cause  a  degeneration  of  the  tracts  passing  through  it,  a  spastic  para- 
plegia gradually  develops,  constituting  the  third  stage  of  the  affection. 
Such  a  condition  may  be  of  long  duration  and  slow  progress,  patients 
having  been  observed  in  whom  it  lasted  twenty  years. 

I  have  seen  several  patients  in  whom  it  seemed  probable  from  the 
symptoms  and  course  that  a  hypertrophic  pachymeningitis  of  the  lum- 
bar region  was  present.  In  one  such  case  a  thick  connective  tissue 
mass  surrounded  the  cord  over  the  entire  lower  third  from  the  sixth 
dorsal  segment  to  the  con  us.  It  was  adherent  to  the  cord  but  was 
separated  at  the  operation  without  injury  to  the  cord.  The  symptoms 
resemble  in  character  those  described  as  occurring  in  pachymeningitis 


452 


COMPSESSION   OF   THE   SPINAL   COBD. 


Fig.  211. 


of  the  cervical  region,  but  are  manifested  in  tiie  lower  extremities,  pain, 
rigidity,  and  paralysis  with  talipes  developing  slowly  and  being  of 
long  duration,  and  attended  by  extreme  atrophy  of  the  legs. 

Prognosis.  —  The  prognosis  is 
fairly  good  if  the  disease  is  diagnos- 
ticated in  its  early  stage. 

Treatment. —  The  frequent  appli- 
cation of  the  actual  cautery  to  the 
back  of  the  neck,  with  the  use  of  in- 
unctions of  mercury  and  large  doses 
of  iodide  of  potash,  is  not  infre- 
quently followed  by  a  cure.  This 
will  always  awaken  the  suspicion  that 
the  lesion  is  syphilitic  in  origin, 
though  such  suspicion  may  not  be 
confirmed  by  pathological  observa- 
tion. Applications  of  tincture  of 
iodine  to  the  neck  and  back  some- 
times give  relief.  Antineuralgic 
remedies  will  be  required,  and  for  the 
treatment  of  the  paralysis  such  meas- 
ures as  are  described  for  the  treat- 
ment of  lesions  of  the  brachial  plexus 
and  of  spastic  paraplegia  may  be  em- 
ployed. 

The  impossibility  of  stripping  off 
the  thickened  membrane  from  the 
cord  without  producing  laceration  of 
the  cord  and  excessive  hemorrhage 
renders  surgical  treatment  impossible. 

VII.  SPONDYLOSE  RHIZOMELIQUE. 
SPINAL  ANKYLOSIS. 

This  is  a  condition  of  bony  growth 
in  the  vertebrae  which  produces  a 
thickening  of  the  bodies  of  the  verte- 
brae and  their  final  union  with  each 
other ;  an  ossification  of  the  ligaments 
as  well  as  a  thickening  of  the  laminse, 
causing  an  encroachment  upon  the 
caliber  of  the  foramina,  and  conse- 
quent pressure  upon  the  nerves  which 
pass  through  them.  The  final  state 
is  shown  in  Fig.  210  which  shows  the 
vertebrae  firmly  welded  into  a  bony 
mass.  It  is  rare  for  pressure  to  be 
made  upon  the  spinal  cord,  but  this  has  been  observed. 

The  result  of  such  ankylosis  is  a  rigid  condition  of  the  back^  which 


M 

^'^^^^l 

1'/ 

fl 

m    1 

9 

IH 

^^^^^^^^^L^' 

nHH 

m_ 

i^ 

^^^^^^^^ 

Position  in  Spondylose  rhizomelique. 
Absolute  rigidity  of  the  spine  and  secondary 
atrophy  of  the  muscles.  Icon,  de  la  SalpS- 
triere,  April,  1911. 


SPONDYLOSE    BRIZOMELIQVE.  453 

is  usually  bent  somewhat  forward  as  is  shown  in  Fig.  211.  Another 
result  is  a  gradually  advancing  paralysis  and  atrophy  of  all  the  muscles 
due  to  compression  of  the  nerves ;  hence  the  thinness  of  the  limbs 
shown  in  the  figure.  The  compression  is  equally  severe  upon  the  sen- 
sory nerves,  hence  parsesthesia,  severe  pain,  and  finally  ansesthesia 
are  present  in  the  limbs. 

These  patients  are  usually  treated  for  years  for  chronic  rheumatism, 
but  it  finally  becomes  evident  that  spinal  ankylosis  is  present. 

Males  are  more  commonly  affected  than  females,  and  it  is  a  disease 
of  middle  age.^  The  onset  is  slow,  as  a  rule.  The  symptoms  are  :  (1) 
Pain  in  the  back,  in  the  sacro-lumbar  region,  or  about  the  shoulders, 
radiating  into  the  limbs  and  increased  by  movement ;  (2)  a  progressive 
stiffness  of  the  spine,  and  then  of  the  shoulders  and  hips,  so  that  the 
body  is  moved  as  a  single  mass  ;  (3)  a  deformity  resulting  in  a  bend- 
ing forward  of  the  entire  spine  in  a  regular  curve,  as  shown  in  the 
figure,  producing  a  groove  across  the  abdomen  just  above  the  umbilicus  ; 
(4)  absence  of  tenderness  on  pressure  on  the  spine ;  (5)  gradual  dim- 
inution and  final  cessation  of  pain  in  the  back  and  limbs  as  the  anky- 
losis becomes  complete  ;  (6)  absence  of  bony  deposits  on  the  long  bones 
or  in  the  small  joints,  which  serves  to  distinguish  it  from  rheumatic 
arthritis ;  (7)  progressive  atrophy  of  the  muscles  of  the  back  and 
limbs,  with  rigidity  of  the  muscles  about  the  shoulders  and  hips,  with 
occasional  fascicular  twitching,  but  no  reaction  of  degeneration ;  this 
atrophy  is  supposed  to  be  due  to  disuse  rather  than  to  degeneration  in 
the  nerves.     The  disease  is  slowly  progressive  and  incurable. 

'  For  full  discussion  see  N.  Eldaroff.     Icon,  de  la  SalpetriSre,  1911,  April  and  June. 


CHAPTER   XXIV. 

LANDRY'S  PARALYSIS.  ACUTE  PROGRESSIVE  PARALYSIS. 

This  disease,  first  described  by  Landry  in  1859,  is  an  acute  ascend- 
ing paralysis  attended  in  many  cases  by  febrile  symptoms  and  consti- 
tutional disturbances  that,  in  the  light  of  our  present  knowledge,  indi- 
cate an  acute  infection.  It  begins  in  the  form  of  paralysis  of  the  lower 
limbs  and  ascends  rapidly  to  the  body,  to  the  arms,  and  to  the  respira- 
tory muscles,  causing  death  within  a  few  days,  either  from  respiratory 
paralysis,  from  disturbance  of  the  power  of  swallowing,  or  from  heart 
failure.  It  is  very  rare  for  .the  disease  to  come  to  a  sudden  standstill 
and  for  recovery  to  ensue. 

Ever  since  the  original  description  of  Landry  observers  all  over  the 
world  have  occasionally  seen  cases  corresponding  pretty  closely  to  the 
description  given  by  Landry,  excepting  that  in  some  cases  sensory 
symptoms  of  the  nature  of  a  rapidly  advancing  anaesthesia  have  been 
present  associated  with  the  paralysis.  More  careful  observation  of 
temperature  than  was  customary  in  Landry's  time  has  demonstrated 
the  fact  that  in  the  large  majority  of  cases  a  febrile  movement  accom- 
panies the  disease.  Such  febrile  movement  was  present  in  a  case  under 
my  own  observation.  In  this  case  the  general  appearance  of  the 
patient,  the  enlargement  of  liver  and  spleen,  a  condition  of  jaundice, 
vomiting,  diarrhcea,  and  acute  nephritis  all  suggested  strongly  a  con- 
dition of  infection.  The  paralysis  became  complete  in  the  course  of  a 
week,  when  the  patient  died. 

Westphal  described  a  case  quite  similar  in  its  rapid  course  to  that  of 
Landry's  original  case,  but  in  it  the  disease  was  a  descending  rather 
than  an  ascending  one  in  its  progress.  Hence  the  ascending  character 
of  the  affection  as  described  by  Landry  must  not  be  considered  essential 
to  the  diagnosis.  In  a  few  cases  the  escape  of  the  bladder  and  rectum 
has  not  been  complete,  and  incontinence  of  urine  and  feces  has  occurred. 
In  a  few  cases,  where  the  disease  has  lasted  beyond  a  week,  reaction 
of  degeneration  has  developed  in  the  muscles ;  hence  the  various  diag- 
nostic signs  of  the  disease  as  laid  down  by  Landry  have  gradually 
been  eliminated,  and  we  have  to  speak  of  Landry's  paralysis  as  in- 
cluded under  acute  progressive  total  paralysis. 

In  the  majority  of  the  cases  recently  recorded  the  distinctly  infectious 
appearance  of  the  patient,  as  already  mentioned  in  toy  case,  has  been 
prominently  noticed,  and  it  seems  at  present  to  be  the  general  consensus 
of  opinion  that  in  Landry's  paralysis  we  have  an  acute  infectious  dis- 
ease which  produces  rapidly  advancing  paralysis.  It  may  be  that  these 
cases  have  been  cases  of  poliomyelitis  of  a  very  severe  type. 

Pathology.  —  The  lesions  found  in  various  cases  of  Landry's  par- 

454 


SYMPTOMS.  455 

alysis  have  differed  widely.  The  early  observers  found  no  lesions,  but 
their  methoifs  of  observation  were  very  imperfect,  and  hence  their  con- 
clusions cannot  be  accepted,  esj)ecially  as  later  investigators  have  almost 
uniformly  found  some  changes  in  the  nervous  system.  Many  pathol- 
ogists have  found  changes  in  the  spinal  cord  consisting  practically  of 
a  widely  disseminated  acute  myelitis,  with  vascular  and  perivascular 
inflammatory  conditions,  with  acute  degenerative  processes,  and  with 
all  the  characteristic  appearances  seen  in  cases  of  very  acute  myelitis.^ 
Other  equally  reliable  observers  have  considered  Landry's  paralysis  a 
multiple  neuritis  and  have  described  numerous  changes  in  all  of  the 
peripheral  nerves  and  in  the  nerve  roots.^  In  these  cases  no  changes 
in  the  spinal  cord  have  been  found.  Unfortunately,  as  yet  bacterial 
examination  of  this  disease  has  failed  to  reveal  the  existence  of  any 
special  germ,  though  in  recent  cases  various  micro-organisms  have  been 
discovered  :  typhoid  bacilli,  pneumococcus,  staphylococcus,  diplococcus, 
etc.^  The  probable  theory,  however,  is  that  it  is  a  disease  of  acute 
infectious  origin,  the  germ  or  toxin  of  which  affects  the  entire  nervous 
system  both  in  its  periphery  and  in  its  central  organs.  In  some  cases 
the  lesion  is  found  in  the  nerves ;  in  others  in  the  neurone  bodies  in 
the  spinal  cord ;  in  others,  still,  in  the  pons  and  cerebral  axis,  the 
specific  action  being  greater  on  the  motor  than  on  the  sensory  portion 
of  the  nervous  system.  The  cortical  neurones  escape.  This  hypothesis 
would  give  an  explanation  for  the  rare  cases  of  acute  bulbar  palsy  or 
acute  ophthalmoplegia  followed  by  descending  paralysis,  the  majority 
of  which  go  on  to  a  fatal  termination  in  a  very  short  space  of  time. 
The  causation  of  the  disease  is  more  likely  to  be  a  specific  infection, 
inasmuch  as  autopsies  in  later  cases  have  demonstrated  changes  in  the 
viscera,  the  liver,  the  spleen,  the  kidneys,  and  the  lymph  glands  such 
as  are  commonly  found  in  other  acute  infectious  diseases.*  Its  causa- 
tion also  finds  a  certain  amount  of  support  in  the  fact  that  a  number 
of  cases  of  Landry's  paralysis  have  developed  in  the  course  of  or  sub- 
sequently to  other  acute  infectious  diseases. 

Symptoms.  —  The  disease  begins  suddenly  and  affects,  first,  the 
muscles  of  the  lower  extremities,  producing  a  rapidly  increasing  flaccid 
paralysis  with  total  disability  to  move  the  muscles  voluntarily.  There 
is  no  tremor,  no  contracture,  no  spasm  or  cramp  in  the  muscles,  and 
percussion  of  the  muscles  does  not  produce  any  muscular  contraction. 
There  is  a  loss  of  tendon  reflexes.  The  electrical  reactions  are  not 
changed,  but  this  is  probably  because  the  duration  of  the  paralysis  is 
not  sufficiently  long  to  warrant  the  appearance  of  a  reaction  of  degen- 
eration. There  may  be  numbness  in  the  legs,  but  there  is  rarely  pain. 
The  paralysis  advances  rapidly  from  the  legs  to  the  thighs,  then  to  the 
muscles  of  the  trunk,  so  that  the  patient  can  no  longer  retract  the 
abdomen  or  sit  up  in  bed  ;  it  then  advances  to  the  muscles  of  the  back 
and  of  the  thorax  and  of  the  arms,  until  finally  all  the  muscular  system 

^  Bailey  and  Ewing,  New  York  Medical  Journal,  July  4,  1896. 

"Barth.     1890. 

^  See  Brain.     1908.     Spring  number. 

*T.  Diller,  Journal  of  Nervous  and  Mental  Disease,  October,  1902. 


456     LANDRY'S    PARALYSIS.     ACUTE    PROGRESSIVE    PARALYSIS. 

up  to  the  neck  is  paralyzed.  It  then  advances  to  the  muscles  of  deglu- 
tition and  of  speech  and  to  the  face,  so  that  an  absolutely  total  par- 
alysis of  the  entire  muscular  system  of  the  body,  including  that  of  the 
eyes,  may  occur  before  death.  When  the  arms  are  affected  the  par- 
alysis begins  in  the  fingers  and  creeps  up  the  arm  as  it  did  up  the  leg, 
and  here,  too,  there  is  no  tendency  to  contracture  or  deformity.  The 
limbs  are  wholly  given  over  to  the  action  of  gravitation,  muscular  tone 
being  abolished.  The  bladder  and  rectum  usually  remain  in  a  normal 
condition,  though  in  a  few  cases  they  have  been  paralyzed.  Sensory 
disturbances  in  some  cases  do  not  appear  at  all.  In  other  cases  the 
general  sense  of  muscular  fatigue  and  very  intense  feelings  of  numb- 
ness and  formication  may  be  felt,  but  examination  usually  fails  to 
reveal  any  marked  anaesthesia.  The  patient's  consciousness  remains 
clear  to  the  very  end  in  the  majority  of  cases.  According  to  Landry's 
account  of  the  disease,  it  may  run  its  course  in  some  cases  within  three 
days,  in  others  it  may  be  longer,  up  to  two  weeks,  and  a  large  majority 
of  patients  die.  In  a  few  cases  the  disease  comes  to  a  standstill ; 
remissions  occur,  which  may  be  followed  by  an  exacerbation  and  death, 
or  by  a  slow  recovery. 

The  prognosis  is  very  grave,  and  the  treatment  can  only  be  of  a' 
general  character,  such  as  would  be  used  in  any  acute  infection  or  in 
acute  myelitis.  Ergot  has  been  recommended  in  large  doses,  but  this 
seems  to  be  an  empirical  suggestion  with  no  rational  basis.  Antiseptic 
treatment  by  hypodermic  injections  of  carbolic  acid  or  of  formalin 
injections  into  the  veins  might  be  tried.  Urotropin  10  grains  every 
three  hours  by  the  mouth  is  to  be  used. 


CHAPTER  XXY. 

SPINAL  MENINGITIS. 

Etiology.  —  Acute  meningitis  limited  to  the  spinal  cord  develops 
after  various  forms  of  disease  of  the  spinal  column  or  of  the  dura  mater, 
especially  after  tuberculous  and  malignant  disease.  It  may  occur  as 
the  result  of  any  form  of  injury  of  the  spinal  column  or  of  the  spinal 
cord,  especially  if  that  injury  involves  a  laceration  of  the  surface  and 
opens  the  way  for  the  ingress  of  germs.  Acute  spinal  meningitis  may 
also  develop  as  a  complication  of  acute  myelitis  and  myelomalacia  from 
any  cause.  It  may  also  arise  as  a  complication  of  bed-sores,  the  septic 
agent  having  direct  access  to  the  meninges,  either  through  the  vessels 
or  along  the  nerve  trunks.  It  may  develop  also  by  extension  of  a 
meningitis  of  the  base  of  the  brain,  especially  after  otitic  meningitis. 
The  most  common  cause  of  acute  meningitis  is  the  presence  of  some 
infectious  agent  in  the  blood  as  the  result  of  pneumonia,  typhoid  fever, 
rheumatism,  furunculosis,  tuberculous  disease  anywhere,  or  any  of  the 
infectious  diseases,  or  septicaemia.  Exposure  to  cold  is  mentioned  as  a 
possible  cause,  though  this  is  questionable. 

Epidemic  cerebro-spinal  meningitis  is  described  in  Chapter 
XLI. 

Pathology. — On  opening  the  spinal  canal  an  accumulation  of  fluid 
is  found  between  the  dura  and  pia,  infiltrating  the  meshes  of  the 
arachnoid,  and  this  fluid  may  be  purulent.  Here  and  there  on  the 
surface  of  the  cord  are  collections  of  pus  or  of  thick  fibrin  and  plastic 
material  and  gelatinous  masses.  Fibrinous  exudations  upon  the  sur- 
face of  the  cord  in  the  pia  are  evident  at  places.  Thus  the  usual 
proofs  of  an  inflammation  of  a  serous  membrane,  serum,  fibrin,  and 
pus  are  found  irregularly  scattered,  or  filling  up  all  the  meshes  of  the 
arachnoid  and  covering  the  pia.  The  bloodvessels  are  distended  with 
blood,  and  small  hemorrhages  may  be  found.  There  is  a  close  adhesion 
of  the  pia  to  the  cord,  and  frequently  also  to  the  dura.  Meningitis 
may  extend  to  the  nerve  roots  at  their  points  of  exit  through  the  mem- 
branes. On  microscopic  examination  an  extensive  congestion  of  the 
finer  bloodvessels  of  the  meninges  and  of  the  arteries  entering  the 
periphery  of  the  cord  is  evident,  and  there  is  an  exudation  of  cells 
about  the  bloodvessels  both  in  the  pia  and  in  the  periphery  of  the  cord. 
Capillary  hemorrhages  are  frequently  observed  in  the  pia  and  within 
the  cord,  together  with  masses  of  fibrin  and  collections  of  pus.  CEde- 
mat<^)us  swelling  of  the  various  layers  of  the  pia  and  the  production 
of  connective  tissue,  with  a  marked  thickening  of  the  pia  layers,  are 
generally  present.     Changes  are  uniformly  observed  on  the  nerve  roots 

457 


458  SPnKlL   MENTNGirm. 

and  also  in  the  spinal  cord.  The  nerve  roots  show  swelling  of  the 
axis  cylinder  and  destruction  of  the  myelin  sheath,  with  all  forms  of 
degeneration  in  the  nerves.  There  is  also  a  congestion  of  the  vessels 
of  the  perineurium  and  endoneurium  and  an  exudation  of  leucocytes 
and  small  cells.  The  spinal  cord  is  usually  affected  to  the  depth  of 
one-half  centimetre  from  the  surface  on  all  sides.  Fine,  small-cell 
infiltration  is  evident  along  the  bloodvessels,  and  many  leucocytes  are 
found  within  the  cord.  The  vessels  are  distended  and  occasionally 
ruptured.  Swelling  of  the  axis  cylinders  of  the  white  columns,  seg- 
mentation of  the  myelin,  and  general  degenerative  processes  in  the 
nerve  fibres  of  the  cord  are  present,  and  these  appear  to  be  more 
intense  near  to  the  bloodvessels,  and  sometimes  can  be  found  at  a  con- 

FiG.  212. 


f  >■ 


Acute  meningomyelitis.  Section  through  the  anterior  border  of  the  spinal  cord  and  meninges, 
showing  infiltration  of  the  tissues  by  inflammatory  products.  (Marinesco,  International  Medical  Con- 
gress, Paris,  1900. ) 

siderable  depth  from  the  surface  of  the  cord,  especially  along  the  larger 
arteries.  Thus  wedge-shaped  regions  of  degeneration,  apex  inward, 
may  be  discovered  in  the  cord.  These  changes  are  usually  more  in- 
tense in  the  posterior  and  lateral  columns  than  in  the  anterior  columns. 
There  is  usually  a  tendency  to  an  increase  in  the  glia  and  to  a  connec- 
tive-tissue production,  and  if  the  condition  goes  on  to  a  chronic  stage 
small  plaques  of  connective  tissue  and  sclerosis  may  be  produced. 
Similar  changes  are  observed  in  the  medulla  oblongata  if  the  disease 
extends  upward  to  the  brain.  If  the  disease  does  not  prove  fatal  there 
may  be  left,  as  a  result  of  an  acute  meningitis,  a  chronic  permanent 
thickening  of  the  pia  mater  with  adhesions  to  the  dura  mater  and  with 
sclerosis  of  the  spinal  cord  and  chronic  degenerative  processes  in  the 
nerve  roots.  In  the  case  of  the  anterior  nerve  roots  these  may  extend 
outward  to  the  peripheral  nerves. 


SYMPfOMS.  459 

Spiller^  has  described  a  form  of  meningitis  which  he  terms  circum- 
scribed serous  meningitis,  the  lesion  consisting  of  a  collection  of  clear 
fluid  in  the  pia-arachnoid,  forming  a  cyst  which  presses  upon  the  cord, 
causing  symptoms  like  those  of  a  tumor.  The  origin  of  these  cysts 
is  unknown. 

Symptoms.  —  Acute  meningitis  usually  develops  suddenly  with 
,fever,  chill,  and  marked  constitutional  disturbances,  with  all  the  phe- 
nomena of  a  febrile  movement — malaise,  nausea,  and  diarrhoea  —  and 
in  addition,  with  local  symptoms  referable  to  the  spinal  cord  and 
especially  to  irritation  of  its  surface  and  nerve  roots.  These  symp- 
toms consist  of  severe  pain  in  the  back  and  radiating  pains  about  the 
body  and  down  the  limbs,  which  are  greatly  increased  by  movement 
or  by  pressure  or  by  any  irritation  of  the  surface.  They  are  soon  fol- 
lowed by  a  condition  of  stiffness  of  the  back  and  of  the  neck,  so  that 
the  entire  body  is  held  rigid,  and  all  attempts  at  movement  are  resisted 
because  of  the  increased  pain  thereby  produced.  An  actual  rigidity  of 
the  muscles  of  the  back  of  the  neck  can  be  felt,  and  this  may  increase 
to  the  point  of  opisthotonos,  especially  in  children.  The  pains  of 
meningitis  are  of  a  neuralgic  character  and  are  very  constant  and  fre- 
quent. They  shoot  from  the  back  around  to  the  front  of  the  body, 
and  they  shoot  up  and  down  the  arms  and  legs.  They  are  attended 
by  hypersesthesia  and  hyperalgesia  of  the  skin,  so  that  all  touch  or 
handling  of  the  surface  is  unendurable,  and  the  patients  very  often  are 
unable  to  bear  the  weight  of  the  bed-clothing,  which  has  to  be  sup- 
ported by  a  basket  arrangement  in  the  bed.  This  hypersensitiveness 
is  often  attended  by  spasms  of  the  muscles  and  twitchings  of  the 
limbs,  and  these  cramps  are  often  very  painful.  Kernig  has  noticed 
that  if  these  patients  are  made  to  sit  up  in  bed  the  legs  are  always 
drawn  up,  with  flexion  of  the  knee.  The  leg  cannot  be  extended  on 
account  of  the  contracture  of  the  flexor  muscles.  In  the  course  of  a 
few  days,  as  the  result  of  the  inflammation  and  degenerative  changes 
in  the  nerve  roots,  anaesthesia  may  develop  in  the  parts  that  have  been 
painful,  and  in  some  cases  paralysis  with  atrophy  develops  in  the 
muscles.  This  is  usually  noticed  first  in  the  legs,  and  is  subsequently 
attended  by  an  increase  of  the  knee-jerks  and  the  development  of 
ankle  clonus.  Later  on,  if  the  paralysis  becomes  extreme,  the  reflexes 
may  be  lost.  Within  a  few  days  of  the  onset  disturbance  in  the  action 
of  the  bladder  develops,  there  being,  frequently,  retention  of  urine, 
requiring  the  use  of  a  catheter,  and  obstinate  constipation ;  later  on  in- 
continence may  ensue.  A  tendency  to  the  development  of  bed-sores 
is  observed  in  all  cases,  and  the  skin  must  be  cared  for  by  constant 
change  of  the  position  and  by  proper  support  upon  an  air-bed  or 
water-bed.  Vasomotor  disturbances  are  common  in  the  shape  of 
flushings  or  sudden  pallors  of  the  skin,  and  at  any  point  upon  the 
body  a  red  line  will  appear  after  the  skin  is  irritated  by  a  sharp  object, 
such  as  a  pencil  or  nail  (tdches  oer^brales  ou  spinaled). 

The  course  of  the  case  is  often  a  rapid  one,  and  within  a  week  im- 
lAmer.  Jour.  Med.  Sci.,  Jan.,  1909. 


460  SPINAL    MENINGITIS. 

plication  of  the  muscles  of  respiration  or  an  affection  of  the  pneu- 
mogastric  nerve  produces  death  from  respiratory  or  heart  failure. 
It  may  be  preceded  by  very  rapid,  irregular  respirations,  by  Cheyne- 
Stokes  respiration,  or  by  intermittent,  irregular  pulse.  In  the  majority 
of  the  cases  the  meningitis  extends  to  the  cranial  cavity,  and  cerebral 
symptoms,  convulsions,  and  cranial  nerve  palsies  develop  just  before 
death.  In  other  cases  the  progress  of  the  case  is  less  rapid,  and  two 
or  three  weeks  pass  before  the  symptoms  become  severe  enough  to  give 
rise  to  alarm,  and  the  patient  may,  even  after  the  most  severe  symp- 
toms, gradually  recover,  though  frequently  a  state  of  chronic  meningitis 
is  left  in  which  paralyses,  muscular  atrophies,  irregular  plaques  of  anses- 
thesia,  and  disturbance  in  the  action  of  the  bladder  and  rectum  remain 
for  many  months. 

In  the  circumscribed  serous  meningitis  of  Spiller  the  symptoms 
were  those  of  a  spinal  tumor,  pain  and  paraplegia  slowly  developing 
—  and  these  cases  were  successfully  treated  by  operation  for  the  sup- 
posed tumor  ;  the  cyst  being  emptied. 

Diagnosis.  —  The  diagnosis  of  acute  meningitis  is  much  aided  by 
Quincke's  lumbar  puncture  (see  page  155). 

In  cases  of  meningitis  the  fluid  contains  leucocytes,  and  bacteria  of 
various  kinds  have  been  found  in  the  serum  —  tubercular  bacilli  in 
tuberculous  patients ;  the  streptococcus,  pneumococcus  or  diplococcus 
in  other  cases.  The  mere  withdrawal  of  the  fluid  where  the  intra- 
spinal pressure  is  great  is  followed  by  great  relief  of  the  symptoms, 
notably  the  pains  and  spasms  of  the  back,  and  Quincke's  puncture 
may  be  done  not  merely  as  a  diagnostic  but  also  as  a  therapeutic 
measure. 

Prognosis.  —  The  prognosis  in  acute  meningitis  will  depend  some- 
what upon  the  cause,  and  is  more  unfavorable  in  tuberculous  cases  than 
in  purely  septic  cases.  The  meningitis  following  pneumonia  and  typhoid 
fever  usually  recovers. 

Treatment.  —  The  treatment  of  meningitis  consists  in  absolute  rest 
in  bed.  An  air-bed  or  water-bed  should  be  employed  early  on  account 
of  the  tendency  to  bed-sores.  It  is  preferable  for  patients  to  lie  upon 
the  sides  rather  than  upon  the  back.  Cold-water  applications,  or  ice- 
bags,  should  be  applied  to  the  spine.  Blisters  or  the  actual  cautery 
may  be  applied  along  the  back.  Cupping  also  may  be  used,  either  wet 
or  dry,  according  to  the  general  condition  of  the  individual.  The 
general  constitutional  disturbances  must  be  treated  by  laxatives  and  by 
antipyretics.  The  pains  require  sedatives,  either  bromides  in  large 
doses  or  bromide  and  chloral  together,  recourse  being  had  to  opium  if 
the  analgesics,  phenacetin,  etc.,  fail. 

CHRONIC  MENINGITIS. 

Pathology.  —  A  few  cases  of  chronic  meningitis  begin  in  an  acute 
process,  but  the  majority  develop  as  a  chronic  syphilitic  inflammation 
from  the  start.     In  chronic  meningitis  there  is  no  production  of  pus 


CHRONIC  MENINGITIS.  461 

and  very  little  fibrin,  but  simply  a  connective-tissue  formation  with  or 
without  an  increase  of  serum.  In  all  cases  the  result  is  a  congestion 
and  distention  of  the  bloodvessels,  a  leucocytosis  and  round-cell  infil- 
tration of  the  membranes  of  the  cord  and  of  the  nerve  trunks,  and  a 
thickening  of  all  the  tissues  involved.  A  chronic  degenerative  process 
is  usually  set  up  in  the  spinal  cord,  especially  in  the  periphery  of  the 
cord,  giving  rise  to  a  chronic  meningomyelitis.  In  fact,  the  majority 
of  cases  of  chronic  meningitis  are  associated  with  chronic  myelitis  of 
low  grade  and  slow  course. 

In  chronic  meningitis  of  syphilitic  origin  gummy  exudations  or 
gelatinous  masses  are  found  all  through  the  meshes  of  the  pia  mater 
and  arachnoid,  and  very  often  surround  the  nerve  roots.  Thus  in  the 
case  reported  by  Buttersack  (Fig.  205)  the  gelatinous  mass  surrounded 
both  the  cord  and  the  nerve  roots  and  was  attended  by  a  round-cell 
infiltration  of  the  cord  itself.  In  syphilitic  meningitis  the  blood- 
vessels are  thickened,  both  veins,  arteries,  and  capillaries,  and  the 
lymphatics  show  deposits  in  their  walls  and  an  infiltration  with  small 
cells.  The  changes  in  the  bloodvessels  accompanying  a  syphilitic 
meningitis  are  those  found  in  all  forms  of  obliterating  endarteritis, 
and  very  frequently  are  attended  by  obliteration  of  the  lumen  of  the 
vessels.  In  -the  syphilitic  forms  of  chronic  meningitis  wedge-shaped 
plaques  of  connective  tissue  are  found  in  the  spinal  cord,  irregular 
regions  of  sclerosis,  and  a  round-cell  infiltration  all  through  the  white 
matter  of  the  cord,  which  occasionally  penetrates  as  deeply  as  the 
gray  matter.  In  the  syphilitic  forms  of  meningitis  these  lesions,  both 
of  the  meninges  and  the  cord,  may  be  continuous  over  a  considerable 
distance,  or  they  may  be  disseminated  irregularly  through  either 
enlargement  or  through  the  entire  cord. 

Symptoms.  — The  course  of  a  case  of  chronic  meningitis  differs  from 
that  of  acute  meningitis  only  in  the  absence  of  febrile  onset  and  in 
the  slower  development  of  the  various  symptoms.  The  same  stiffness 
in  the  back  and  limbs,  tendency  to  cramps,  shooting  pains,  hyper- 
sesthesia,  paralyses,  anaesthesia,  and  disturbance  in  the  action  of  the 
bladder  and  rectum,  develop  slowly,  together,  or  in  succession. 

Diagnosis.  —  The  diagnosis  of  the  condition  is  usually  very  easy 
from  the  symptoms  that  are  present.  Ordinary  pains  in  the  back, 
even  when  attended  by  rigidity  and  by  some  neuralgic  pain  and  oc- 
casional cramps,  are  not  sufficient  to  establish  a  diagnosis,  as  they  may 
be  due  to  neurasthenia,  may  appear  in  traumatic  neurosis,  or  may 
follow  slight  falls  and  injuries  of  the  back.  Under  these  circum- 
stances, however,  the  intensity  of  the  symptoms  is  much  less  than  in 
meningitis,  and  in  case  of  doubt  Quincke's  puncture  will  fail  to  reveal 
the  existence  of  an  increase  of  cerebro-spinal  fluid.  Muscular  rheuma- 
tism may  produce  a  severe  pain  in  the  back  with  more  or  less  tender- 
ness in  the  muscles  and  in  the  spine,  but  never  goes  on  to  develop  the 
other  symptoms  of  meningitis  already  mentioned.  Myelitis,  though 
frequently  attended  by  a  complicating  meningitis,  is  not,  as  a  rule, 
attended  by  pain  of  as  intense  a  character;,  and  there  are  few  neuralgic 


462  SPINAL    MEXIXaiTIS. 

pains  and   no   tendency  to  a   si^asm  or  cramps  in  the  muscles  or  to 
rigidity  of  the  back. 

Treatment.  —  In  the  chronic  cases  a  thorough  course  of  inunctions 
of  mercury,  followed  by  iodide  of  potash  in  full  doses,  from  twenty  to 
one  hundred  grains,  three  times  a  day,  will  be  of  service  not  only  in 
the  syphilitic  but  also  in  other  cases.  Cautery  applied  to  the  spine 
will  give  relief  to  many  of  the  pains  and  to  the  stiffness.  Massage  and 
long-continued  warm  baths,  or  spinal  douches,  or  sponging  of  the  spine 
alternately  with  hot  and  cold  water,  are  also  of  service.  The  general 
strength  of  the  patient  should  be  supported,  and  all  forms  of  good  food 
and  tonics  should  be  used  freely. 


SECTION  III. 
DISEASES  OF  THE  BEAIN. 

CHAPTER   XXVL 

THE   CEREBRAL   CIRCULATION. 

The  majority  of  cases  of  brain  disease  which  are  met  with  in  prac- 
tice are  due  primarily  to  disease  of  the  bloodvessels.  Endarteritis 
produces  a  state  of  malnutrition  in  the  brain  which  may  show  itself  by 
mental  failure  or  by  premature  senility  or  even  by  signs  of  dementia. 
It  also  causes  thrombosis  and  embolism,  and,  by  the  final  weakening  of 
the  vessel  wall,  rupture  and  hemorrhage.  In  the  former  case  necrosis 
of  the  brain  tissue  is  produced,  with  arrest  of  its  functions.  In  the 
latter  case  laceration  and  destruction  of  the  brain  tissue  is  caused  with 
both  irritation  and  arrest  of  function.  Hence  a  knowledge  of  the  dis- 
tribution of  the  cerebral  bloodvessels  is  a  necessary  preliminary  to  a 
study  of  the  various  types  of  apoplexy. 

The  blood  reaches  the  brain  through  the  vertebral  and  internal 
carotid  arteries.  The  vertebral  arteries  unite  to  form  the  basilar, 
which  in  its  course  gives  off  the  arteries  of  the  cerebellum  and  pons. 
The  basilar  divides  into  the  posterior  cerebral,  which  supply  the  thal- 
amus, the  tegmentum  of  the  crura,  the  corpora  quadrigemina  and  crura. 

Fig.  213. 


Int.  Car. 

Mid.  C. 

Showing  distribution  of  bloodvessels  to  internal  capsule.  The  artery  marked  x  is  the  so-called 
artery  of  cerebral  hemorrhage,  and  it  is  readily  seen  that  its  rupture  destroys  the  fibres  in  the  internal 
capsule.     (Duret.) 

and  tlie  temporo-occipital  basilar  part  of  the  hemispheres.     The  pos- 
terior cerebral  send  the  posterior  communicating  arteries  forward  to 

463 


464  THE    CEREBRAL    CIRCULATION. 

join  the  internal  carotid.  These  too  send  small  vessels  into  the  basal 
ganglia.  The  internal  carotid  divides  into  the  middle  and  the  anterior 
cerebral,  the  latter  being  united  by  the  anterior  communicating,  which 
thus  completes  the  circle  of  Willis.  From  the  arteries  composing  this 
circle  many  little  branches  perforate  the  base  of  the  brain  supplying  its 
basal  ganglia. 

The  middle  cerebral  artery,  passing  outward  through  the  fissure  of 
Sylvius,  gives  off  in  its  course  many  basal  arteries  which  enter  the 
anterior  perforated  space.  These  ascend  through  and  outside  the  len- 
ticular nucleus  and  supply  the  internal  capsule  and  the  anterior  part 
of  the  optic  thalamus.  (Fig.  213.)  The  middle  cerebral  artery  divides 
as  it  passes  outward  into  several  branches  which  radiate  over  the  island 
of  Reil,  and  spread  outward  over  the  convexity  of  the  brain  supplying 
the  greater  part  of  the  lateral  surface.  (Plate  XXII.)  The  larger 
branches  go  to  the  third  frontal  convolution,  to  the  anterior  and  pos- 
terior central  convolutions,  and  along  the  fissure  of  Sylvius.  The 
Sylvian  artery  gives  off  numerous  branches  to  the  operculum  and 
supramarginal  convolution  above  and  to  the  temporal  convolutions 
below,  and  finally  terminates  by  spreading  out  over  the  parietal  lobules 
and  the  convolutions  joining  the  temporal  and  occipital  regions. 

The  anterior  cerebral  artery  passes  forward,  supplying  the  base  of 
the  frontal  lobe,  then  curves  upward  on  its  median  surface,  sending 
numerous  branches  over  the  edge.  It  supplies  the  median  surface  of 
all  the  frontal  convolutions  and  a  small  part  of  the  cortex  of  the  lateral 
surface  near  to  the  median  line,  excepting  the  third,  which  receives  its 
supply  from  a  branch  of  the  middle  cerebral.  Its  terminal  branches 
pass  backward  as  far  as  the  cuneus,  anastomosing  on  the  way  with  the 
terminals  of  the  middle  cerebral  artery,  and  with  those  of  the  posterior 
cerebral  artery. 

The  anastomoses  of  the  cortical  terminal  vessels  are  most  perfect,  as 
shown  in  Plate  XXIII.  But  many  of  the  small  basilar  vessels  have 
few  or  no  anastomosis  with  adjacent  branches,  and  hence  have  been 
called  terminal  arteries.  The  effect  of  an  embolism  in  such  arteries  is 
to  cause  an  area  of  necrosis  which  is  permanent.  The  basal  arteries 
do  not  anastomose  freely  with  the  cortical  arteries,  and  hence  the  white 
matter  lying  abpve  the  basal  ganglia  and  beneath  the  cortex  is  relatively 
imperfectly  supplied  with  blood.  This  protects  it  from  the  occurrence 
of  hemorrhage,  but  predisposes  it  to  softening  after  thrombosis  or  em- 
bolism. The  cerebellum  is  supplied  by  bloodvessels  which  arise  from 
the  vertebral,  basilar,  and  posterior  cerebral  arteries.  The  blood 
supply  of  the  crura  cerebri  and  pons  and  medulla  is  derived  from 
branches  of  the  basilar  and  posterior  communicating  arteries. 

It  is  evident  that  the  effect  of  an  embolism  or  thrombosis  in  any  of 
these  vessels  may  be  a  very  small  or  an  extensive  necrosis  of  tissue, 
according  to  the  size  of  the  vessel  plugged.  The  extent  of  a  hemor- 
rhage in  the  brain  will  also  depend  on  the  size  of  the  vessel  ruptured. 
There  are  certain  vessels  in  which  embolism  and  hemorrhage  are  more 
common  than  iu  others.     The  small   vessels,  which  enter  the   base 


PLATE    XXII 


Fia 


OTa 


The  Vascular  Supply  of  the  Cerebral  Cortex.      (Dejerine.) 
The  regions  supplied  by  different  arterial  branches  are  shown  in  different  colors 

The  median  surface. — Branches  of  the  anterior  cerebral  artery.  Fia.  Anterior  frontal.  Fim. 
Middle  frontal.  Ftp.  Posterior  frontal.  Branches  of  the  posterior  cerebral  artery.  OTa.  Temporo- 
occipital  anterior.     OTm.  Temporo-occipital  median.     K.  Calcarine. 

The  lateral  surface. — Branches  of  the  middle. cerebral  artery.  Fi.  Inferior  frontal.  Fa.  Ascend- 
inK  frontal.  Pa.  Ai^cending  parietal.  Pi.  Inferior  parietiil.  Pc.  Anijular.  Ta.  Anterior  temporal. 
Tm.    Mirldle  temi)oral.      Tp.   Posterior  tciriporal. 


THE  CEREBRAL  CIRCULATION. 


465 


through  the  anterior  perforated  space  come  off  from  the  upper  surface 
of  the  internal  carotid  artery  or  the  middle  cerebral.  The  current  of 
blood  rushes  directly  into  these,  as   it   is  forced   up  from  the  heart 


Fig.  214. 


Superficial  veins  of  the  external  surface  of  the  left  hemisphere.        1.  Great  anastomotic  vein  of 
Froland.    2.  Lateral  sinus.    3.  Longitudinal  sinus.     (Testut.) 


Fig.  215. 


An  injfotpd  and  oorrodnd  preparation  of  the  cerebral  veins  and  sinuses.     (Huntington.) 

more  forcibly  than  into  other  vessels,  to  reach  which  it  has  to  pursue 
a  more  tortuous  course.     Hence,  these  are  the  vessels  more  commonly 
30 


466  THE  CEREBRAL   CIRCULATION. 

ruptured  under  an  increase  of  pressure  or  plugged  by  a  clot  or  em- 
bolus from  the  heart.  The  distribution  of  these  vessels  is  to  the 
basal  ganglia  and  internal  capsule.  Hence  the  frequency  of  capsular 
lesions.  The  terminal  character  of  these  vessels  makes  repair  im- 
possible. The  free  anastomosis  of  the  cortical  vessels  has  already  been 
noticed.  A  thrombosis  or  embolus  in  one  of  them  rarely  has  a  perma- 
nent effect,  as  the  necrosis  at  first  begun  is  arrested  by  the  development 
of  a  collateral  supply  of  blood.  Hence  cortical  lesions  have  a  more 
favorable  prognosis  than  capsular  lesions. 

The  venous  circulation  in  the  brain  is  shown  in  Figs.  214  and  215. 
The  veins  from  the  interior  of  the  brain  empty  into  the  veins  of  Galen 
and  then  into  the  straight  sinus.  Those  from  the  cortex  reach  the 
longitudinal,  lateral,  and  cavernous  sinuses. 

Intracranial  pressure.  —  Intracranial  pressure  produces  changes  in 
the  pulse  and  arterial  tension  which  can  be  accurately  measured  by 
the  Riva  Rocci  blood  pressure  apparatus.^  When  the  contents  of  the 
skull  is  slowly  increased  by  a  small  tumor  or  by  a  small  clot  the  dis- 
placement of  cerebro-spinal  fluid  and  the  narrowing  of  the  veins  pre- 
vent tangible  effects.  But  when  a  large  clot  suddenly  forms,  the 
pressure  causes  venous  stasis  and  a  slowing  of  capillary  circulation. 
Nature  attempts  to  correct  this  by  raising  the  arterial  tension,  and  thus 
maintains  the  nutrition  of  the  medullary  centres  necessary  to  life.  If 
the  pressure  however  increases,  beyond  the  power  of  the  heart  to  over- 
come, even  while  the  arterial  system  is  contracted,  then,  a  weak  rapid 
heart  action,  a  relaxed  arterial  tension,  and  an  irregular  respiration  are 
followed  by  collapse  and  death.^  The  condition  of  the  arterial  tension 
is  therefore  a  good  index  of  the  degree  of  intracranial  pressure.  A 
tension  of  220  mm.  developed  suddenly  is  dangerous  and  a  tension  of 
280  is  fatal.  Trephining  is  the  only  means  of  relieving  intracranial 
pressure  and  may  be  resorted  to  successfully  in  cases  of  intracranial 
hemorrhage  when  the  arterial  tension  rises  to  the  danger  point.^ 

The  subject  of  decompressive  operations  for  the  relief  of  intiacranial 
pressure  has  been  widely  discussed  in  the  past  few  years  and  will  be 
fully  described  in  the  chapter  on  brain  tumors. 

1  This   can  be  bought  of  Eimer  and  Amend,  New  York,  in  portable  form,  for  |8. 

2  Kocher,  Hirndruck,  Nothnagel's  Spec.  Path.  u.  Ther.,  IX.,  3,  2. 
^Harvey  Gushing,  Amer.  Jour,  Med.  Sci.,  June,  1903,  and  September,  1902. 


PLATE   XXIII 

Fig.   1. 


The  Arterial  Supply  of  the  Cortex  Cerebri.      (Szymonowicz.) 
Section  tliroughi  tlie  cerebral  cortex  of  a  rabbit;  bloodvessels  injected  red.      X  40. 


Fig.  2. 


The  Arterial  Supply  of  the  Cortex  Cerebelli.      (Szymonowicz.) 
Section  through  the  cerebellar  cortex  of  a  guinea-pig:  bloodvessels  injected  red.     X  44. 


CHAPTER  XXVII. 

THE   CEREBRAL   DISEASES   OF   VASCULAR   ORIGIN. 

CEREBRAL  HYPEREMIA  AND  ANEMIA. 

The  investigations  of  Mosso  have  proven  that  any  mental  effort  oi 
emotion  is  attended  by  a  functional  hyperemia  of  the  brain  and  that 
sleep  is  attended  by  a  functional  anaemia. 

Formerly  a  number  of  abnormal  conditions  were  ascribed  to  hyjper- 
cemia  of  the  brain.  It  is  doubtful,  however,  if  they  were  dependent 
upon  this  cause,  and  it  is  improbable  that  congestion  of  the  brain  alone 
is  a  condition  that  can  be  more  than  temporary  or  that  can  produce 
serious  permanent  symptoms.  It  is  admitted  that  in  many  constitu- 
tional states  both  active  and  passive  cerebral  congestion  may  occur. 
Thus  fever,  persistent  mental  strain,  great  emotional  excitement,  sun- 
stroke, and  an  excess  of  alcohol,  quinine,  and  strychnine  produce  con- 
gestion of  the  brain.  Obstruction  to  the  flow  of  blood  from  the  cerebral 
sinuses  may  lead  to  venous  congestion.  In  this  condition  the  symp- 
toms of  the  original  underlying  disease,  such  as  tumor  or  abscess  of 
the  brain,  thrombosis  of  a  sinus,  or  tumors  and  aneurisms  in  the  neck, 
chronic  cardiac  disease,  emphysema,  etc.,  will  occur,  in  addition  to 
symptoms  due  to  the  hypersemia  of  the  brain.  There  is  doubtless  a 
stage  of  congestion  of  the  brain  in  all  cases  of  meningitis  and  of  enceph- 
alitis, but  here  again  it  is  not  possible  to  distinguish  between  the  symp- 
toms due  to  the  two  conditions.  The  symptoms  produced  by  congestion 
of  the  brain  are  headache  of  a  severe  character,  with  feelings  of  pulsa- 
tion, vertigo  increased  by  a  sudden  effort  or  change  of  position,  tinnitus 
aurium,  sensations  of  pressure  about  the  eyes  and  head,  insomnia,  mental 
irritability,  with  sense  of  incapacity  to  carry  on  a  train  of  thought,  and 
restlessness.  The  convulsions  and  coma  in  sunstroke  are  due  to  tox- 
aemia and  not  to  the  congestion.  It  is  probable  that  the  majority  of 
the  symptoms  mentioned  may,  in  some  cases,  be  due  to  imperfect  metab- 
olism, or  to  chemical  changes  in  the  nerve  cells,  rather  than  to  mere 
mechanical  pressure.  I  am  always  reluctant  to  ascribe  morbid  states 
to  congestion  of  the  brain,  and  I  regard  such  a  diagnosis  as  unsatisfac- 
tory and  tentative.  I  do  not  believe  in  the  existence  of  a  chronic  con- 
gestion. The  treatment  of  the  symptoms  during  their  temporary 
duration  is  by  ice  to  the  head  and  the  free  use  of  ergot  and  bromide 
of  sodium.  Such  treatment  will  relieve  the  headache  and  sensations 
of  pressure  in  the  course  of  a  few  hours,  but  should  not  be  kept  up 
more  than  two  or  three  days.  It  is  well  to  give  calomel,  followed  by 
saline  laxatives,  to  restrict  the  diet,  to  cut  off  all  alcoholic  stimulants, 
and  stop  the  use  of  tea,  coff(!e,  and  tobacco  ',  in  other  words,  to  elimi- 

467 


468  CEREBRAL    DISEASES    OF   VASCULAR    ORICm. 

nate  from  the  body  all  those  poisons  which  may  be  the  cause  both  of 
the  congestion  and  of  the  symptoms.  If  the  cause  of  venous  conges- 
tion can  be  ascertained  it  is  to  be  removed,  if  possible.  If  impossible, 
wet  cups  behind  the  ears,  hot  foot  baths,  counter-irritation  to  the  neck, 
and  sedatives  may  relieve  the  symptoms. 

Ancemia  of  the  brain  may  be  due  to  loss  of  blood  or  to  a  general 
anaemic  condition.  The  latter  is  not  uncommon  both  in  young  girls 
and  in  women  at  the  menopause  as  a  primary  disease.  It  may  also  be 
secondary  to  other  constitutional  or  organic  diseases  that  produce  mal- 
nutrition. The  symptoms  of  anaemia  of  the  brain  due  to  a  loss  of  blood 
are  faintness,  vertigo,  nausea,  confusion  of  ideas,  and  finally  a  loss  of 
consciousness,  with  suspension  of  respiration  and  great  feebleness  of 
the  heart.  They  are  usually  relieved  soon  by  a  recumbent  posture,  by 
inhalation  of  ammonia,  and  by  stimulation  with  alcohol.  In  extreme 
cases  transfusion  with  salt  solution  may  be  required. 

The  symptoms  of  anaemia  of  the  brain  due  to  general  anaemia  are 
headache,  felt  chiefly  on  the  vertex,  somnolence,  especially  after  meals, 
depression  of  spirits,  inability  to  concentrate  the  attention,  or  to  con- 
duct any  active  mental  process  for  any  length  of  time,  and  insomnia. 
The  insomnia  of  anaemia  is  characteristic.  The  patient  gets  to  sleep 
easily  on  lying  down;  but  sleeps  only  a  short  time,  and  then  wakes 
and  is  unable  to  get  to  sleep  again. 

ARTERIAL  SCLEROSIS. 

Pathology.  —  Arterial  sclerosis  is  the  result  of  endarteritis.  This 
is  a  chronic  inflammatory  process  in  the  intima  and  muscular  coats  of 
the  vessels,  with  a  production  of  new  cellular  tissue,  which  may(l) 
increase  until  it  obliterates  the  lumen  of  the  vessel,  producing  throm- 
bosis ;  or,  (2)  undergo  calcareous  degeneration,  roughening  the  wall 
and  inducing  fibrin  deposits,  which  may  occlude  the  vessel,  producing 
thrombosis,  or  be  washed  onward,  producing  embolism;  or,(3)  undergo 
fatty  degeneration,  thus  eroding  the  intima  and  weakening  the  muscular 
coat,  causing  aneurisms  (miliary  or  larger)  which  rupture,  producing 
cerebral  hemorrhage.  Miliary  aneurisms  are  about  1  mm.  or  smaller 
in  diameter,  and  are  found  on  many  vessels.  While  they  are  usually 
due  to  endarteritis,  von  Monakow  believes  that  they  may  form  in  senile 
arteries  by  a  bulging  of  a  normal  wall,  the  muscular  coat  of  which  is 
merely  thinned  but  not  diseased. 

The  causes  of  endarteritis  are  syphilis,  the  abuse  of  alcohol,  chronic 
poisoning  by  lead,  the  development  of  poisons  within  the  body  as  the 
result  of  imperfect  nutrition,  or  of  indigestion  due  to  too  much  food 
and  too  little  exercise,  gout,  nephritis,  and  old  age.  The  affection  is 
distinctly  hereditary,  and  in  many  families  death  from  apoplexy  is  the 
rule.  The  use  of  tobacco,  coffee,  and  tea  is  said  to  predispose  to 
endarteritis,  but  this  is  not  established. 

Symptoms.  —  In  the  conditions  of  arterial  sclerosis  it  is  probable 
that  from   time  to  time  a  temporary  stasis  of  blood  may  occur  in  the 


ABTERIAL   SCLEBOSIS. 


469 


domain  of  small  arterial  branches  causing  a  sudden  local  anaemia  of  a 
small  area  ctf  the  brain.  It  is  known  that  such  a  stasis  in  the  arteries 
of  the  leg  gives  rise  to  a  sudden  paralysis  of  the  leg  causing  intermittent 
claudication,  in  which  condition  the  leg  is  useless  for  a  few  minutes. 
The  frequency  with  which  a  sudden  sense  of  inability  to  talk,  to  walk, 


Fig.  216. 


Endarteritis  obliterans.  Occlusion  of  the  right  posterior  communicating  artery  of  the  circle  of 
Willis.  The  vessel  has  dwindled  to  a  fibrous  cord.  Foci  of  atheromatosis  are  noticeable  on  the 
basilar  artery.  M.,  aged  forty-one  years ;  case  of  pseudodementia  paralytica  urfemica.  Reproduced 
from  a  photograph.     ( Berkeley,  Mental  Diseases. ) 

to  do  any  special  act,  or  to  think,  without  any  loss  of  consciousness, 
occurs  in  patients  suffering  from  arterial  sclerosis  leads  to  the  supposi- 
tion that  similar  stasis  may  occur  in  the  brain  — not  of  sufficiently  long 
duration  to  cause  permanent  softening.  The  so-called  serous  apoplexy, 
from  which  patients  die,  M'ith  no  apparent  lesion  in  the  brain  except 
an  cfidema,  may  also  be  thus  explained. 


470  CEREBRAL  Diseases  op  vasculab  oeigin. 

A  careful  study  of  two  hundred  consecutive  cases  of  apoplexy  in 
private  practice  of  which  I  have  records  has  shown  that  in  80  per  cent, 
of  these  cases  there  have  been  distinct  prodromata  of  the  apoplectic 
attack.  For  months,  or  even  years,  before  the  occurrence  of  the 
rupture  or  occlusion  of  the  blood  vessel  the  patients  have  complained 
of  various  cerebral  symptoms.  These  symptoms  all  point  to  a  dis- 
turbance of  cortical  activity.  The  symptoms  are  very  numerous  and 
variable.  Those  that  are  the  most  commonly  observed  are  a  disturb- 
ance of  mental  action  which  the  patients  complain  of  as  a  dullness  and 
hebetude,  difficulty  of  clear  thinking,  difficulty  in  remembering  events 
of  recent  occurrence,  a  sense  of  perplexity  in  regard  to  matters  which 
should  be  clear ;  a  sense  of  difficulty  in  the  management  of  aflFairs 
which  should  be  easy  ;  and  an  irritability  of  temper  and  lack  of  control 
over  emotions,  that  become  a  source  of  annoyance  both  to  themselves 
and  to  tile  members  of  their  family.  Other  patients  complain  of  tem- 
porary sensations  of  numbness  or  weakness  in  one  limb,  or  in  one  side 
of  the  body ;  of  irregular  darting  pains  through  the  head ;  of  ringing 
in  the  ears,  or  of  sounds  in  the  head  ;  of  slight  uncertainties  of  vision, 
occasional  sudden  dimness  being  not  uncommon,  and  of  vertigo  and 
of  loss  of  speech.  These  symptoms  occur  with  such  uniformity  as  a 
preliminary  to  apoplectic  attacks  that  they  must  be  traced  to  the  mal- 
nutrition of  the  neurones  consequent  upon  the  original  arterial  disease. 
As  all  the  processes  of  nutrition  in  brain  cells  and  fibres  must  be  carried 
on  primarily  by  a  process  of  osmosis  through  the  vessel  walls,  it  stands 
to  reason  that  the  earliest  evidence  of  disease  in  these  walls  will  be 
imperfect  function  consequent  upon  malnutrition. 

The  practical  outcome  of  these  observations  is  to  warn  the  practi- 
tioner against  dismissing  a  middle-aged  person  who  complains  of  these 
symptoms  with  the  mere  diagnosis  of  neurasthenia.  In  every  such 
case  the  condition  of  the  blood  vessels  should  be  investigated,  atten- 
tion being  paid  not  merely  to  the  nervous  manifestations,  but  also  to 
the  condition  of  the  heart,  to  the  possible  existence  of  an  accentuated 
second  sound  of  the  heart,  to  the  tension  of  the  arteries,  not  merely  in 
one  wrist  but  in  various  parts  of  the  body,  especially  in  the  temple, 
and  to  the  condition  of  the  kidneys.  If  these  factors  be  recognized 
and  the  condition  of  arterial  sclerosis  be  made  the  object  of  treatment, 
the  so-called  neurasthenia  will  be  much  more  likely  to  yield  to  treat- 
ment than  if  attention  be  merely  concentrated  upon  remedies  directed 
to  the  nervous  system. 

The  treatment  of  the  symptoms  is  by  stimulation  of  the  heart,  but 
this  should  be  temporary  only.  The  underlying  state  is  the  only 
proper  object  for  treatment.     (See  page  506.) 

Temporary  states  of  delirium  or  of  mental  confusion  in  old  persons 
and  senile  dementia  are  usually  the  result  of  long-continued  malnutrition 
of  the  brain  due  to  chronic  arterial  sclerosis.  They  may  develop 
gradually,  or  may  come  on  rather  suddenly  as  a  sequel  of  some  acute 
illness,  especially  with  imperfect  functional  activity  of  the  kidneys. 

The  symptoms  are  in  part  those  just  mentioned  as  characteristic  of 


AMEMIAL  SCLilKOSlS. 


471 


arterial  sclerosis.  But  other  symptoms  are  observed.  One  symptom 
especially  fifequent  is  the  marked  disturbance  of  sleep.  The  patients 
become  excited  toward  evening,  do  not  get  to  sleep,  or,  if  they  do, 
wake  suddenly  early  in  the  night  and  remain  awake.     They  insist  on 


Fig.  217. 


H-\ 


■■/ 


\    . 


« .V-;,: 


^■'4 -vj"- .•■":;;'■. ■■.<^?-  .  <■   i  -%■■;■■• 

*'-'f-&jM->         V''--       Su-'.^- 

>->-'^i.'m .     ■■  1  \:i...'-f  ■■■■. 

i   'A'     ...     }.'^' 


1^ 
'"If 


Section  of  frontal  cortex  in  senile  dementia.  Loss  of  tangential  fibres  in  surface  layer  and  of 
great  distension  of  ttie  capillaries  with  sclerotie  changes.  1  laques  of  gliosis  seen  as  dark  spots 
scattered  widely.  The  cells  show  fatty  degeneration  under  higher  power.  T.  Simchowicz.  Histol. 
Studien  ueber  die  Senile  Demenz,  Nissl's  Arbeit.,  iv.,  267,  1911. 

getting  up,  are  bewildered  as  to  their  surroundings,  do  not  know  their 
family  or  servents,  are  alarmed  at  being  among  strangers,  beg  to  be 
taken  home  and  become  more  and  more  irritable  and  excited  as  their 
wishes  are  opposed.     At  times  they  are  violent,  wish  to  go  out,  attempt 


472  CEBEBBAL   DISEASES   OF    VASCULAB    OBIGIN. 

to  break  the  windows  or  doors,  and  cannot  be  persuaded  that  it  is 
night  and  that  they  are  at  home.  Hallucinations  of  sight  and  of  hear- 
ing may  be  present.  Toward  morning  they  become  exhausted  and  by 
dawn  sink  into  a  restless  sleep  which  grows  deeper  and  they  may  sleep 
till  noon. 

Such  patients  gradually  become  confused  in  their  minds  in  the  day- 
time as  well  as  at  night.  They  show  their  lack  of  recognition  of  their 
surroundings,  think  they  are  not  at  home,  do  not  recognize  the  familiar 
objects  about  them,  but  usually  know  their  family.  They  dwell  on 
the  past ;  allude  to  persons  who  are  dead  as  though  they  were  alive  ; 
telj  of  things  long  past  as  if  of  recent  occurrence ;  but  have  no  rec- 
ollection of  recent  events.  Thus  one  patient  said  in  January  that  it 
was  June,  that  he  was  still  in  his  country  place  and  had  no  recollection 
of  having  come  to  town  in  October  ;  said  that  he  had  been  down  town 
within  three  weeks,  though  he  has  not  been  down  town  for  ten  months  ; 
had  no  recollection  of  important  business  aflPairs  which  had  caused  him 
great  anxiety  last  year ;  and  was  wholly  indifferent  to  matters  told 
him  which  should  have  excited  him. 

In  spite  of  these  evidences  of  mental  failure  some  of  these  patients 
are  quite  sane  at  intervals.  Their  memory  returns  for  a  time  but  goes 
again  in  a  few  hours.  In  regard  to  matters  which  have  long  been  a 
subject  of  thought  and  interest — such  as  the  disposal  of  property — the 
marriage  of  children  —  their  religious  convictions  —  their  habits  of 
life  —  they  may  show  a  power  of  memory  and  of  judgment  which  is 
surprisingly  accurate,  in  view  of  the  temporary  states  of  bewilderment 
and  excitement  to  which  they  are  subject.  In  one  gentleman,  who 
was  always  excited  at  night  and  sleepless,  and  who  had  at  night  hallu- 
cinations of  sight  and  hearing,  and  was  at  times  unable  to  recognize 
his  own  sons,  the  capacity  to  estimate  the  value  of  real  estate  in  various 
parts  of  -New  York  was  good  up  to  his  death  ;  and  his  knowledge  of 
his  property  was  good,  though  for  months  before  the  end  he  would 
make  a  new  will  every  day,  some  containing  most  absurd  provisions, 
others  quite  in  accord  with  a* will  made  before  his  mind  became  weak. 

Such  patients  take  sudden  likes  and  dislikes  to  those  about  them, 
often  preferring  an  ignorant  servant  to  the  members  of  the  family,  and 
often  showing  active  hostility  to  those  formerly  dearest  to  them. 
Under  such  prejudices  undue  influence  is  easily  exerted  and  their  legal 
capacity  may  be  impaired. 

Such  cases  usually  terminate  by  an  apoplectic  attack  or  by  some 
intercurrent  pneumonia,  cystitis,  or  heart  failure.  The  condition  itself 
is  not  fatal.  The  excitement  may  be  quieted  by  active  purgatives  or 
by  hyoscine  hydrobromate,  which  must  be  given  with  great  care. 

APOPLEXY. 

The  result  of  chronic  endarteritis  is  usually  an  attack  of  apoplexy. 
This  may  be  due  to  cerebral  hemorrhage,  or  to  cerebral  thrombosis  or 
to  cerebral  embolism. 


APOPLEXY. 


473 


Pathology  of  Cerebral  Heraorrhage.  —  When  a  cerebral  vessel  rup- 
tures in  the  membranes  the  blood  spreads  out  on  the  surface  of  the 
brain,  filling  the  fissures  and  depressing  the  cortex.  The  clot  which 
forms  is  thin  and  flat.  It  may  become  organized  and  adherent  to  the 
pia  mater,  or  it  may  lie  as  a  clot  for  weeks.  In  one  case  in  which 
McBurney  operated  for  the  removal  of  such  a  clot  from  the  third 
frontal  convolution,  for  the  relief  of  aphasia,  three  months  after  the 
hemorrhage  the  clot  was  only  partly  organized  and  was  easily  sponged 


Cerebral  hemorrhage.    Large  clot  in  the  centrum  ovale  above  the  basal  ganglia.    Patient  lived 
three  days  after  the  onset  in  coma.     (Larkin.) 

off  from  the  surface  and  from  the  fissures.  If  the  hemorrhage  is  sub- 
cortical it  tears  the  surrounding  brain,  sometimes  breaking  into  the 
ventricles  and  filling  them  with  blood.  It  may  be  a  small  clot  or  it 
may^  be  almost  as  large  as  the  hemisphere,  disintegrating  its  greater 
portion.  Usually  it  is  about  two  inches  in  diameter.  The  clot  is 
globular  or  irregular  in  shape  in  the  brain,  or  forms  a  cast  of  the  ven- 
tricles. It  compresses  the  brain  tissue  around  it,  and,  as  a  rule,  a 
nmnber  of  capillary  hemorrhages  are  found  in  the  adjacent  brain. 
The  adjacent  brain  is  always  oedematous.  The  clot  gradually  shrinks, 
and  if  small  undergoes  absorption,  leaving  a  hematin  stain.  The  dis- 
integrated brain  tissue  undergoes  fatty  degeneration,  is  liquefied,  and 
is  in  part  absorbed.  There  remains  a  mass  of  softened  tissue,  mingled 
with  blood,  which  after  a  time  becomes  organized  by  the  formation  of 
connective  tissue  in  and  about  it.  This  connective  tissue  may  form  a 
thick  layer,  or  even  a  firm  fibrous  plaque,  upon  the  surface  of  the  brain, 
closely  adherent  to  the  cortex,  and  supplied  with  numerous  blood- 
vessels. In  one  case  operated  upon  two  years  after  an  injury,  with 
hemorrhage,  it  was  as  hard  as  cartilage.  If  it  is  within  the  brain  a 
firm  mass  of  connective  tissue  enclosing  the  softened  mass  may  grow. 


474 


CEBEBBAL  DISEASES   OF    VASCULAR   ORIGIN. 


Then,  as  the  softened  mass  within  this  wall  becomes  liquefied,  the  final 
result  is  a  cyst  filled  with  fluid,  either  bloody  or  clear,  and  serous.  In 
one  case  I  saw  a  connective-tissue  structure  resembling  a  honeycomb, 
its  cavities  filled  with  clear  fluid.  A  clot  begins  to  lose  its  bloody 
color  from  the  second  to  the  fifth  week,  depending  on  its  size ;  after 
the  eighth  to  the  twelfth  week  it  is  a  yellowish  mass  with  hematin 
stains,  and  within  three  to  six  months  the  connective  tissue  formation 
is  well  under  way.  This  contracts  during  a  year  after  its  formation, 
and  the  process  cannot  be  said  to  have  reached  its  final  stage  of  scar 
tissue  or  cystic  formation  until  the  end  of  the  second  year.     A  cyst 

Fic4.  219. 


Extensive  subarachnoid  iiemorrhage  over  entire  base  of  brain  by  diapedesis  ;  no  rupture  of  the  ves- 
sels ;  no  large  clots.     (Larkin.) 

wall  may  be  apparent  within  a  month  of  the  onset  in  small  hemor- 
rhages, encapsulating  the  softened  mass.  The  torn  brain  never  unites. 
It  undergoes  a  fatty  degeneration  in  the  vicinity  of  the  clot,  and  a  proc- 
ess of  secondary  degeneration  begins  at  once  in  the  course  of  the  tracts 
which  are  injured.  By  the  end  of  the  week  such  secondary  degenera- 
tions can  be  detected  by  the  Marchi  stain  at  long  distances  from  the 


APOPLElT. 


475 


original  lesion,  and  after  six  weeks  a  sclerosis  or  replacement  hyper- 
plasia of  connective  tissue  has  begun  in  the  entire  length  of  the  tract 
which  is  degenerated.  These  degenerations  are  very  extensive  through 
the  centrum  ovale,  as  they  affect  all  the  association  and  commissural 
fibres  which  are  ruptured.  They  can  also  be  traced  through  the 
projection  fibres  downward  into  the  spinal  cord  or  upward  to  the 

Fig.  220. 


Cerebral  hemorrhage.  Large  clot  within  the  lateral  ventricle.  Boy  of  14  suflfered  from  splenic 
anaemia.  The  hemorrhage  occurred  from  capillary  veins  in  the  ependyiua.  No  large  vessel  was  found 
ruptured  ;  death  in  three  hours.     (Larkin.) 


cortex.  Figs.  14  and  15  show  such  descending  degeneration  in  the 
spinal  cord  after  hemorrhages  of  small  and  of  great  extent  in  the 
internal  capsule. 

The  clot  exerts  considerable  pressure  upon  the  brain  tissue  about  it, 
not  only  at  the  time  of  its  formation  but  for  some  time  after.  This 
pressure  is  quite  sufficient  to  suspend  the  function  of  the  compressed 
brain,  and  may  even  be  sufficient  to  set  up  degenerative  processes. 
The  brain  tissue  which  is  merely  compressed  will  resume  its  function 
when  the  pressure  is  removed.  Therefore,  the  immediate  and  tempor- 
ary effects  of  a  cerebral  hemorrhage  are  usually  much  more  severe  and 


476  CEBEBBAL   DISEASES   OF   VASCULAM   OSIGIN. 

extensive  than  the  permanent  ones.  And  hence  a  gradual  and  pretty- 
considerable  relief  from  the  initial  symptoms  is  to  be  looked  for  in  cases 
of  cerebral  hemorrhage.  Such  a  relief,  due  to  slow  absorption  of  the 
clot,  is  necessarily  much  slower  than  the  relief  which  occurs  after 
embolism  or  thrombosis,  where  the  collateral  circulation  is  established 
within  a  week. 

The  situation  of  a  cerebral  hemorrhage  depends  upon  the  fact  that 
certain  arteries  of  the  brain  are  particularly  liable  to  the  formation  of 
miliary  aneurisms.  According  to  Durand-Fardel,  in  75  per  cent,  of 
the  cases  these  aneurisms  are  found  on  the  small  branches  of  the  middle 
cerebral  artery  that  enter  the  anterior  perforated  space,  viz.,  the  len- 
ticulo-striate  and  lenticulo-thalamic  branches.  These  arteries  are  fairly 
large  branches ;  they  are  almost  in  a  vertical  line  above  the  heart,  the 
blood  rushes  in  from  the  internal  carotid  artery  under  high  pressure, 
and  they  are  terminal  arteries  with  no  collateral  circulation.  Hence 
they  have  been  termed  by  Charcot  the  hemorrhagic  arteries  of  the  brain, 
and  as  a  matter  of  fact  more  than  60  per  cent,  of  cerebral  hemorrhages 
are  due  to  their  rupture.  A  clot  thus  forming  destroys  the  lenticular 
or  striate  nucleus,  or  the  optic  thalamus,  and  the  internal  capsule  which 
lies  adjacent  to  them,  or  if  it  forms  higher  up  and  nearer  the  terminal 
branches  it  lies  in  the  centrum  ovale.  In  either  case  if  the  hemorrhage 
is  large  it  is  liable  to  break  its  way  into  the  ventricle. 

The  Sylvian  artery  and  its  branches  have  little  support  to  their  walls, 
as  they  lie  upon  and  not  in  the  brain,  and  are  also  prone  to  the  develop- 
ment of  miliary  aneurisms.  Cerebral  hemorrhage  is  not  uncommon  in 
the  Sylvian  fissure  or  on  the  cortex,  being  found  there  in  24  per  cent, 
of  the  cases.  The  remaining  16  per  cent,  of  the  cases  are  divided 
between  the  brain  axis  and  the  cerebellum,  the  last-named  organ  being 
most  rarely  affected.  In  about  20  per  cent,  of  the  cases  the  hemor- 
rhage breaks  through  into  the  ventricles,  or  occurs  originally  from  a 
vetricular  blood  vessel. 

A  cerebral  hemorrhage  may  result  from  a  diapedesis  of  blood  cells 
from  numerous  capillaries,  without  rupture  of  the  walls  (see  Fig.  222). 
The  blood  then  collects  about  the  vessels  and  infiltrates  the  brain  com- 
pressing individual  neurones  and  axones,  but  not  producing  large  clots 
or  cysts.  The  condition  resulting  is  described  by  the  French  as  hem- 
orrhagic softening  {ramollissement  hemorrhagiqiiej.  It  is  present  in 
many  cells  in  the  brain  tissue  about  a  large  clot.  It  is  the  condition 
which  occurs  in  purpura,  in  pernicious  anaemia,  and  in  leucocythaemia. 

Pathology  of  Cerebral  Embolism  or  Thrombosis.  —  The  artery  being 
occluded  by  an  embolus  coming  from  the  heart  or  larger  arteries,  or 
by  a  clot  forming  upon  the  roughened  wall,  or  near  a  constriction 
formed  by  oblitering  endarteritis,  the  brain  tissue  which  is  supplied 
with  blood  through  it  is  cut  off  from  its  nutrition.  The  first  arrest  of 
blood  supply  is  usually  extensive,  but  when  collateral  circulation  is 
established  the  final  area  or  region  which  softens  may  be  small.  The 
sudden  stoppage  of  circulation  in  the  artery  and  in  its  corresponding 
veins  suspend  the  functions  in  this  extensive  area,  but  as  the  collateral 


APOPLEXY. 


477 


circulation  is  resumed  the  functions  reappear  in  all  but  the  parts  which 
are  perma6ently  deprived  of  blood.  In  the  cortex  the  collateral 
supply  by  anastomosing  vessels  is  extensive.  In  the  ganglia  and 
capsule  it  is  very  imperfect,  as  the  arteries  are  terminal.  Hence  the 
permanent  effect  of  occlusion  is  more  serious  in  lesions  of  the  arteries 
of  the  base  than  in  those  of  the  branches  in  the  cortex.  If  a  large 
vessel  in  the  cortex  —  e.  g.,  a  main  branch  of  a  Sylvian  artery,  or  the 
middle  cerebral  trunk  itself — is  plugged,  the  area  of  softening  may  be 
extensive.  When  a  vessel  is  occluded  a  clot  forms  within  it  which 
extends  backward  to  the  next  large  branch.  In  some  cases  a  second 
embolism  is  produced  by  a  portion  of  this  clot  being  swept  off  into 
this  branch ;  then  a  second  attack  follows  the  first  within  twenty-four 
hours.  This  is  the  explanation  of  the  recurrence  of  symptoms  which 
have  apparently  subsided  on  the  day  after  the  attack  in  cases  of 
embolism.  Thus  in  one  of  my  patients  three  attacks  occurred  on 
three  successive  weeks ;  the  first  caused  paralysis  of  the  arm,  the 
second  of  arm  and  leg,  the  third  of  the  entire  side  and  face. 

Fig.  221. 


Transverse  section  of  the  middle  cerebral  artery  from  a  case  of  obliterating  endarteritis.    The 
proliferation  of  the  inner  coat  and  the  reduction  of  the  lumen  of  the  vessel  are  evident.    (Spiller.) 


A  serous  infiltration  and  oedema  of  the  brain  occurs  in  the  entire 
domain  of  the  artery  whicli  is  occluded,  within  an  hour ;  but  this  is 
to  some  extent  relieved  when  the  collateral  circulation  is  established. 
Capillary  hemorrhage  or  diapedesis  of  blood  cells  is  also  found  in  the 
redematous  brain.  There  are  some  cases  of  apoplexy  in  which  the 
only  le-sion  found  after  death  is  an  cedematous  state  of  one  hemisphere. 
These  are  the  cases  in  which  a  thrombosis  of  one  internal  carotid  or 
middle  cerebral  has  been  followed  by  a  ra})idly  fatal  termination  before 
softening  has  been  pr<;duced. 


478  CEEEBEAL   DISEASES   OF    VASCULAB    ORIGIN. 

The  pathological  process  in  cases  of  embolism  in  which  the  embo- 
lus is  of  bacterial  origin,  as  in  ulcerative  endocarditis,  or  in  pysemic 
emboli,  is  somewhat  different,  as  there  is  an  acute  encephalitis  of  limited 
extent  set  up  in  the  necrotic  area.  In  addition  to  the  fatty  degenera- 
tion there  is  an  infiltration  with  leucocytes  and  the  production  of  pus 
in  which  micro-organisms  are  found.  In  such  cases  there  is  a  tendency 
of  the  process  to  extend  beyond  the  limit  of  the  tissue  first  deprived 
of  its  blood  and  to  the  production  of  an  extensive  encephalitis. 

A  fatty  degeneration  and  a  necrosis  of  brain  tissue  follow  the  occlu- 
sion of  the  vessel  on  the  third  day.     In  the  softened  tissue  there  are 

Fig.  222. 


Obliterating  endarteritis.  The  intima  is  greatly  thickened,  and  one-half  of  the  lumen  is  filled  by 
deposit  of  newly-organized  tissue  that  has  undergone  fatty  degeneration  in  its  outer  part.  A  throm- 
bus Is  seen  adherent  on  one  side  to  the  intima.  It  filled  the  vessel,  but  contracted  during  the  process 
of  hardening  the  specimen. 

at  first  extravasations  of  blood  and  pigment  granules  (red  softening) ; 
later,  after  four  weeks,  these  are  absorbed  and  the  tissue  in  a  stage  of 
fatty  degeneration  is  yellow  (yellow  softening).  If  there  is  simple 
necrosis  without  extravasation  or  fatty  degeneration  in  the  white  mat- 
ter, the  brain  has  its  natural  color,  but  is  soft  (white  softening).  Round 
granule  cells  are  everywhere  to  be  found  in  this  softened  tissue,  and 
are  mingled  with  drops  of  myelin  in  all  stages  of  fatty  degeneration. 
The  perivascular  spaces  are  filled  with  leucocytes  and  small  cells.' 
The  softened  tissue  undergoes  liquefaction  or  becomes  organized  by  a 
connective  tissue  growth.  This  is  formed  partly  by  hyperplasia  of 
the  glia  and  partly  from  the  connective  tissue  of  the  smaller  vessels 
which  proliferates.  It  contracts,  leaving  a  depression,  or  a  cavity,  or 
^  See  Friedmann,  Handbk.  der  path.  Anat.  des  Nervensj^stems,  S.  489,  1903, 


APOPLEXY.  479 

a  scar.  In  some  cases  a  cyst  with  smooth  walls  and  serous  contents  is 
formed.  Such  a  small  cyst  is  shown  in  Fig.  254,  in  the  midst  of  a 
diffuse  tumor  of  the  optic  thalamus.  The  size  of  the  area  of  softening 
and  of  the  subsequent  scar  may  be  very  small,  so  that  at  the  autopsy 
a  mere  patch  of  sclerosis  is  found.  On  the  other  hand,  the  softening 
may  involve  almost  an  entire  hemisphere. 

Occasionally  bilateral  lesions  are  found.  Secondary  degenerations 
follow  softening  exactly  similar  to  those  following  hemorrhage,  already 
described. 

The  situation  of  an  area  of  softening  due  to  embolism  is  not  wholly 
a  matter  of  accident.  As  the  majority  of  emboli  come  from  the  heart 
it  is  natural  that  embolism  of  the  left  hemisphere  should  be  more  fre- 
quent than  that  of  the  right  hemisphere ;  (the  ratio  is  five  to  four 
cases),  the  course  of  the  blood  current  from  the  aorta  being  more  direct 
into  the  left  carotid  artery.     Such  an  embolus  if  large  enters  the  middle 

:  Fig.  223. 


Obliterating  endarteritis.  Thickening  of  all  the  coats  of  the  artery.  The  intima  has  encroached 
upon  the  lumen.  The  new  tissue  has  undergone  fatty  degeneration,  and  a  considerable  mass  of  fatty 
debris  in  which  cholesterin  crystals  can  be  seen,  lies  between  it  and  the  media.  A  thrombus  is  seen 
adherent  to  the  intima. 

cerebral  artery  and  stops  it,  or  one  of  its  branches  in  the  Sylvian  fissure, 
thus  causing  a  necrosis  in  the  central  area  of  the  brain  cortex.  If 
small  it  is  more  likely  to  go  directly  up  with  the  blood  current  into 
one  of  the  branches  of  the  middle  cerebral  that  enters  the  perforated 
space.  It  is  found  that  60  per  cent,  of  emboli  enter  these  vessels,  25 
per  cent,  enter  one  of  the  branches  of  the  Sylvian  artery,  and  the 
remaining  15  per  cent,  enter  some  of  the  smaller  branches  of  the  brain 
axis.     Softening  of  the  cerebellum  from  embolism  is  very  rare. 

The  situation  of  the  area  of  softening  due  to  thrombosis  depends  on 
the  size  and  position  of  the  thrombus.     It  is  determined  by  statistics 


480 


CEEEBBAL   DISEASES    OF    VASCULAB    ORIGIN. 


that  thrombi  form  in  the  following  cerebral  vessels  in  the  order  named, 
viz.,  middle  cerebral  or  its  branches,  basilar,  vertebral,  anterior  cere- 
bral, and  posterior  cerebral. 

The  pathological  changes  following  thrombosis  differ  in  no  way  from 
those  due  to  embolism. 

The  relative  frequency  of  these  brain  lesions  may  be  gathered  from 
the  following  statistics.  In  1908  consecutive  autopsies  at  the  Presby- 
terian Hospital  there  were  112  cases  of  cerebral  hemorrhage  and  48 
cases  of  cerebral  softening.  Old  scars  were  found  in  ten  brains,  and 
cysts  were  found  in  thirteen  brains,  all  but  two  in  the  basal  ganglia. 
In  a  few  cases  multiple  lesions  were  found. 

The  situation  of  the  hemorrhages  and  softening  was  as  follows : 


Table  X.  — Situation  of  Lesions  in  Apoplexy. 


Frontal  lobe  . 

Parietal  lobe. 

Occipital  lobe 

Temporal  lobe 

Caudate  nucleus 

Lenticular  nucleus 

Optic  thalamus 

Internal  capsule 

Lateral  ventricle 

Pons 

Cerebellum  . 

Inferior  peduncle  of  cerebellum 

Under  corpora  quadrigemina. 

On  floor  of  fourth  ventricle    . 

Central  part  of  cerebral  hemisphere 

Multiple  small  hemorrhages  . 


Hemorrhage. 

20 

10 

5 

9 

6 
14 
10 
18 
10 
11 

4 

1 

4 

2 

1 

4 


Softening. 
5 
10 
4 
5 
2 
11 
4 
6 

i 

4 


Etiology.  —  Males  are  more  frequently  affected  by  the  vascular 
diseases  than  females,  and  hence  death  from  apoplexy  occurs  oftener  in 
men.  Of  one  hundred  consecutive  cases  of  my  own  eighty  were 
males.  No  age  is  exempt  from  cerebral  hemorrhage.  It  may  occur 
prior  to  birth,  and  the  infant  may  be  born  with  a  clot  in  the  brain. 
Rupture  of  the  vessels  is  the  probable  cause  of  the  majority  of  cases 
of  infantile  cerebral  palsy  which  follow  traumatism  during  labor  or 
severe  convulsions.  Hence  the  lesion  may  occur  in  infancy  or  child- 
hood. But  persons  in  middle  life  are  most  liable,  the  age  from  forty 
to  fifty  being  the  age  at  which  the  greater  number  of  deaths  occur. 


Table  XL — Age  of  Onset  in  Apoplexy. 


Cases  of 

apoplexy,  wit 

1  autopsy. 

Without 

, 

autopsy. 
Starr. 

Total 

Age. 

Tooth. 

Dana. 

Gintrac. 

1  to  20 

.      8 

4 

19 

12 

43 

21    "  30 

.     7 

6 

19 

6 

38 

31    "  40 

.  17 

10 

37 

15 

79 

41    "  50 

.  43 

11 

21 

53 

128 

51    "  60 

.  24 

7 

19 

52 

102 

61    "70 

.  20 

10 

26 

41 

97 

71   "  80 

.     5 

5 

22 

14 

46 

81   "  90 

,  ... 

... 

... 

5 

5 

APOPLEXY.  481 

When  the  condition  of  endarteritis  is  present  any  severe  effort, 
physical  or  mental,  any  sudden  emotional  shock,  any  sudden  exposure 
to  cold,  a  blow  on  the  head,  or  indulgence  in  alcohol,  or  the  use  of  a 
strong  heart  stimulant  may  cause  a  rupture.  Thus,  lifting  a  heavy 
weight,  straining  at  stool,  the  effort  of  coughing  violently,  or  vomiting, 
and  the  effort  of  running  fast  have  been  the  active  causes  of  an 
apoplexy  in  cases  of  my  own.  I  have  seen  two  cases  in  children  suf- 
fering from  whooping-cough.  I  have  known  a  sudden  grief  and 
sudden  fright,  and  also  a  long-continued  anxiety  followed  by  disap- 
pointment, and  in  several  cases  a  fit  of  rage,  to  be  the  cause  of  a 
hemiplegic  or  aphasic  attack.  I  have  known  many  cases  to  follow  a 
debauch.  A  cold  bath  has  been  known  to  cause  an  attack,  but  this  I 
have  seen  but  once,  when  it  was  taken  soon  after  a  hearty  meal. 
Grant  believes  that  strychnine  and  quinine  in  large  doses  may  cause 
an  attack.  I  have  seen  two  attacks  in  a  young  woman  subject  to 
purpura.  Oppenheim  records  a  case  of  hemorrhage  in  the  brain  fol- 
lowing an  operation  for  bleeding  piles.  The  operation  was  followed 
by  hemorrhage  from  the  lungs  and  from  the  bladder  before  the 
apoplexy  occurred.  The  sudden  arrest  of  menstruation  is  said  to  have 
caused  apoplexy.  A  hemorrhage  sometimes  occurs  during  a  severe 
epileptic  fit.  This  is  a  common  history  in  children.  Cerebral  hemor- 
rhage is  the  cause  of  death  in  many  cases  of  chronic  nephritis,  because 
of  the  existence  of  hypertrophy  of  the  heart  and  of  disease  of  the 
arterial  walls,  which  are  constant  accompaniments  of  that  affection. 
The  increased  intra-arterial  pressure  produced  by  the  force  of  the 
blood  thrown  up  from  the  hypertrophied  heart  is  responsible  for  the 
majority  of  cases  of  cerebral  hemorrhage  occurring  during  Bright's 
disease.  Cases  of  hemiplegia  occurring  with  or  after  diphtheria, 
scarlet  fever,  and  pneumonia  are  due  to  rupture  of  the  arteries.  It  is 
possible  that  the  toxins  of  the  disease  weaken  their  walls. 

The  condition  of  the  bloodvessels  in  purpura  may  lead  to  cerebral 
hemorrhage,  also  the  state  of  the  blood  in  pernicious  anaemia  and  in 
leucocythsemia. 

The  causes  of  embolism  are  cardiac  disease,  with  the  formations  of 
vegetations  upon  the  valves  or  of  small  fibrin  deposits  on  the  rough 
endocardium  which  are  washed  into  the  circulation.  In  ulcerative 
endocarditis  these  contain  numerous  micro-organisms.  Similar  emboli 
may  come  from  the  interior  of  the  larger  arteries,  and  atheroma  of  the 
aorta  is  occasionally  the  cause  of  cerebral  embolism.  An  embolus  in 
a  small  vessel  may  arise  from  a  thrombus  in  a  larger  vessel. 

Clots  may  also  come  from  the  lungs  and  even  from  the  veins  of  the 
body  by  way  of  the  larger  veins  in  the  lungs.  Thus  cerebral  embolism 
is  a  frequent  complication  of  tuberculous  disease  of  the  lungs  and  an 
occasional  complication  of  pneumonia.  I  have  known  two  cases  to 
follow  phlebitis  of  the  saphenous  vein.  In  these  cases  secondary 
thrombi  from  the  lung  were  the  probable  cause.  The  exciting  cause 
of  the  embfjlism  may  be  a  sudden  physical  effort  or  a  sudden  mental  or 
emotional  shock. 
31 


482  CEBEBBAL   DISEASES   OF    VASCULAR   ORIGIN. 

The  cause  of  thrombosis  is  any  form  of  endarteritis  which  narrows 
the  bloodvessel  and  finally  obliterates  its  lumen,  leading  to  an  ob- 
struction of  the  blood  current  and  to  the  formation  of  a  clot.  Ob- 
literating endarteritis  of  syphilitic  origin  is  the  usual  condition  leading 
to  such  a  filling  up  of  the  vessel,  and  as  it  is  a  very  common  condition 
it  is  the  active  cause  of  the  majority  of  cases  of  apoplexy  in  young 
persons.  In  fact  it  may  be  stated  without  hesitation  that  the  vast 
majority  of  cases  of  apoplexy  occurring  before  the  age  of  forty  are  due 
to  syphilitic  endarteritis.  Senile  endarteritis  is  also  very  common. 
The  exciting  cause  of  the  formation  of  a  thrombus  is  anything  which 
weakens  the  action  of  the  heart  and  thus  slows  the  blood  current. 
Cases  of  apoplexy  occurring  during  sleep  are  usually  cases  of  throm- 
bosis. 

Fig.  224. 


f       \ 


Bilateral  softening  of  the  lenticular  nuclei  after  gas  poisoning.    (Larkin.) 

Fig.  224  shows  a  condition  of  softening  in  both  corpora  striata  con- 
sequent upon  thrombosis  in  a  case  of  gas  poisoning.  The  artery  sup- 
plying the  lenticular  nucleus  turns  at  a  rigiit  angle  in  its  course.  If 
the  heart  is  weakened  the  current  of  blood  is  arrested  at  this  turn 
stasis  occurs,  a  thrombus  forms  and  softening  results.  This  is  the 
cause  of  death  in  fatal  cases.  It  is  the  cause  of  motor  paralysis  in  those 
who  recover. 

A  general  state  of  weakness,  a  syncope,  the  reaction  which  follows 
effort,  mental  excitement,  or  emotional  shock,  a  fright,  or  a  condition 
of  exhaustion  from  malnutrition  or  starvation,  may  induce  such  a  state 
of  weakness  in  the  heart  as  to  lead  to  a  stasis  in  the  vessels ;  and  if 
such  a  stasis  occurs  in  a  vessel  whose  lumen  is  contracted  or  whose 
wall  is  rough  a  clot  may  form.  It  is  not  certain  whether  pathological 
states  of  the  blood  may  lead  to  the  formatioa  of  clots  within  the 
vessels,  though  this  is  the  theory  which  accounts  for  the  cases  of 
thrombosis  occurring  in  infectious  diseases,  in  phthisis,  in  gout, 
rheumatism,  anaemia,  chlorosis,  leucocythemia,  and  in  the  puerperal 
state. 

Symptoms.  —  The  symptoms  produced  are  so  nearly  alike  in  cases 
of  hemorrhage  from  a  vessel  and  softening  due  to  obstruction  in  a  vessel 


APOPLEXY.  483 

that  they  may  be  described  together.  It  is  admitted  that  In  extreme 
cases,  sucli  as  a  large  hemorrhage  or  a  small  embolism  they  differ 
widely,  and  that  then  a  differentiation  can  be  reached.  But  it  has  been 
my  experience,  from  a  study  of  a  large  number  of  such  cases  during 
life  and  a  comparison  of  symptoms  with  pathological  findings,  that  it 
is  not  possible  to  arrive  at  a  positive  accurate  diagnosis  between  a  clot 
and  a  softening  in  more  than  one-half  of  the  cases.  Hence  these  symp- 
toms are  here  discussed  together. 

Premonitory  Symptoms.  —  In  all  cases  of  endarteritis  the  patients 
suffer  from  symptoms  of  disturbance  of  the  circulation  in  the  brain  for 
some  months  or  even  years  before  an  actual  attack  of  an  apoplectic 
nature  occurs.  Miliary  aneurisms,  however,  do  not  usually  produce 
any  symptoms,  and  may  be  latent  until  they  burst.  The  cases  which 
uniformly  escape  prodromata  are  cases  of  cerebral  embolism  due  to 
heart  disease  or  those  not  arising  from  endarteritis. 

These  symptoms  are  peculiar  sensations  of  oppression  in  the  head, 
an  internal  pressure,  the  sensation  of  a  band  about  the  forehead,  or  of 
fulness  and  pulsation  of  the  vessels.  These  come  and  go  irregularly 
for  months,  sometimes  after  physical  or,  more  commonly,  after  mental 
effort  or  emotional  excitement ;  sometimes  in  connection  with  atmos- 
pheric changes,  to  which  some  persons  are  particularly  susceptible ; 
sometimes  without  known  cause.  The  influence  of  alcoholic  stimulants 
is  more  quickly  felt  by  persons  who  have  endarteritis,  and  in  such 
persons  disturbances  of  digestion  are  likely  to  cause  vertigo  and 
headache. 

Headache  is  probably  the  most  common  symptom  of  endarteritis. 
It  is  usually  a  dull  frontal  or  occipital  pain,  rarely  unilateral,  and  vary- 
ing in  intensity  from  hour  to  hour  while  it  lasts.  It  occurs  after  mental 
or  physical  effort  or  very  often  after  anything  which  exhausts  the 
strength  or  depletes  the  nutrition.  It  may  be  due  to  congestion  of  the 
brain,  and  is  then  shown  by  a  redness  of  the  face,  with  distended  veins 
and  red  eyes  and  a  feeling  of  fulness  and  pulsation.  It  is  equally  fre- 
quent, however,  in  conditions  of  cerebral  anaemia  and  malnutrition  ;  for 
pale,  ill-nourished  persons,  or  those  who  have  had  exhausting  diseases, 
or  who  have  a  weak  heart  are  just  as  subject  to  headache  as  are  the 
plethoric.  In  such  patients,  however,  there  is  no  congestion  of  the 
face  and  eyes ;  and  the  pain  is  often  felt  in  the  vertex.  Headache  is 
rarely,  if  ever,  a  continuous  symptom,  and  the  attacks  are  sometimes 
few  and  infrequent,  in  other  cases  very  common.  Few  patients  escape 
prior  to  an  apoplectic  attack.  The  headache  in  syphilitic  cases  is 
usually  felt  in  the  afternoon  and  evening.  It  is  remarkable  that  very 
severe  and  persistent  headache  often  disappears  at  once  after  an  apo- 
plectic attack.  When  such  an  attack  is  followed  by  severe  or  persis- 
tent headache  the  prognosis  is  most  unfavorable. 

Attacks  of  vertigo  are  among  the  common  symptoms  of  endarteritis, 
coming  suddenly  after  efforts,  such  as  straining  at  stool,  running,  or 
lifting  weights,  or  after  excitement,  or  in  persons  of  weak  heart,  after 
a  meal.     They  usually  alarm  the  individual^  being  sometimes,  but  not 


484  CEBEBBAL   DISEASES   OF    VASCULAR   OEIGIN. 

often,  bad  enough  to  cause  staggering  or  the  need  of  lying  down. 
They  are  commonly  merely  of  the  nature  of  a  sense  of  swaying,  or  a 
sense  of  uncertain  stability,  or  of  a  movement  of  the  floor.  If  a  person 
has  a  severe  attack  of  vertigo  with  inability  to  rise  and  with  attendant 
faintness  and  vomiting,  it  is  probably  due  to  the  formation  of  a  small 
thrombus  or  to  a  minute  hemorrhage  in  the  cerebellum.  Such  attacks 
often  pass  off  and  leave  no  permanent  trace,  but  occasionally  they  are 
followed  soon  by  more  serious  symptoms. 

Insomnia  is  a  symptom  of  endarteritis.  The  patients  cannot  get  to 
sleep  on  going  to  bed,  and  lie  and  toss  and  think  and  worry ;  finally 
they  are  exhausted  and  fall  into  a  light  sleep  in  which  they  twitch  and 
turn  and  are  troubled  by  distressing  dreams,  waking  in  the  morning 
without  any  sense  of  rest.  This  is  a  condition  commonly  associated 
with  congestion  of  the  brain.  It  may  be  due  to  too  forcible  heart 
action,  as  in  cases  of  endarteritis  associated  with  nephritis  and  hyper- 
trophy of  the  left  ventricle,  or  in  cases  where  the  arteries  are  so  rigid 
as  to  have  lost  their  elasticity  and  power  of  physiological  contraction 
during  sleep.  After  this  kind  of  insomnia  rupture  of  the  artery  with 
the  formation  of  a  clot  is  more  common  than  thrombosis.  In  other 
cases  the  obstruction  to  the  circulation  causes  insomnia  by  depriving 
the  brain  of  blood  and  nutrition.  In  this  condition  the  patients  are 
unusually  drowsy,  doze  in  the  daytime,  especially  after  meals,  and  fall 
asleep  as  soon  as  they  go  to  bed.  They  sleep  heavily,  often  snoring 
when  this  is  not  their  habit,  but  after  three  or  four  hours  they  wake, 
and  then  cannot  get  to  sleep  again.  The  insomnia,  therefore,  in  such 
patients  is  worse  in  the  early  morning  hours.  They  watch  the  day 
break  and  are  early  risers.  This  type  of  insomnia  is  more  difficult  to 
treat  successfully  than  the  other  type.  For  while  the  former  can  be 
cured  by  warm  baths,  by  hot  drinks,  and  food,  by  the  synthetic  hyp- 
notics and  bromides,  the  latter  often  is  intractable.  Sometimes  a  light 
meal  taken  in  the  night  on  waking,  or  a  heart  stimulant,  is  followed 
by  a  nap,  but  hypnotics  are  useless.  These  patients  often  have  their 
apoplectic  attacks  during  the  night,  and  it  is  usually  due  to  thrombosis 
rather  than  to  the  rupture  of  a  vessel.  When  syphilis  is  the  cause  of 
endarteritis  the  patients  usually  have  difficulty  in  getting  to  sleep  early ; 
but  they  sleep  heavily  and  are  wakened  with  difficulty,  being  dazed  for 
some  moments  after  waking. 

Mental  symptoms  are  frequent  prodromata  of  an  apoplexy,  and  are 
often  present  in  a  state  of  endarteritis.  These  may  consist  of  a  sense 
of  difficulty  in  fixing  the  attention,  a  defective  memory  of  names,  a 
slight  tendency  to  use  one  word  in  place  of  another,  a  confusion  of 
thoughts,  or  even  an  inability  to  think  without  conscious  effort  and 
distress,  and  a  marked  emotional  state  due  to  a  lack  of  self-control. 
Thus  patients  may  be  irritable  and  impatient  on  slight  cause,  may  get 
enraged  at  little  things,  or  may  laugh  or  cry  with  hardly  any  provoca- 
tion. It  is  common  for  these  symptoms  to  be  associated  with  headache 
and  to  be  of  temporary  character,  passing  away  with  the  pain.  Patients 
may  suffer  for  many  months  from  these  mental  symptoms,  and  such 
symptoms  may  be  the  only  ones  observed  even  in  an  attack. 


AFOPLEXT.  485 

Seiisations  of  numbness  in  one  arm  or  in  one  leg  or  in  one  entire  side 
are  not  uncommonly  complained  of  by  persons  who  are  the  subjects  of 
endarteritis,  and  are  very  common  prodromata  of  an  apoplexy.  In 
two  hundred  consecutive  cases  of  apoplexy  in  my  private  practice  of 
which  I  have  full  histories,  a  complaint  of  preceding  numbness  had 
been  made  in  eighty-six.  This  consists  of  a  tingling  feeling,  or  the 
same  sensation  as  when  one's  foot  is  asleep.  It  lasts  a  few  moments 
or  even  a  day  or  two,  and  then  passes  off.  It  may  be  produced  ex- 
perimentally in  one-half  of  the  body  by  compressing  the  opposite 
carotid  artery,  and  hence  is  due  to  a  disturbance  of  the  circulation 
producing  anaemia.  When  such  an  attack  of  numbness  wakens  a 
person  at  night  it  may  cause  much  alarm.  It  is  not  to  be  confounded 
with  local  pressure  on  the  ulnar  nerve,  as  it  is  diffused  over  the  entire 
hand;  nor  with  the  numb  fingers  of  acro-parsesthesia,  which  is 
bilateral ;  nor  with  venous  stasis  in  the  limb  from  pressure  which  is 
attended  by  congestion  and  pain.  It  may  remain  permanently,  under 
which  circumstances,  an  actUal  rupture  of  a  vessel  or  a  stasis  of  blood 
in  it  has  occurrred.  A  nd  after  an  attack  of  apoplexy,  with  or  with- 
out paralysis,  numbness  in  the  affected  side  is  almost  always  present 
and  remains.  Hence,  when  such  attacks  of  numbness  occur  they  are 
an  infallible  warning  of  the  danger  of  apoplexy.  Yet  I  now  have 
patients  under  my  care  who  have  had  many  such  attacks  for  several 
years,  but  never  have  had  anything  more  serious.  The  numbness  is 
rarely  attended  by  an  objective  anaesthesia.  It  is  a  purely  subjective 
symptom.  Sometimes  a  slight  awkwardness  is  associated  with  it,  but 
when  an  actual  ataxia  develops  it  is  because  a  lesion  has  occurred. 

Transient  attacks  of  aphasia  or  of  unilateral  paralysis,  usually  of  one 
hand  or  foot,  occasionally  precede  a  permanent  attack. 

Ringing  in  the  ears,  noises  like  bells  or  whistles  heard ;  flashes  oj 
light  before  the  eyes,  or  a  sudden  sense  of  blindness  in  one  side  passing 
off  in  a  few  moments  ;  a  subjective  sense  of  a  bad  odor  or  an  unusual 
taste  have  occurred  in  different  persons  as  prodromata  of  apoplexy 
and  as  symptoms  of  endarteritis,  but  are  far  less  common  than  the 
other  symptoms  mentioned.  Hemorrhage  from  the  nose  is  common  in 
persons  with  endarteritis,  and  retinal  hemorrhages  are  sometimes  found 
by  ophthalmoscopic  examination. 

The  prodromata  of  an  apoplectic  attack  may  occur  for  many  months 
or  years  and  never  be  followed  by  a  stroke.  They  should  never  be 
disregarded,  however,  by  patient  or  physician,  and  it  is  far  better  to 
take  pains  to  avoid  the  active  causes  of  a  cerebral  hemorrhage  or 
thrombosis  than  to  suffer  its  consequences. 

Some  form  of  prodroma  had  been  felt  in  more  than  one-half  of  my 
two  hundred  cases. 

The  Apoplectic  Attack  which  announces  the  occurrence  of  a  cerebral 
hemorrhage,  or  an  embolism,  or  the  formation  of  a  thrombus  is  always 
of  sudden  onset  and  causes  a  great  mental  shock  to  the  patient. 
Apoplexy,  the  falling  as  if  struck  down,  is  not  always  attended  by 
unconsciousness.     In  fifty-four  out  of  two  hundred  cases  a  loss  of 


486  CEBEBBAL   DISEASES    OF    VASCULAE    ORIGIN. 

consciousness  occurred.  But  in  the  liospital  patients  observed  the 
proportion  was  much  greater,  and  in  patients  who  die,  unconsciousness 
at  the  onset  is  the  rule.  The  coma  is  attended  by  stertorous  breathing, 
by  full,  slow,  tense  pulse,  not  irregular  or  intermittent,  by  marked 
venous  congestion  of  the  face,  and  by  complete  relaxation  of  the  limbs, 
so  that  it  is  sometimes  difficult  to  determine  whether  one  side  of  the 
body  is  paralyzed.  In  some  cases  the  head  and  eyes  are  turned  toward 
the  lesion.  The  pupils  are  usually  dilated  in  cases  of  hemorrhage, 
the  one  on  the  side  of  the  lesion  being  the  larger,  and  they  fail  to  react 
to  light.  In  pons  lesions  they  are  contracted.  The  corneal  reflex  is 
lost,  so  that  irritation  of  the  eye  does  not  cause  a  wink.  In  cases  of 
hemorrhage  distention  of  the  retinal  veins  has  been  observed.  The 
temperature  usually  falls  at  first,  and  then  begins  to  rise  within  a  few 
hours,  the  paralyzed  side  being  a  degree  higher  than  the  other.  There 
may  be  an  immediate  unconscious  evacuation  of  the  bladder  and 
rectum,  if  these  are  full.  If  not,  there  is  usually  retention  of  urine 
during  the  period  of  unconsciousness,  and  the  bladder  has  to  be 
emptied  by  catheter  to  avoid  its  rupture.  The  state  of  coma  may 
continue,  the  temperature  may  rise  to  104°  or  105°  F.,  oedema  of  the 
lungs  and  heart  failure  may  follow,  and  the  patient  may  die  within  a 
few  hours  or  on  the  following  day.  Occasionally  in  persons  of  good 
vitality  the  fatal  result  may  be  delayed  as  long  as  a  week,  consciousness 
never  returning.  In  these  cases  the  greater  relaxation  of  the  limbs  on 
one  side,  a  manifest  drawing  of  the  face  and  elevation  of  the  corner  of 
the  mouth  on  the  non-paralyzed  side,  with  dribbling  of  saliva  on  the 
other  side,  the  failure  to  elicit  reflex  movements  by  pricking  one  side, 
the  loss  of  skin  reflexes  and  loss  or  exaggeration  of  tendon  reflexes, 
the  occurrence  of  automatic  or  instinctive  movements  on  the  non- 
paralyzed  side,  or  a  marked  contracture  of  the  arm  and  leg,  indicate 
the  existence  of  a  hemiplegia.  In  one  girl  who  never  recovered  con- 
sciousness, and  whose  lesion  was  an  extensive  hemorrhage  at  the  base 
after  fracture,  any  attempt  to  uncover  the  body  caused  an  automatic 
grasp  and  effort  to  draw  up  the  bedclothing  on  the  unparalyzed  side. 
The  condition  of  coma  is  more  likely  to  occur  when  the  lesion,  either 
a  clot  or  softening,  is  deep  in  the  hemisphere,  is  near  the  lateral  ven- 
tricle, or  involves  the  optic  thalamus.  It  is  also  an  indication  of  a 
large  hemorrhage  or  the  plugging  of  a  large  vessel.  Extensive  corti- 
cal clots  or  thrombi  almost  always  cause  coma,  but  small  ones  do  not. 

The  condition  of  coma  is  usually  attended  by  a  marked  increase  of 
arterial  tension,  as  shown  by  the  Riva  Kocci  apparatus  and  by  the 
sounds  of  the  heart.  This  is  the  probable  cause  of  the  increased 
secretion  of  uriue  which  always  follows  an  attack.  The  urine  if  ex- 
amined is  often  found  to  contain  albumin  and  sometimes  sugar,  which 
disappear  within  a  week  after  the  attack,  and  may,  therefore,  be  due  to 
the  increased  arterial  pressure. 

The  attack  of  apoplexy  may  be  attended  by  a  convulsion.  This 
may  be  general,  and  if  repeated  is  an  indication  that  the  clot  has  burst 
into  the  ventricles.     It  is  a  most  unfavorable  prognostic  symptom. 


APOPLEXY.  487 

The  convulsion  may  also  be  unilateral.  This  is  an  almost  infallible 
sign  of  a  lefeion  of  the  cortex  in  the  central  region. 

If  the  patient  recovers  consciousness  after  the  attack  the  mind  is 
usually  very  dull,  and  response  to  questions  is  for  some  hours  impos- 
sible. Then  attempts  to  talk  are  made,  and  if  there  is  no  aphasia  they 
may  be  intelligent.  Frequently  it  is  some  days  before  the  patient 
recognizes  his  surroundings.  As  the  mind  becomes  clearer  a  definite 
estimate  of  the  symptoms  present  becomes  possible. 

In  the  majority  of  cases  paralysis  is  found.  This  was  present  in 
one  hundred  and  seventy-seven  out  of  two  hundred  patients.  It  occurs 
in  the  form  of  hemiplegia  and  affects  one  side  of  the  body  only.  The 
face  is  drawn  to  the  non-paralyzed  side,  as  there  is  no  longer  any  op- 
position to  the  normal  tone  of  the  non-paralyzed  muscles.  This  gives 
an  unnatural  appearance  to  the  face  which  sometimes  leads  the  unprac- 
ticed  observer  to  suppose  that  the  drawn  side  is  the  one  which  is 
paralyzed.  The  muscles  about  the  eye  which  close  the  lids  are  not 
involved,  and  thus  the  facial  paralysis  in  hemiplegia  differs  from  that 
due  to  an  affection  of  the  facial  nucleus  in  the  pons  or  of  the  facial 
nerve,  in  which  the  eye  cannot  be  closed.  The  motion  of  the  eyes  is 
not  affected  except  in  lesion  of  the  crura  or  of  the  corpora  quadri- 
gemina.  Deviation  toward  one  side  may  occur  during  the  coma,  but 
this  does  not  persist  when  consciousness  returns.  The  tongue  is  also 
paralyzed,  and  when  protruded  turns  toward  the  paralyzed  side,  being 
pushed  over  by  the  healthy  muscle.  Swallowing  may  be  difficult  and 
choking  frequent  for  some  days  after  an  attack,  and  this  is  a  sign  of 
unfavorable  prognosis.  The  head  can  usually  be  turned  freely,  though 
in  severe  cases  the  same  tendency  to  turn  both  eyes  and  head  away  from 
the  paralyzed  side  present  in  the  coma  may  persist  for  some  days.  It 
is  due  to  the  action  of  normal  muscles  whose  opponents  are  paralyzed. 
The  arm  and  leg  are  completely  paralyzed,  are  relaxed  and  offer  no 
resistance  at  first  to  passive  motions.  The  paralyzed  muscles  show  a 
normal  electrical  reaction,  and  have  no  tendency  to  rapid  atrophy  or  to 
fibrillary  twitching. 

During  the  coma  and  state  of  shock  the  tendon  reflexes  may  be  lost, 
but  they  soon  return,  and  by  the  end  of  the  second  day  are  exaggerated 
on  the  paralyzed  side,  the  knee-jerk  being  high,  and  a  crossed  thigh- 
jerk  being  sometimes  obtained ;  that  is,  a  slight  adduction  of  the  well 
thigh  and  extension  of  the  well  leg  follows  a  tap  on  the  patella  tendon 
of  the  paralyzed  side.  Ankle  clonus  is  often  obtained,  and  also  a 
retraction  of  the  great  toe  on  irritating  the  sole  of  the  foot  (Babinski's 
sign).  These  symptoms  continue  even  though  the  paralysis  improves. 
The  skin  reflexes  are  lost  on  the  paralyzed  side,  a  sign  of  great  diag- 
nostic importance  in  distinguishing  a  true  from  a  false  (hysterical) 
hemiplegia.  A  loss  of  the  abdominal  reflex  occurs  in  lesions  of  the 
frontal  region  even  when  there  is  no  paralysis.  The  body  is  some- 
times paralyzed  so  that  the  patient  cannot  turn  in  bed,  or  move  his 
body,  or  sit  up.  Inspection  of  the  chest  usually  shows  that  respiratory 
movements  are  more  active  on  the  paralyzed  side.     The  control  of  the 


CEREBRAL    DISEASES    OF    VASCVLAJR    ORIGIN. 

bladder  and   rectum  are  lost,   and  either  involuntary  evacuations  or 
retention  may  continue  for  many  days. 

As  time  goes  on  the  hemiplegia  may  gradually  subside.  I  have 
known  it  to  pass  off  in  part  after  three  mouths  of  complete  paralysis, 
so  that  the  patient  eventually  walked  and  signed  his  name.  As  a  rule, 
some  beginning  voluntary  motion  is  evident  by  the  end  of  two  or  three 
weeks,  the  power  of  drawing  up  the  leg  being  the  first  to  return,  then 
to  stretch  out  the  leg,  then  the  power  to  move  the  ankle,  and  finally 
some  flexion  of  the  elbow  and  shoulder.  The  finer  motions  of  the 
hand  and  foot  are  regained  last  of  all,  if  they  return  at  all.  The  face 
appears  straighter  in  the  course  of  two  months.  Walking  can  be  re- 
sumed after  a  light  attack  within  a  month,  but  may  be  regained  after  a 
year  of  paralysis. 

Every  possible  variation  in  the  degree  of  the  original  hemiplegia 
may  be  observed.  In  light  attacks  a  sense  of  weakness  only  is  com- 
plained of,  and  these  patients  are  awkward,  but  do  not  lose  power  in 
any  muscle  absolutely,  and  are  soon  able  to  use  the  hand  and  to  walk. 
Such  patients  recover  entirely,  and  may  have  no  trace  of  the  attack  at 
the  end  of  the  year.  After  severe  attacks,  however,  in  which  the  motor 
tract  is  injured,  and  in  which  a  descending  degeneration  occurs,  a  par- 
tial hemiplegia  remains.  The  muscles  gradually  become  rigid  and  slow 
and  stiff  in  motion,  the  power  of  finer  movements  being  chiefly  im- 
paired ;  the  tendon  reflexes  are  exaggerated,  as  is  also  the  muscular 
irritability,  so  that  tapping  a  muscle  causes  it  to  contract. 

Nevi  finds  that  if  a  hemiplegic  while  standing  bends  the  body 
forward  to  a  right  angle  with  the  thighs,  the  limb  on  the  paralyzed 
side  becomes  flexed,  while  that  on  the  sound  side  remains  straight ;  and 
if,  when  lying  down,  the  legs  are  lifted  up,  the  one  on  the  paralyzed 
side  becomes  flexed  when  an  angle  of  40°  is  reached,  while  the  sound 
leg  can  be  lifted  to  at  least  65°  without  flexion.  This  is  of  use  in  dif- 
ferentiating an  organic  from  a  hysterical  hemiplegia.^ 

The  gait  in  hemiplegia  is  typical.  The  entire  side  of  the  body  seems 
to  move  together,  the  body  being  stiif,  the  shoulder  falling  toward  the 
weaker  side,  the  arm  being  carried  in  an  adducted  flexed  position  with 
wrist  pronated  and  fingers  flexed,  the  leg  being  drawn  forward  with 
little  bending  of  the  knee  and  ankle,  or  being  carried  about  in  the  arc 
of  a  circle,  the  ball  of  the  foot  and  its  inner  side  scraping  the  floor. 
The  foot  is  sometimes  turned  in  and  scraped  along  the  floor  on  its  outer 
side. 

When  the  original  lesion  has  been  located  in  the  cortex  but  one  limb 
may  be  paralyzed.  When  it  lies  in  the  crus  the  eye  on  the  side  of  the 
lesion  cannot  be  turned  inward,  upward,  or  downward;  its  pupil  is 
dilated,  and  there  is  a  falling  of  the  upper  lid.  When  it  lies  in  the 
lower  half  of  the  pons  a  condition  of  alternating  paralysis  is  produced, 
the  face  being  paralyzed  on  the  side  of  the  lesion,  the  arm  and  leg  upon 
the  other  side.  In  this  condition  the  facial  palsy  involves  the  upper 
branch  of  the  facial  nerve  so  that  the  eye  cannot  be  closed.  In  a  few 
cases  of  lesion  in  the  medulla  the  face  has  escaped,  the  tongue  has  been 
^  Nouvelle  Icon,  de  la  Salp^triere,  Jan. ,  1910. 


APOPLEXY.  489 

paralyzed  on  the  side  of  the  lesion,  and  arm  and  leg  on  the  opposite 
side.  In  ^lemorrhage  into  the  ventricles,  and  occasionally  in  small 
lesions  of  the  pons  and  medulla  in  the  raphe,  a  bilateral  or  a  general 
paralysis  of  all  the  limbs  is  produced.  Thus  the  original  or  permanent 
distribution  of  the  paralysis  indicates  the  position  of  the  lesion.  In 
200  cases  of  apoplexy  right  hemiplegia  occurred  in  96  cases,  being 
permanent  in  73  ;  left  hemiplegia  occurred  in  55,  being  permanent  in 
44,  alternating  paralysis  occurred  in  4,  a  lesion  of  the  cms  cerebri  in 
1,  and  total  paralysis  in  2. 

In  some  cases  hemiancesthesia  accompanies  the  hemiplegia  ;  in  a  few 
cases  it  is  the  only  symptom  produced.  In  the  majority  of  cases  of 
apoplexy  it  is  absent.  The  symptom  is  a  sensation  of  numbness  in  one 
side,  accompanied  by  a  certain  degree  of  loss  of  the  power  of  per- 
ceiving touch,  or  pain,  or  sensations  of  heat  and  cold.  The  loss  of 
sensation  is  never  permanently  complete  in  organic  lesions.  In  all 
cases  after  the  shock  has  passed  away  and  consciousness  is  clear  some 
sensation  is  perceived,  especially  in  the  shoulder,  or  thigh,  or  foot. 
One  of  these  senses  may  be  partly  lost,  the  others  being  preserved. 
Thus  in  several  of  my  patients  touch  was  very  much  impaired,  but 
hot  and  cold  objects  and  painful  pricks  were  felt  keenly.  In  one  case 
touch  was  not  affected,  but  pain  could  not  be  felt,  nor  differences  of 
temperature.  In  two  cases  the  patient  had  a  constant  sensation  of 
cold  in  the  left  side,  could  not  feel  warm  objects  as  such  at  all,  but 
felt  them  as  cold,  and  felt  touches  and  painful  sensations,  but  less 
keenly  than  on  the  well  side.  In  one  of  these  cases  there  was  a  hemi- 
plegia as  well ;  in  the  other  cerebellar  symptoms  without  hemiplegia. 
In  another  case  the  sense  of  pain  and  of  heat  was  lost,  but  cold  could 
be  felt  and  a  subjective  feeling  of  cold  was  present  all  the  time.  This 
patient  was  slightly  hemiansesthetic  but  had  no  paralysis.  An  alter- 
nating hemiansesthesia  may  be  caused  by  lesions  in  the  pons  or  medulla, 
as  in  three  of  my  patients,  without  any  paralysis.  The  degree  of  the 
anaesthesia  may  vary,  being  very  slight  in  many  patients  and  only 
detected  by  careful  comparison  of  the  two  sides  of  the  body  simul- 
taneously tested.  In  many  cases  the  hemiansesthesia  found  immedi- 
ately after  the  attack  passes  off,  and  in  the  course  of  a  month  sensation 
is  normal.  It  is  only  when  the  sensory  tract  lying  behind  the  motor 
tract  in  the  capsule  is  destroyed,  or  when  the  optic  thalamus  is  affected 
that  a  permanent  blunting  of  sensation  remains.  And  this  is  never 
total,  as  each  hemisphere  of  the  brain  receives  some  impressions  from 
both  sides  of  the  body.  In  200  cases  right  hemiansesthesia  was  found 
in  21,  being  permanent  in  17  ;  left  hemiansesthesia  was  found  in  20, 
being  permanent  in  19  ;  alternating  ansesthesia  was  present  in  3  and 
permanent.  A  loss  of  tactile  memories  is  sometimes  present  and  indi- 
cates a  lesion  of  the  cortex  in  the  parietal  area.  Thus  patients  who 
are  blindfolded  cannot  recognize  objects  placed  in  the  hand.  This  may 
occur  even  when  the  liand  is  not  ansesthetic.  It  is  termed  astereog- 
nosis.     (See  page  109.) 

Ilemiataxia  or  an  impairment  of  muscular  sense  and  consequent 


490  CEREBRAL   DISEASES   OF   VASCULAR   ORIGIN. 

awkwardness  of  movement  is  an  infrequent  accomplishment  of  hemi- 
plegia. It  may  occur  alone.  It  leads  to  an  incoordination  of  motion, 
both  in  the  gait  and  in  the  use  of  the  hand.  In  4  cases  out  of  200 
it  was  noted,  and  in  all  it  was  permanent,  in  3  being  at  first  associated 
with  hemiplegia.  In  one  case  it  was  attended  by  a  complete  loss  of 
temperature  sense,  the  sense  of  pain  being  preserved. 

Hemianopsia  is  a  frequent  symptom  of  apoplexy.  It  is  bilateral 
homonymous  hemianopsia,  causing  blindness  in  one-half  of  both  eyes. 
As  a  rule  the  patient  appreciates  the  blindness  in  the  eye  whose  tem- 
poral field  is  blind,  and  is  not  aware  that  the  other  eye  is  aifected  until 
the  physician  tests  the  visual  fields.  Hemianopsia  is  usually  a  per- 
manent symptom.  In  only  2  out  of  13  cases  in  the  series  of  200  cases 
did  it  pass  away.  But  a  gradual  increase  in  the  area  of  the  visual  field 
is  common,  and  I  have  charts  which  show  a  great  improvement  in 
vision.  In  the  patients  who  recover  the  visual  tract  has  been  com- 
pressed but  not  destroyed.  When  the  lesion  is  in  the  visual  tract 
beneath  the  cortex  the  visual  fields  are  more  irregular  and  asymmetrical 
in  outline  than  when  the  cortex  is  affected.  Right  hemianopsia  is 
more  common  than  left  hemianopsia  —  9  to  4  in  my  series  of  cases. 
(See  page  120.) 

A  loss  of  visual  memories  indicates  a  lesion  of  the  cortex  of  the 
occipital  lobe  and  angular  gyrus.  It  is  termed  mind-blindness  or  psy- 
chical blindness,  and  when  limited  to  the  memories  of  written  language 
is  termed  word-blindness.  I  have  seen  this  as  the  chief  and  only  per- 
manent local  symptom  of  an  apoplexy  ;  the  patient,  who  could  talk  and 
write,  and  was  not  paralyzed,  was  unable  to  recognize  friends  and 
familiar  places,  and  was  unable  to  read  at  all  even  sentences  he  had 
just  written.  It  is  usually  associated  with  right  hemianopsia.  A  loss 
of  color  vision  alone,  hemichromatopsia,  in  one-half  of  both  eyes  has 
been  described,  but  is  very  rare.  I  have  never  seen  this  condition. 
(See  page  122). 

Aphasia  is  a  common  symptom  in  apoplexy,  being  associated  with 
right  hemiplegia  in  right-handed  people  and  with  left  hemiplegia  in 
left-handed  people.  In  two  of  my  cases  it  was  attended  by  left  hemi- 
plegia ;  both  patients  were  left-handed.  It  is  usually  of  the  form  of 
motor  aphasia,  but  any  of  the  various  forms  may,  of  course,  be  found. 
Aphasia  may  occur  as  the  only  symptom  of  an  apoplexy ;  but  this  is 
rare.  In  the  200  cases  of  apoplexy,  118  patients  had  aphasia  at  the 
onset,  and  in  84  this  condition  was  never  entirely  recovered  from, 
though  a  very  marked  improvement  occurred  in  the  majority  of  patients 
when  they  lived  over  two  months.  In  1 7  of  these  cases  aphasia  occurred 
alone ;  in  the  others  it  was  associated  with  hemiplegia.  In  one  case 
it  passed  away  entirely,  though  it  was  the  only  symptom  of  the  attack, 
and  in  another  case  it  subsided  wholly  after  four  months.  In  many 
cases  the  aphasia  remained  after  the  hemiplegia  had  entirely  passed  off. 
The  various  forms  of  aphasia  and  their  significance  in  determining  the 
location  of  the  lesion  have  already  been  considered  (page  136). 

The  mental  symptoms  occurring  in  apoplexy  have  not  been  suf- 
ficiently emphasized  in  text-books.     Some  cases  of  apoplexy  occur  in 


APOPLEXT.  491 

which  they/ilone  are  present ;  confusion  of  mind,  and  inability  to  think, 
to  remember,  or  to  fix  the  attention,  together  with  a  great  emotional 
excitement  and  lack  of  control,  being  felt.  These  are  cases  of  organic 
dementia  and  frequently  present  many  of  the  symptoms  already  de- 
scribed as  characteristic  of  senile  dementia.  Other  cases  cannot  be 
considered  as  demented,  for  though  some  mental  symptoms  are  present, 
and  the  mind  is  never  as  strong,  as  active,  and  as  useful  as  before  the 
attack,  the  mental  symptoms  pass  away.  I  have  a  man  under  my 
observation  now,  perfectly  well,  who  twelve  years  ago  had  an  attack 
with  loss  of  consciousness  and  dementia,  with  lack  of  memory  lasting 
a  month,  and  inability  to  transact  business  for  six  months,  but  with  no 
aphasia  or  paralysis.  In  63  of  my  200  cases,  marked  mental  symptoms 
were  present  and  were  permanent.  In  two  of  these  patients  delusional 
insanity  finally  developed.  In  40  a  peculiar  lack  of  emotional  control 
was  the  marked  symptom.  One  man  was  in  a  state  of  constant  uncon- 
trollable laughter,  would  laugh  aloud  whenever  he  spoke  or  made  any 
voluntary  movement  in  his  left  side,  which  was  partly  hemiplegic. 
In  two  men  frequent  crying  was  complained  of,  and  occurred  on  any 
excitement,  even  when  the  patients  were  amused.  Such  emotional  acts 
may  be  as  uncontrollable  as  a  convulsive  spasm  would  be,  and  are 
equally  without  intention.  In  several  cases  such  emotional  attacks, 
either  of  laughing  or  crying,  were  common  on  any  excitement.  In 
some  cases  after  the  patients  recover  from  the  shock  of  the  attack  there 
remains  for  months  a  distress  on  mental  effort,  a  state  of  bewilderment, 
in  which  they  lose  the  thread  of  conversation,  cannot  read  or  write  for 
any  length  of  time  without  becoming  confused,  and  are  forgetful. 
Some  patients  lose  all  recollection  of  their  surroundings,  and  hence  im- 
agine that  they  are  away  from  home,  in  a  strange  place.  One  such 
patient,  an  active,  intelligent  business  man,  has  been  reduced  to  a  piti- 
able condition,  without  paralysis,  aphasia,  or  dementia,  yet  so  incapable 
that  in  reply  to  almost  any  question  he  answers,  "  I  don't  know,  I  can't 
remember,  I  am  of  no  use."  These  patients  distrust  their  own  capa- 
city, become  vacillating  and  capricious,  are  sometimes  suspicious,  some- 
times easily  influenced  uncjuly  by  others.  In  several  of  these  cases  a 
permanent  loss  of  control  of  the  bladder  and  rectum  has  persisted  after 
the  attack.^ 

Seguin  and  Brissaud  have  noticed  that  a  loss  of  emotional  control 
occurs  more  frequently  in  lesions  of  the  right  frontal  lobe,  and  this  I 
can  confirm.  In  many  cases  in  which  the  mental  symptoms  are  the 
chief  permanent  symptoms  there  has  been  a  slight  facial  paralysis  or 
a  slight  hemiplegia  or  aphasia  with  the  attack,  which  has  subsequently 
passed  off,  thus  showing  that  the  lesion  was  anterior  to  the  central  or 
motor  region.  These  patients  rarely  if  ever  have  hemiansesthesia  or 
hemianopsia. 

Cerebellar  nymptoms,  consisting  of  violent  vertigo,  double  vision,  and 
a  staggering  gait  when  the  attack  is  over,  may  be  the  chief  features 
of  an  apoplectic  attack.     In  such  cases  consciousness  is  rarely  lost, 

'  YoT  furtlier  cjiKes  see  the  Medical  Record,  November  13,  1897,  "On  Some  Unusual 
Forms  of  Apoplectic  Attack."     Also  May  16,  190G,  on  Cerebellar  Apoplexy, 


492  CEitESEAL   DISEASES    OF    VASCULAB    OBIGIN. 

but  the  patient  is  usually  extremely  prostrated,  feels  weak  and  greatly 
alarmed,  may  vomit,  and  break  into  a  cold  sweat,  and  remain  incapable 
of  moving  in  bed  or  of  sitting  up  for  some  days.  All  attempts  at 
motion  are  attended  by  an  increase  of  the  vertigo  and  a  renewal  of 
the  nausea  and  vomiting.  The  pulse  is  weak,  and  may  be  either  very 
rapid  and  intermittent  or  slow.  In  one  such  case  I  have  seen  Cheyne- 
Stokes  respiration.  In  my  collection  of  200  cases  there  were  four  that 
presented  these  symptoms.  As  patients  recover  the  staggering  gait 
may  gradually  improve,  but  there  remains  a  sense  of  uncertainty  and 
a  tendency  to  fall  in  one  direction.  Thus  one  patient  always  felt  as 
if  about  to  fall  to  the  right  side.  She  subsequently  had  a  second 
attack  with  hemiplegia,  and  a  third  attack  which  left  her  demented. 
Nystagmus  is  associated  with  the  vertigo  in  these  cases  and  usually 
persists  as  a  permanent  symptom.  The  head  is  usually  held  stiffly 
and  sometimes  inclines  toward  the  side  of  the  lesion. 

In  severe  cases  of  hemiplegia  with  hemiansesthesia  there  is  a  very 
marked  tendency  to  the  development  of  bed-sores  upon  the  buttock 
and  heel  of  the  paralyzed  side.  The  surface  becomes  red  after  slight 
pressure  of  the  body  weight,  blebs  soon  appear,  and  an  ulcerated  sur- 
face forms  which  shows  a  tendency  to  slough  and  break  down  rapidly, 
so  that  within  a  week  a  deep  sore  may  be  present,  even  exposing  the 
bone  and  causing  necrosis.  The  lesion  in  the  brain  is  certainly  cap- 
able of  inducing  vasomotor  symptoms  in  the  paralyzed  side,  such  as 
cyanosis  and  its  attendant  malnutrition ;  but  it  is  my  conviction  that 
the  development  of  a  bed-sore  is  always  due  to  imperfect  care  and 
lack  of  cleanliness,  and  that  if  the  patient  is  turned  every  half-hour 
and  the  skin  properly  bathed  with  warm  water  containing  an  antiseptic, 
then  with  alum-water  or  alcohol,  such  sores  may  be  prevented.  This 
is  very  difficult  when  the  urine  and  feces  are  passed  unconsciously, 
and  sometimes,  with  lack  of  skilful  nursing,  bed-sores  will  appear. 
They  are  dangerous,  as  many  patients  who  would  otherwise  recover 
die  of  the  septic  infection  thus  produced. 

A  patient  who  is  paralyzed  may  have  difficulty  in  swallowing,  and 
attempts  at  feeding  may  result  in  food  being  inhaled  into  the  lungs. 
Great  care  has  to  be  taken  to  prevent  this  in  patients  who  remain 
semiconscious  for  a  week  or  more  after  an  attack.  The  result  will  be 
to  develop  a  pneumonia  of  septic  origin.  Charcot  believes  that  the 
vasomotor  paralysis  may  involve  the  lung,  and  in  proof  cites  the 
frequency  with  which  pneumonia,  either  septic  or  spontaneous,  de- 
velops on  the  paralyzed  side  in  these  cases.  Such  a  pneumonia  is  a 
common  cause  of  death  after  an  apoplectic  attack,  and  must  be  regarded 
as  of  serious  prognostic  import. 

Patients  who  are  unconscious  during  the  attack  have  retention  of 
urine  and  inability  to  empty  the  i-ectum.  As  a  rule,  the  bladder 
empties  itself  as  soon  as  it  fills  up,  without  their  knowledge  or  control. 
Sometimes  there  is  a  continual  dribbling  of  urine;  and  for  many  days 
after  consciousness  returns  this  symptom  may  continue.  It  is  some- 
times necessary  to  catheterize  such  patients  regularly.     It  is  always 


APOPLEXY.  493 

well  to  have  a  bed  urinal  in  the  bed,  or  pads  of  absorbent  cotton 
about  the  genitals,  which  can  be  changed  frequently.  The  danger  of  a 
septic  cystitis  is  never  to  be  forgotten,  as  it  is  the  cause  of  death  in  a 
certain  number  of  cases  of  apoplexy.  Minkowski  ^  has  shown  that  the 
cortical  centres  of  the  brain  control  the  detrusor  of  the  bladder,  while 
the  subcortical  centers  control  the  sphincter  of  the  bladder.  If  the 
subcortical  centres  in  the  corpora  striata  and  optic  thalariius  are  de- 
stroyed the  patient  has  incontinence  of  urine.  If  the  cortical  centres 
or  the  tracts  from  them  to  the  spinal  centre  in  the  sacral  segments  of 
the  cord  are  affected  the  patient  has  retention  of  urine.  The  rectum 
may  require  regular  washing  out,  or,  in  case  of  impaction,  a  digital 
evacuation. 

Irregular  Types  of  Apoplexy.  —  Some  very  extraordinary  forms  of 
attack  are  occasionally  seen.  Thus  one  patient  had  a  sudden  attack  of 
vomiting  and  vertigo,  and  immediately  developed  a  monospasm  in  the 
arm  with  a  right  hemiplegia  without  aphasia.  There  was  no  loss  of 
consciousness,  but  the  hemiplegia  began  with  a  convulsive  motion  in 
the  right  arm,  which  continued.  Gradually  the  hemiplegia  passed  oif, 
but  the  convulsive  movement  continued.  And  for  three  months  the 
arm  was  constantly  flung  about  with  violent  flail-like  movements  which 
could  not  be  stopped  by  the  will.  These  were  more  violent  than  athe- 
toid  motions,  and  were  still  continuing  at  the  last  report. 

In  another  patient  I  saw  a  series  of  convulsive  attacks,  five  occur- 
ring in  the  course  of  seven  days,  each  limited  to  the  left  side  of  the 
face  and  arm,  each  lasting  a  quatter  of  an  hour,  and  each  causing  a 
temporary  paralysis  which  by  the  end  of  three  weeks  had  entirely  passed 
off.  The  only  permanent  symptom  was  a  slight  degree  of  mental  dul- 
ness  and  apathy  with  incapacity  for  business. 

In  another  patient  a  series  of  attacks  of  a  convulsive  nature  limited 
to  the  right  leg  occurred  during  ten  days  ;  each  attack  was  very  vio- 
lent, so  that  the  entire  body  was  shaken,  and  lasted  for  nearly  half  an 
hour,  leaving  the  leg  paralyzed.  Then  the  attacks  ceased,  the  condi- 
tion of  paralysis  gradually  subsided  in  six  months  and  she  has  had  no 
return  during  the  past  seven  years. 

One  man  suffered  from  a  sudden  distress  in  his  head,  followed  in  an 
hour  by  a  general  convulsion,  which  left  him  with  right  hemiplegia 
and  aphasia.  During  the  following  three  months  the  condition  of 
paralysis  entirely  subsided  and  he  recovered  his  speech,  but  never 
recovered  his  mathematical  faculty.  He  cannot  do  the  simplest  sum. 
This  first  attacK  occurred  in  1887.  Since  that  time  until  his  death  in 
1905  he  has  had,  every  six  or  twelve  months,  sudden  attacks  consist- 
ing of  confusion  of  mind,  complete  inability  to  speak,  though  he  under- 
stands what  is  said,  a  twitching  which  begins  in  his  fingers  and  thumb, 
and  gradually  involves  the  whole  right  arm,  then  the  right  side  of  the 
face,  and  then  the  right  leg,  attended  by  a  tingling  of  the  entire  right 
side.  This  convulsion  lasts  several  hours,  then  ceases  slowly  and 
leaves  a  paralysis  which  passes  off  entirely  during  the  following  two 

»Deut.  Arch.  f.  Nervenh.,  Aug.  21,  1907. 


494  CEBEBBAL    DISEASES    OF    VASCULAR    OBIGIN. 

days.  I  know  of  over  twenty  such  attacks  having  occurred  during  the 
past  fifteen  years.  In  the  interval  he  appeared  hearty  and  vigorous  ; 
though  eighty  years  old,  he  walked  five  miles  a  day.  He  had  some 
difficulty  in  talking,  found  words  slowly,  but  was  not  aphasic.  His 
memory  was  poor,  he  had  no  cardiac  disease,  but  his  arteries  were  tor- 
tuous and  hard.  I  have  similar  histories  in  three  other  cases.  In  all 
these  cases  the  probable  lesion  was  cortical  hemorrhage,  or  thrombosis, 
as  spasm  is  considered  a  sign  of  cortical  irritation. 

General  convulsions  do  not  often  occur  in  an  apoplectic  attack  in 
adults  unless  the  clot  bursts  into  the  lateral  ventricle.  In  children, 
however,  they  are  very  common  at  the  onset,  and  as  we  shall  see  in 
discussing  infantile  hemiplegia,  convulsions  come  either  with  or  after 
the  attack  in  the  majority  of  the  cases.  In  two  young  girls  patients  of 
my  own,  where  the  hemiplegia  occurred  during  an  attack  of  whooping- 
cough,  general  convulsions  accompanied  the  attack.  In  adults  their 
occurrence  is  of  bad  import,  but  in  children  this  is  not  so. 

In  one  patient  who  had  a  sudden  attack  of  left  hemiplegia  at  the  age 
of  sixty-eight  years,  a  violent  tremor  developed  in  the  left  hand  soon 
after  the  attack.  This  persisted  for  five  months  until  his  death.  The 
hand  and  arm  soon  became  cyanotic  and  oedematous,  were  covered  with 
cold  sweat,  and  were  wholly  paralyzed.  He  became  violently  insane 
soon  after  his  attack,  had  well-marked  hallucinations  and  delusions,  did 
not  sleep  for  two  months,  and  finally  died  of  exhaustion. 

In  several  patients  a  febrile  movement  has  persisted  after  the  apo- 
plectic attack,  and  severe  headache  with  insomnia  of  an  obstinate  kind, 
not  much  relieved  by  anything  excepting  large  doses  of  morphine,  have 
appeared.  In  these  patients  the  hemiplegia  has  shown  no  tendency  to 
improve,  and  in  all  a  fatal  result  has  followed  within  two  months.  I 
look  upon  this  combination  of  symptoms  with  much  dread,  and  con- 
sider that  in  all  such  cases  an  acute  inflammation  must  be  set  up  in  the 
brain  about  the  clot  or  softened  area. 

In  the  cases  where  the  clot  invades  the  ventricles,  general  convul- 
sions are  usual,  are  frequently  repeated,  the  paralysis  affects  both  sides 
of  the  body,  sometimes  a  rigidity  of  the  limbs  follows,  the  pupils  are 
widely  dilated,  the  temperature  falls,  the  pulse  is  slow,  and  respiration 
is  labored  and  stertorous.  Such  an  attack  may  follow  a  milder  attack 
after  twenty-four  or  forty-eight  hours,  the  original  clot  breaking 
through  into  the  ventricle.  Hence  the  need  of  keeping  patients  quiet 
after  even  a  slight  attack.  These  patients  usually  remain  comatose  and 
die  within  twenty-four  hours,  of  cyanosis  and  asphyxia.  Yet  in  one 
hospital  case  I  have  seen  a  gradual  recovery  after  two  weeks  of  uncon- 
sciousness attended  by  all  these  symptoms,  and  the  final  result  was  an 
extreme  state  of  hemiplegia  which  remained  six  years  to  my  knowledge 
without  recurrence.  But  one  patient  in  my  series  of  200  cases  showed 
symptoms  of  ventricular  hemorrhage,  and  died  in  the  attack. 

Bulbar  paralysis  of  the  apoplectic  type  is  illustrated  by  the  following 
case  :  A  man  previously  healthy  but  alcoholic  was  suddenly  attacked 
at  the  age  of  fifty-three  years  with  weakness  of  the  entire  left  side,  fol- 


APOPLEXY.  495 

lowed  in  a  few  minutes  by  paralysis  in  the  entire  right  side  and  attended 
by  difficulty  in  speech  and  in  swallowing.  There  was  no  headache,  no 
convulsion,  and  no  loss  of  consciousness.  His  condition  had  been 
stationary  for  five  years,  when  I  saw  him.  His  eyes  were  normal. 
There  was  no  weakness  of  his  face,  but  his  speech  was  thick,  voice 
loud  and  husky,  and  not  under  control,  and  swallowing  and  articula- 
tion were  very  imperfect.  His  tongue  protruded,  but  was  thin  and 
had  a  marked  tremor.  He  was  very  emotional,  laughed  and  cried 
without  cause.  His  power  in  arms  and  legs  was  good,  but  he  had  an 
extreme  degree  of  ataxia  in  all  motions  of  hands  and  legs,  a  very  ataxic 
gait,  increased  by  closing  his  eyes,  but  his  knee-jerks  were  normal. 
His  control  of  his  bladder  was  imperfect.  He  had  no  pain,  no  vertigo, 
no  deafness.  The  probable  lesion  was  a  small  hemorrhage  in  the 
raph6  of  the  medulla  affecting  both  interolivary  tracts,  and  his  ninth 
and  twelfth  nerves.  Such  cases  have  been  observed  and  the  lesion 
found  by  Senator,  Meyer,  and  Spitzka. 

Attacks  of  Apopledie  Nature  without  Loss  of  Consciousness.  —  In  the 
majority  of  cases  of  apoplexy  a  loss  of  consciousness  does  not  occur. 
Under  these  circumstances,  at  the  time  of  the  attack  the  patient  feels 
suddenly  a  sense  of  pressure  in  the  head,  or  a  dizziness,  with  headache, 
or  finds  it  necessary  to  sit  down,  or  is  able  to  drag  himself  slowly  to  a 
chair,  or  falls  if  he  cannot  reach  a  seat.  He  then  notices  a  numbness 
in  one  limb  which  extends  to  the  entire  side  of  the  body,  and  is  attended 
by  some  weakness  in  the  arm  and  leg.  Or  he  becomes  confused  in  his 
mind  and  cannot  find  words,  and  soon  develops  aphasia.  Or  his  numb- 
ness increases,  his  arm  becomes  ataxic,  and  he  moves  his  leg  with  dif- 
ficulty. Or  he  suddenly  becomes  conscious  of  a  blindness  in  one  eye 
which  on  examiuation  is  found  to  be  a  hemianopsia.  Or  he  feels  be- 
wildered and  confused,  and  can  no  longer  understand  what  is  said  to 
him.  Or  he  is  dizzy  and  staggers  in  walking.  Any  one  or  any  com- 
bination of  these  symptoms  may  come  on  suddenly,  may  recede,  and 
then  return,  to  leave  the  patient  finally  in  a  state  of  incapacity  of  some 
kind.  As  a  rule,  when  the  attack  is  slight  and  not  accompanied  by 
unconsciousness  or  followed  by  fever,  there  is  a  fairly  rapid  improve- 
ment ;  and  after  two  weeks  the  patient  is  able  to  sit  up  and  begins  to 
recover  his  lost  power.  But  a  complete  recovery  does  not  often  follow, 
and  some  trace  of  the  original  symptoms  usually  remains.  Aphasia  is 
not  wholly  recovered  from,  some  hesitancy  of  speech  or  tendency  to 
misplace  words  remains,  some  local  symptoms  continue,  and  a  trace  of 
hemiplegia  is  seen  in  his  gait  for  the  rest  of  his  life.  If  the  symptoms 
pass  off  entirely,  as  they  did  in  16  of  my  200  cases,  the  patient  may 
remain  well  for  some  years,  but  the  probability  is  that  a  second  attack 
will  occur. 

Attacks  of  G^radual  Onset.  —  Thus  far  attacks  of  a  sudden  nature 
have  been  considered.  But  in  a  certain  number  of  cases  the  onset  of 
the  nervous  symptoms  is  gradual.  Thus  in  several  of  my  patients  four 
or  five  days  have  elapsed  before  all  the  symptoms  have  appeared.  In 
these  cases  the  mental  dulness  and  apprehension,  the  sense  of  something 


496  CEBEBBAL   DISEASES    OF    VASCULAB   OBIGIN. 

occurring  iu  the  head,  with  pain,  vertigo  and  weakness,  are  present 
throughout  the  period  of  onset.  In  some  cases  aphasia  first  appears, 
then  becomes  more  complete ;  right  hemiplegia  is  added,  and  finally 
hemiansesthesia.  In  other  cases  the  paralysis  advances  slowly  from 
face  to  arm,  and  then  to  leg,  or  in  the  reverse  order,  and  if  riglit  sided, 
aphasia  may  finally  develop.  Mills  ^  has  described  a  slowly  progres- 
sive hemiplegia  ascending  from  the  leg,  the  exact  pathology  of  which 
is  unknown.  In  some  cases  hemiansesthesia  precedes  the  hemiplegia. 
In  some  hemianopsia  first  occurs,  and  then  a  hemiansesthesia  or  sensory 
aphasia,  which  may  subsequently  subside.  In  still  other  cases  the 
mental  symptoms  become  more  and  more  marked,  confusion  of  thought 
is  evident  in  speech  and  action,  and  a  dementia  finally  develops,  often 
attended  by  emotional  excitement,  crying  or  laughing  without  cause. 
In  some  cases  monoplegia,  or  monospasms,  or  a  loss  of  sensation  in  one 
limb  only,  is  the  final  result,  the  initial  symptom  being  more  extensive 
than  the  final  ones.  It  is  in  these  cases  of  slow  onset  that  the  various 
types  of  aphasia  are  particularly  frequent,  and  psychical  blindness  or 
deafness  or  some  form  of  subcortical  aphasia  persists. 

The  recurrence  of  apoplectic  attacks  is  a  danger  to  which  all  patients 
are  liable.  Twenty-two  patients  among  my  two  hundred  cases  suffered 
from  more  than  one  attack,  one  died  in  his  fourth  and  one  in  his  fifth 
attack.  I  have  known  one  person  to  survive  seven  attacks,  and  many 
to  survive  the  third  which  according  to  popular  superstition,  is  uni- 
formly fatal.  The  same  person  may  have  a  recurrence  of  the  same 
symptoms  in  each  attack,  or  may  have  different  symptoms  each  time, 
depending  wholly  upon  the  bloodvessel  which  is  affected.  One  man, 
aged  seventy  years,  had  an  extraordinary  series  of  attacks.  The  first 
and  second  were  attacks  of  right  hemiplegia  with  aphasia,  the  third 
was  of  left  hemiplegia.  These  occurred  on  successive  days  and  lasted 
a  few  hours.  Five  days  later  he  had  a  fourth  attack.  During  the 
following  ten  days  he  had  three  more,  one  right-sided,  two  left-sided. 
All  this  time  he  was  growing  more  feeble,  but  after  each  attack  the 
paralysis  passed  off  in  a  few  hours.  Six  days  after  the  seventh  attack 
he  had  an  eighth  which  left  a  permanent  left  hemiplegia  from  which  he 
died  four  days  later.  His  arteries  were  rigid,  his  heart  was  weak,  but 
the  sounds  were  clear.  The  diagnosis  made  was  multiple  thrombosis, 
and  he  was  treated  by  nitroglycerin  and  strychnine,  the  administration 
of  which  in  each  attack  seemed  to  be  followed  by  a  recovery  from  the 
paralysis.  The  autopsy  showed  multiple  areas  of  softening  due  to 
thrombosis.     In  several  cases  multiple  lesions  were  found  at  autopsy. 

There  is  no  rule  to  determine  the  interval  between  attacks.  A  few 
months,  a  year,  or  even  ten  years  may  elapse  before  the  recurrence. 
The  average  in  twenty-two  cases  showed  that  the  chance  is  in  favor  of 
a  second  attack  within  two  years  of  the  first  one.  But  one  of  my 
patients  did  not  have  his  second  attack  until  fifteen  years  after  the  first 
one. 

The  Terminal  Condition.  —  The  course  of  the  disease  after  an  attack 

*  Journal  of  Nervous  and  Mental  Diseases,  April,  1900  ;  October,  1903, 


APOPLEXY.  497 

of  apoplexy  is  usually  one  of  slow  improvement  and  a  greater  or  less 
degree  of  n&covery.  The  terminal  condition  may  be  much  more  favor- 
able than  the  original  symptoms  would  lead  one  to  suppose. 

Many  patients  remain  in  a  condition  of  partial  mental  enfeeblement 
with  less  control  of  the  emotions  than  before  the  attack,  and  some 
defects  of  memory,  especially  of  events  occurring  about  the  time  of  and 
subsequent  to  the  attack. 

Many  patients  suffer  from  a  partial  hemiplegia.  The  face  recovers 
almost  entirely,  a  slight  flatness  on  one  side,  and  a  little  deviation  of 
the  tongue  only  remaining.  Often  the  facial  expression  of  amusement 
or  of  grief  is  perfectly  shown  when  voluntary  movement  is  at  fault. 
Sometimes,  as  in  one  of  my  cases,  the  voluntary  power  returns  in  the 
face,  but  the  paralysis  is  evident  when  emotion  is  shown.  This 
patient  had  hemiplegia  with  hemiansesthesia.  The  arm  is  usually 
permanently  paralyzed  to  a  greater  degree  than  the  leg,  and  is  more 
commonly  subject  to  contractures.  The  flexor  muscles  are  usually 
contracted  and  hold  the  elbow  flexed,  the  wrist  flexed  and  pronated 
and  the  fingers  closed,  and  the  extensors  and  supinators  being  weak 
cannot  overcome  them.  In  one  patient  the  arm,  forearm,  and  fingers 
were  rigidly  extended,  the  fixed  position  being  similar  to  that  assumed 
in  reaching  out  for  an  object  a  foot  away  from  the  hip.  The  conse- 
quence is  a  marked  deformity  of  the  hand  in  severe  cases  and  an 
awkward  movement  in  light  cases.  A  rigidity  of  the  muscles  develops 
within  two  months  of  the  onset,  or  sooner,  so  that  even  passive  motion 
is  difficult.  This  may  sometimes  be  overcome  for  the  time  being  by 
massage,  by  rapid  motions  of  flexion  and  extension  of  a  limb  by  hot 
applications,  or  by  an  Esmarch  bandage.  As  a  rule,  the  rigidity  is 
absent  when  the  patient  is  asleep,  and  only  returns  after  waking  when 
he  begins  to  move  the  limb  voluntarily.  The  limb  may  be  so  stiff  as 
to  be  painful,  and  pain  on  motion  of  the  joints  is  a  frequent  complaint 
in  hemiplegic  patients.  The  arm  is  usually  held  close  to  the  side,  and 
complete  abduction  or  circumduction  at  the  shoulder  is  rarely  possible. 
The  leg  is  also  rigid,  is  moved  as  a  whole,  and  is  held  straight.  The 
flexors  of  the  knee  and  the  extensors  of  the  foot  are  paralyzed  to  a 
greater  degree  than  other  muscles,  and  their  opponents  are  held  rigid. 
Hence  the  knee  is  not  bent  in  walking,  and  the  foot  often  assumes  an 
equino-varus  position.  In  very  severe  old  cases  in  which  the  patient 
is  confined  to  bed,  an  extreme  flexion  of  hip  and  knee  with  adduction 
of  the  thigh  may  develop.  The  rigid  muscles  may  be  hypersensitive 
to  percussion  or  may  be  too  rigid  to  contract  when  tapped. 

The  tendon  reflexes  are  uniformly  exaggerated  on  the  paralyzed 
side,  and  not  infrequently  on  the  healthy  side  as  well  to  a  less  degree. 
Ankle  clonus  is  easily  obtained,  and  in  extreme  cases  of  hemiplegia  I 
have  obtained  a  clonus  by  depressing  the  patella,  by  depressing  the 
toes,  by  extending  the  wrist  or  fingers  or  thumb.  In  cases  which 
recover  entirely  from  paralysis  this  increase  of  tendon  reflex  may 
remain.      Babinski's  reflex  is  always  present. 

In  some  cases  subsequent  to  hemiplegia  a  slow  spontaneous  but  iu- 
32 


498 


CEBEBBAL   DISEASES   OF    VASCULAR    ORIGIN. 


voluntary  movement  begins  in  the  upper  extremity,  producing  flexion 
and  extension  of  the  fingers  and  hand  and  often  extensive  motions  of 
the  elbow  and  shoulder.  These  are  more  rarely  seen  in  the  foot  and 
in  the  face.  This  is  athetosis.  It  is  increased  by  any  voluntary  effort 
of  the  paralyzed  or  of  the  healthy  hand,  and  cannot  be  arrested.     Emo- 


FiG.  225. 


Athetoid  movements  of  the  fingers.     (Curschmann.) 

tional  excitement  also  increases  the  motion.  This  symptom  is  seen  in 
infantile  hemiplegia  oftener  than  in  adult  cases,  but  it  occurs  in  adults. 
I  have  known  it  to  cause  peculiar  grimaces  in  the  face  and  an  interrup- 
tion of  speech  from  contortion  of  the  muscles  of  the  face  and  tongue. 
Hammond  ascribed  it  to  a  lesion  of  the  optic  thalamus,  but  it  is  now 
regarded  as  an  evidence  of  cortical  irritation.     When  such  symptoms 


APOPLEXY.  499 

are  due  to  lesions  iu  the  optic  thalamus  it  is  probable  that  the  lesion 
causes  an  irritation  of  sensory  fibres  which  is  conducted  to  the  cortex, 
and  there  gives  rise  to  motor  impulses.  Thus  in  several  children  suf- 
fering from  athetosis  no  cortical  lesion  has  been  found  at  operation. 
The  most  pronounced  case  in  an  adult  that  I  have  seen  developed  after 
an  operation  for  the  removal  of  a  brain  tumor  from  the  posterior  cen- 
tral convolution,  and  continued  for  a  year  after  the  operation,  subsiding 
slowly  as  voluntary  power  was  regained.  I  ascribed  it  to  pressure 
from  a  clot  which  was  gradually  absorbed.  Hemiathetosis  is,  there- 
fore, an  occasional  sequel  of  an  apoplectic  attack.  Hemitremor  is  also 
occasionally  developed,  a  tremor  which  may  be  constant  even  during 
rest,  or  which  may  only  occur  on  voluntary  motion.  It  occurred  in 
but  one  of  my  cases.  Rhein  ^  has  shown  that  it  indicates  a  lesion  of 
the  lenticular  nucleus  iu  some  cases.  Hemichorea  may  remain  after  a 
hemiplegia  has  subsided.  In  one  case  where  it  had  been  a  symptom 
for  fifteen  years  Aufschlager  found  an  old  cyst  in  the  optic  thalamus.  ^ 
In  other  cases  lesions  have  been  found  in  the  superior  cerebellar 
peduncles  or  in  the  red  nucleus  of  the  tegmentum. 

The  lack  of  voluntary  exercise  usually  leads  to  a  slight  atrophy  of 
the  paralyzed  muscles.  An  extreme  and  rapidly  advancing  condition 
of  atrophy  without  any  change  in  electrical  reaction  has  been  occasion- 
ally observed,  and  cannot  be  explained. 

The  joints  occasionally  become  painful,  probably  from  disuse,  and 
require  massage  and  passive  motion.  A  true  synovitis  with  hemor- 
rhage into  the  joint  sometimes  develops,  as  Charcot  pointed  out,  but 
this  is  exceedingly  rare.  I  have  never  seen  a  case,  and  question  any 
causal  relation  between  the  apoplexy  and  its  occurrence. 

A  condition  of  hemiansesthesia,  hemiataxia,  and  hemianopsia  occa- 
sionally remains.  The  first  brings  with  it  a  disagreable  sense  of  ting- 
ling or  numbness,  and  leads  to  awkward  movements  even  when  ataxia 
is  absent.  The  second  leads  to  marked  incoordination,  of  finer 
motions,  and  patients  drop  objects  from  their  hands  when  not  looking 
at  them.  Hemianopsia  is  rarely  recovered  from,  but  patients  become 
accustomed  to  having  but  one-half  of  the  field  of  vision  clear,  and  guard 
against  accidents  from  objects  approaching  the  blind  side.  Deafness 
from  brain  lesion  is  so  rare  as  to  be  a  curiosity.  Mills  has  reported  a 
case  with  bilateral  destruction  of  the  temporal  lobes.  These  defects  of 
sensation  are  much  more  likely  to  remain  stationary  than  is  the  condition 
of  paralysis.  Any  one  of  the  many  forms  of  aphasia  already  described 
may  persist  after  a  vascular  lesion.  In  fact  it  is  largely  from  the 
study  of  small  local  areas  of  softening  of  the  brain  due  to  embolism  or 
thrombosis  that  our  knowledge  of  the  possible  forms  of  aphasia  and 
apraxia  is  due.  It  is  not  necessary  to  recount  these  symptoms  here. 
The  peculiar  combinations  of  cranial  nerve  symptoms  with  hemiplegia 
characteristic  of  pons  and  medulla  lesions  need  not  be  described,  as 
they  have  been  considered  in  the  chapter  on  diagnosis  of  local  lesions. 

'Jour.  Nerv.  and  Ment.  Dis,,  Dec,  1907. 

2  5ieitschrift  f.  klin.  Med.,  Bd.  LI.,  th.  2  u.  3,  1903. 


500  CEEEBEAL   DISEASES    OF    VASCULAB    OBIGIN. 

Diagnosis. — An  apoplectic  attack  may  be  distinguished  from  an  at- 
tack of  syncope  by  tlie  pallor,  stoppage  of  heart,  and  weak  respiration, 
dilatation  of  the  pupils,  preservation  of  knee-jerks,  rapid  recovery,  and 
absence  of  coma,  or  subsequent  local  symptoms  of  a  brain  lesion  in 
syncope. 

An  epileptic  attack,  with  cry,  biting  of  the  tongue  and  general  con- 
vulsion is  followed  by  a  state  of  coma  which  may  be  mistaken  for 
apoplexy  when  no  history  of  the  onset  can  be  obtained.  But  an  epi- 
leptic's tongue  is  scarred,  and  there  are  usually  scars  upon  his  head  or 
extremities  as  evidenc3  of  prior  attacks.  Epilepsy  is  a  disease  of  youth, 
and  the  epileptic  rarely  shows  signs  of  the  vascular  conditions  which 
lead  to  apoplexy.  The  coma  is  not  very  deep,  the  patient  when  aroused 
shows  no  paralysis,  the  pupils  are  equally  dilated,  there  are  often  hemor- 
rhages in  the  conjunctiva  and  capillary  hemorrhages  on  the  face.  And 
within  an  hour  or  two  returning  consciousness  enables  a  history  of  prior 
attacks  and  the  absence  of  any  paralysis  to  be  established. 

The  coma  of  alcoholic  intoxication  is  not  as  deep  as  that  of  apoplexy, 
and  the  drunken  man  can  usually  be  aroused,  and  gives  evidence  of 
his  condition  by  his  breath,  his  dirty  appearance,  his  maudlin  resist- 
ance, his  delirium  and  restlessness,  and  his  lack  of  paralysis.  The 
corneal  and  pupil  and  tendon  reflexes  are  preserved.  It  should  not 
be  forgotten,  however,  that  some  cases  of  apoplexy  occur  in  a  state  of 
intoxication,  or  from  traumatism  in  this  state,  and  symptoms  of  com- 
pression of  the  brain,  unilateral  paralysis,  or  inequality  of  the  pupils, 
should  lead  to  careful  watching  in  any  case  of  doubt.  The  signs  of 
alcoholic  intoxication  will  pass  off  in  a  few  hours,  a  brisk  purgative  or 
a  large  salt  enema  hastening  recovery. 

The  coma  of  opium  poisoning  is  attended  by  very  slow  respiration, 
small,  rapid  pulse,  cool  skin  of  blue  color,  and  extreme  contraction  of 
the  pupils ;  it  is  never  as  deep  as  the  coma  of  apoplexy,  and  never 
attended  by  unilateral  symptoms  or  loss  of  the  corneal  or  tendon 
reflexes. 

A  ursemic  condition  leading  to  convulsions  and  coma  may  be  mis- 
taken for  apoplexy  if  a  full  history  of  the  preceding  symptoms  of 
nephritis  is  not  obtainable.  But  the  symptoms  of  oedema  of  the  tibiae 
and  face,  the  urinary  odor  of  the  breath,  the  small  amount  of  bloody 
urine  of  high  specific  gravity  and  loaded  with  albumin  and  containing 
casts  will  point  to  the  correct  diagnosis  in  cases  of  acute  Bright's  dis- 
ease. Ursemic  coma  is  the  culmination  of  an  attack  which  begins  with 
vomiting,  headache,  and  convulsions.  In  cases  of  chronic  cirrhotic  kid- 
neys apoplexy  is  a  common  occurrence,  and  hence  in  such  cases  the 
finding  of  casts  and  albumin  in  small  amount,  with  urine  of  a  low  specific 
gravity,  does  not  aid  in  the  diagnosis.  And  here  it  is  only  by  the 
absence  of  the  active  local  symptoms  of  apoplexy,  especially  the  lack  of 
unilateral  paralysis  or  difference  in  reflex  actions  on  the  two  sides,  that 
a  diagnosis  is  to  be  reached.  It  is  often  wise  to  watch  a  case  for 
twenty-four  hours  before  arriving  at  a  conclusion. 

The  same  may  be  said  of  diabetic  coma,  though  here  the  exami- 


blAGNOSIS.  501 

nation  of ^he  urine  is  of  far  greater  value,  as  the  glycosuria  which 
follows  a  cerebral  hemorrhage  is  rarely  as  extreme  as  that  found  in 
diabetes.  The  odor  of  acetone  is  unmistakable,  and  is  not  present  in' 
apoplexy  unless  this  is  associated  with  diabetes.  In  cases  of  doubt 
reliance  must  be  placed  upon  the  previous  history,  as  diabetic  coma  is 
not  of  sudden  onset,  is  usually  preceded  by  headache,  anxiety,  distress, 
and  sensations  of  suffocation,  followed  by  somnolence.  But  it  is  to 
be  remembered  that  diabetic  patients  sometimes  have  apoplectic 
attacks. 

An  apoplectic  attack  may  be  the  first  symptom  of  general  paresis, 
or  may  occur  at  any  time  in  the  course  of  the  disease.  The  symptom 
will  then  be  due  to  a  hemorrhage  in  the  meninges,  usually  a  dural 
hematoma,  and  will  be  identical  with  those  of  a  cortical  cerebral  hemor- 
rhage at  the  time  of  the  attack.  The  history  of  mental  symptoms  of 
a  grandiose  kind,  of  mental  failure,  and  loss  of  memory  ;  of  attacks  of 
emotional  excitement ;  of  disturbances  of  speech  ;  of  unusual  physical 
efforts  in  a  person  really  weak ;  of  exaggerated  knee-jerks,  and  other 
signs  of  paresis,  will  lead  to  the  diagnosis.  And  the  result  will  be  a 
much  more  rapid  and  complete  recovery  from  the  hemiplegia  or  aphasia 
produced  by  the  attack  than  is  ever  seen  in  a  hemorrhage  which  destroys 
the  brain. 

The  differentiation  between  cerebral  hemorrhage,  thrombosis,  and 
embolism  is  always  difficult  and  often  impossible ;  but  when  the  char- 
acteristic symptoms  of  any  one  of  these  affections  are  present  a  prob- 
able diagnosis  can  be  reached. 

In  hemorrhage,  if  the  person  is  young,  there  is  a  history  of  whoop- 
ing-cough, of  convulsions,  or  of  purpura  ;  if  old  (and  the  majority  of 
cases  occur  after  the  age  of  forty),  the  individual  is  ruddy,  muscular, 
or  corpulent,  has  a  history  of  nephritis  or  of  endarteritis,  has  no  his- 
tory of  syphilis,  has  no  cardiac  murmur,  but  has  a  strong  and  possibly 
hypertrophied  heart.  There  is  usually  a  history  of  mental  excitement 
or  physical  exertion  preceding  the  attack,  but  rarely  any  history  of 
premonitory  vertigo  or  mental  confusion.  There  is  a  history  of  very 
sudden  loss  of  consciousness,  with  flushing  or  cyanosis  of  the  face,  a 
marked  pulsation  of  the  bloodvessels,  the  pulse  being  full,  slow,  and 
irregular,  of  high  tension,  a  slow  stertorous  respiration,  and  deep  coma 
from  which  these  patients  cannot  be  aroused.  There  is  often  a  differ- 
ence in  the  size  of  the  pupils,  and  they  fail  to  react  to  light,  and  the 
eyes  are  turned  to  one  side.  During  the  first  day  there  is  a  fall  of 
temperature  to  97.5°  F.,  followed  by  a  slow  rise  to  102°  F.  A  rigidity 
of  the  paralyzed  limbs  occasionally  appears,  but  unilateral  twitching 
or  convulsions  are  rare.  The  paralyzed  side  at  first  feels  cooler,  but 
when  the  temperature  of  the  body  begins  to  rise  it  is  often  found  to 
be  a  degree  higher  than  the  other  side.  The  paralyzed  extremities 
are  sometimes  slightly  cedematous,  often  cyanotic,  and  sweat  freely. 
The  urine  often  contains  albumin  and  sugar  after  a  hemorrhage.  A 
rapid  increase  of  leucocytes  in  the  blood  follows  hemorrhage.  When 
the  patient  recovers  consciousness  after  twenty-four  hours,  or  not  until 


502  CEBEBBAL   DISEASES    OF    VASCVLAB    OBIGIN. 

after  two  or  three  days,  he  is  much  dazed,  comes  to  himself  very  slowly, 
complains  of  pain  in  the  head,  is  restless,  and  tries  to  move ;  is  sleep- 
less, but  is  very  dull  mentally  and  incapable  of  showing  an  apprecia- 
tion of  his  condition  for  at  least  a  week.  Hemiplegia  is  almost  always 
present ;  often  associated  with  hemiansesthesia,  and  sometimes  with 
hemianopsia,  though  the  two  latter  symptoms  may  subside  within  the 
first  ten  days.  Aphasia  is  almost  always  present  if  the  right  side  of 
the  body  is  paralyzed,  but  is  rarely  one  of  the  pure  special  types,  such 
as  word-deafness,  or  word-blindness,  or  a  pure  motor  aphasia.  Bed- 
sores occur  more  frequently  in  cases  of  hemorrhage  than  in  thrombosis 
or  embolism,  and  so  does  pneumonia.  During  the  month  following  a 
hemorrhage  there  is  a  gradual  but  steady  improvement  in  both  mental 
capacity  and  in  the  local  symptoms.  Some  permanent  defect,  however, 
is  always  left  with  signs  of  secondary  degeneration  in  the  motor  tract 
in  the  majority  of  cases.  Retinal  hemorrhages  occasionally  may  be 
seen  with  the  ophthalmoscope. 

In  thrombosis  the  patient  if  young  has  a  history  or  physical  signs  of 
syphilis,  and  if  old  is  the  subject  of  endarteritis,  his  temporal  arteries 
being  tortuous,  his  radial  arteries  hard,  his  pulse  irregular,  and  his 
second  heart  sound  accentuated.  There  may  be  a  history  of  some 
acute  illness,  especially  of  an  infectious  type,  just  prior  to  the  attack. 
There  is  a  history  of  some  premonitory  symptoms  extending  back 
several  months,  such  as  headache,  vertigo,  or  insomnia,  even  if  there 
have  been  no  slight  attacks  of  numbness  or  of  paralysis.  It  is  particu- 
larly in  these  cases  that  the  prodromata  which  have  been  described 
occur.  There  is  no  nephritis,  and  the  urine  is  normal.  There  may 
be  a  history  of  some  shock  or  fright,  of  some  general  sense  of  faintness 
or  weakness  just  prior  to  the  attack.  There  is  usually  a  slow  onset  of 
the  attack,  giving  a  few  moments  of  alarm  and  conscious  illness ;  in 
fact,  the  patient  may  feel  his  paralysis  coming  on  before  consciousness 
is  lost.  In  the  majority  of  the  cases  there  is  no  loss  of  consciousness. 
The  face  is  pale,  the  pulse  normal,  not  full  or  slow,  the  respiration  is 
regular,  and  there  is  no  fall  of  temperature.  The  pupils  are  equal  and 
react  to  light.  The  coma  is  not  very  deep  when  it  occurs,  and  irrita- 
tion of  the  body  produces  automatic  movements  which  show  one  side 
to  be  paralyzed.  Twitching  of  the  limbs  is  common,  convulsions  rare. 
The  coma  does  not  last  more  than  twelve  to  twenty-four  hours,  and 
when  consciousness  returns  the  mind  is  clear,  at  first,  though  excite- 
ment and  even  delirium  often  follow.  There  are  no  signs  of  increased 
intracranial  pressure.  The  local  symptoms  are  less  extensive  than  in 
hemorrhage,  and  rather  more  so  than  in  embolism.  They  show  con- 
siderable variation  in  the  first  week.  Thus  a  hemiplegia  may  pass  into 
a  monoplegia,  a  hemiansesthesia  may  wholly  disappear,  and  a  general 
aphasia  subside  into  some  special  type,  as  word-deafness  or  word-blind- 
ness. In  other  words,  the  compensatory  collateral  circulation  in  the 
brain  being  established  and  the  initial  oedema  subsiding,  the  functions 
at  first  suspended  may  be  resumed.  But  the  area  of  the  brain  perma- 
nently softened  does  not  get  back  its  power,  and  hence  permanent  local- 


DIAGNOSIS.  503 

izable  symptoms  of  small  extent  remain,  and  these  show  little  tendency 
to  improve.  Mental  symptoms  of  the  nature  of  impairment  of  memory, 
loss  of  emotional  control  and  excitement  as  permanent  symptoms  are 
more  common  in  thrombosis  than  in  hemorrhage  or  embolism,  and  both 
precede  and  follow  the  attack.  And  there  is  a  marked  tendency  to 
recurrence  of  attacks,  usually  without  loss  of  consciousness,  with  a 
renewal  of  symptoms  which  have  in  part  subsided,  or  with  new  symp- 
toms of  a  local  kind.  The  ophthalmoscope  may  show  arterial  sclerosis 
in  the  retina. 

In  embolism  the  patient  is  usually  young  and  has  an  audible  heart 
murmur  or  a  history  of  phlebitis,  endocarditis,  rheumatism,  or  infec- 
tious disease  ;  is  not  the  subject  of  nephritis,  and  has  no  signs  of  endar- 
teritis. He  has  no  special  apoplectic  appearance,  and  is  pale  rather 
than  ruddy  during  the  attack.  The  attack  occurs  without  premonition, 
and  not  after  effort  or  motion.  It  is  not  always  attended  by  a  loss  of  con- 
sciousness. When  coma  occurs,  and  as  a  rule  it  does  not,  it  is  not  very 
deep,  and  irritation  of  the  body  usually  causes  some  automatic  motions, 
even  if  there  is  no  semiconscious  response.  The  pulse  is  not  full  and  not 
slow,  but  is  such  as  would  be  present  in  the  cardiac  state  found.  The 
respiration  is  rarely  stertorous,  and  the  temperature  does  not  fall.  The 
pupils  are  not  unequal  in  size  and  usually  react  to  light.  Unilateral 
spasms,  or  twitching  of  the  paralyzed  limbs,  or  convulsions  beginning 
in  one  limb  and  extending  to  others  finally  becoming  general,  are  not 
infrequent,  and  during  the  attack  and  after  consciousness  is  regained 
these  may  recur.  There  is  no  difference  in  the  temperature  of  the  two 
sides,  and  no  oedema  or  cyanosis.  Leucocytosis  is  less  mgirked  than  in 
hemorrhage.  When  the  patient  recovers  consciousness  he  does  so 
rapidly,  does  not  complain  of  symptoms  of  pressure  in  the  brain,  but 
often  passes  into  a  state  of  delirium  which  may  last  some  days.  Ex- 
tensive local  symptoms  are  much  less  common  than  limited  ones.  Thus 
some  special  type  of  aphasia  easily  distinguished  from  other  types, 
monoplegia  rather  than  hemiplegia,  or  hemianopsia  alone  are  the  symp- 
toms found  after  the  third  day.  There  is  often  a  very  surprising  im- 
provement in  all  the  local  symptoms  on  the  second  day,  with  a  return  of 
them  on  the  third  or  fourth  day,  followed  then  by  a  very  slow  improve- 
ment. Sometimes  the  local  symptoms  are  permanent.  In  other  cases 
they  may  entirely  pass  away,  and  no  trace  of  the  attack  may  remain. 

The  ophthalmoscope  may  show  embolism  of  the  central  artery  of 
the  retina. 

When  in  any  case  the  symptoms  mentioned  are  present  there  is  little 
doubt  as  to  the  diagnosis.  But  in  many  cases  there  is  an  absence  of 
many  of  the  distinguishing  symptoms,  and  a  differentiation  is  impossible. 
This  fact  is  substantiated  also  by  autopsy  records.  Thus  in  twenty  cases 
of  supposed  embolism  where  cardiac  disease  was  present  and  the  attack 
was  fairly  typical,  nine  were  found  after  death  to  have  been  cases  of 
hemorrhage  (Kleber  ^).  It  has  been  my  experience  that  in  few  con- 
ditions are  errors  of  diagnosis  more  common  than  in  apoplexy. 

^  Von  Monakow.  Gehirnpathologie ;  Spec,  Path.  u.  Ther.  von  Nothnagel,  Bd.  xi., 
S.  776. 


504  CEBBBMAL   DISEASES   OF    VASCVLAB   OMIGIN. 

Ad  heroic  method  of  diagnosis,  by  boring  a  hole  in  the  skull  and 
aspirating  the  brain,  thus  finding  hemorrhages  and  relieving  pressure, 
has  been  advocated  by  Neisser,^  who  reports  a  case  of  extra-dural 
hemorrhage  in  the  posterior  fossa,  in  which  180  cc.  of  blood  was 
removed.  Deaths  have  been  reported  due  to  hemorrhage  in  the  brain 
after  such  explorations.  They  may  be  warranted  to  ascertain  the 
existence  of  extra-dural  clots  but  not  otherwise. 

Prognosis.  —  The  prognosis  in  an  apoplectic  attack  depends  some- 
what upon  the  cause  of  the  apoplexy.  Cerebral  hemorrhage  is  much 
more  likely  to  be  fatal  than  thrombosis  or  embolism.  The  first  attack 
is  less  likely  to  be  fatal  than  subsequent  attacks.  The  older  the 
patient  the  less  likelihood  of  his  recovery.  Very  deep  coma,  its 
duration  for  more  than  a  day,  a  rapid  fall  of  temperature,  or  a  rapid 
rise  of  temperature,  the  development  of  Cheyne-Stokes  respiration, 
heavy  stertor,  the  deviation  of  head  and  eyes  to  one  side,  are  all 
important  and  very  unfavorable  symptoms,  a  sudden  extreme  degree  of 
arterial  tension,  over  240  mm. ;  or  a  sudden  fall  of  tension,  below  80 
mm.  is  unfavorable.  (See  page  466.)  If  convulsions  occur  with 
signs  of  increased  intracranial  pressure  a  ventricular  hemorrhage  has 
probably  occurred  and  death  is  sure.  Retinal  hemorrhages  are  of  bad 
import,  as  they  show  a  high  degree  of  pressure  in  the  skull,  and  hence 
a  large  clot.  Bilateral  paralysis,  especially  if  attended  by  cranial 
nerve  palsies,  shows  a  lesion  of  the  pons  or  medulla  which  is  almost 
surely  fatal.  It  is  not  to  be  forgotten  that  a  slight  attack  may  be  fol- 
lowed immediately  by  another,  more  severe,  and  this  by  a  fatal  attack. 
If  the  patient  recovers  consciousness  after  the  attack  the  development 
of  delirium,  of  high  temperature,  of  insomnia,  of  great  restlessness,  of 
pain  in  the  paralyzed  limbs,  a  tendency  to  bed-sores,  and  the  failure 
to  control  the  sphincters  make  a  fatal  termination  within  two  weeks 
probable.  If  few  or  none  of  these  symptoms  occur,  and  the  temper- 
ature becomes  normal  within  a  week,  and  some  slight  improvement  in 
the  local  symptoms  is  manifest,  life  will  probably  be  spared. 

The  greatest  caution  must  be  exercised  in  giving  a  prognosis  on  the 
degree  of  recovery  from  the  local  symptoms. 

Aphasia  which  is  general  at  the  outset  always  passes  away  in  part, 
and  has,  in  my  experience,  more  chance  of  improvement  than  any  other 
local  symptom.  A  pure  type  of  aphasia,  motor  or  sensory  at  the  out- 
set, is  usually  permanent,  though  careful  education  enables  a  patient  in 
the  course  of  time  to  regain  his  speech  at  least  to  some  degree.  Aphasia 
alone  without  hemiplegia  may  be  entirely  recovered  from,  or  may 
remain  permanent  and  incurable.  In  56  cases  of  attacks  of  aphasia 
14  recovered  the  use  of  speech  completely. 

Hemiplegia,  even  if  total,  may  not  remain.  It  usually  passes  oflP  in 
part,  and  it  is  safe  to  predict  that  the  patient  will  walk  again,  even  in 
severe  cases.  In  one  case  under  my  care  the  patient  walked  well  at 
the  end  of  a  year,  though  he  had  made  no  voluntary  movement  what- 
ever in  arm  or  leg  at  the  end  of  three  months.  In  another  case  a  year 
elapsed  before  the  patient  could  stand  alone,  yet  two  years  later  he 

'Deut.  Zeitsch.  f.  Nervenh.,  Vol.  xxxiv.,  p.  6, 


PMOGNOSIS.  605 

walked  without  a  cane.  The  arm  and  hand  are  always  more  completely 
and  permanently  paralyzed  than  the  leg,  and  many  patients  who  can 
walk  well  never  regain  the  power  of  writing  or  of  feeding  themselves. 
Yet  I  have  seen  complete  return  of  all  but  the  very  finest  of  motions, 
such  as  the  power  to  pick  up  a  pin  or  to  button  the  clothing.  The 
increase  of  reflex  in  the  knee  and  ankle  is  not  an  unfavorable  sign. 
But  if  contracture  of  the  limbs  develops  after  the  first  month  a  complete 
recovery  never  takes  place.  In  the  series  of  200  cases  here  collected, 
of  177  patients  with  hemiplegia  22  recovered  completely,  and  in  every 
patient  who  lived  beyond  the  second  month  a  very  marked  improve- 
ment occurred.  The  sooner  the  return  of  any  motion  the  more  com- 
plete will  be  the  eventual  recovery.  The  paralysis  commonly  passes 
away  from  the  face  entirely,  so  that  only  on  very  careful  testing  is  a 
difference  in  the  two  sides  to  be  seen.  This  is  probably  because  even 
in  bad  cases  the  instinctive  movements  of  facial  expression  are  not 
affected,  excepting  when  the  optic  thalamus  is  destroyed,  and  the  con- 
trol of  the  face  is  bilateral  in  many  persons.  The  tongue  usually  re- 
covers with  the  face.  It  is  evident,  therefore,  that  a  promise  of  much 
improvement  in  the  power  of  motion  may  almost  always  be  given. 
Post-hemiplegic  athetosis  and  tremor  rarely  if  ever  subside  or  improve. 

The  prognosis  in  regard  to  sensory  disturbances  is  less  favorable 
than  in  regard  to  paralysis.  The  initial  hemiansesthesia  usually  sub- 
sides. But  when  it  persists  more  than  a  month  it  never  wholly  leaves 
the  patient,  and  it  is  usually  attended  by  a  feeling  of  tingling  and 
numbness  which  gives  rise  to  much  discomfort.  This  numbness  also 
makes  a  patient  awkward  in  his  movements,  even  when  it  is  not  asso- 
ciated with  paralysis. 

Hemianopsia  when  due  to  shock  or  to  pressure  on  the  visual  tract 
usually  passes  off  within  ten  days  or  two  weeks.  If  it  persists  beyond 
that  time  it  is  due  to  a  lesion  of  the  tract  or  cortex,  and  is  incurable. 
I  have  never  seen  the  symptom  of  hemianopsia  pass  away  when  it  had 
persisted  one  month.  The  limits  of  the  visual  field  may  extend  a  little 
and  the  patient  may  learn  in  time  to  disregard  and  even  to  forget  his 
blindness,  but  a  perimetric  examination  usually  shows  that  it  is 
stationary. 

The  irregular  motor  and  sensory  and  cranial  nerve  symptoms  due 
to  lesions  of  the  crura,  pons,  and  medulla,  when  they  are  not  followed 
soon  by  a  fatal  ending  are  usually  permanent  and  have  a  bad  prognosis. 
They  are  due  to  a  rupture  of  small  tracts  which  never  unite,  and  are 
followed  by  secondary  degenerations  which  are  permanent. 

The  cerebellar  symptoms  often  pass  away,  but  a  tendency  to  vertigo 
may  persist. 

The  mental  symptoms  attendant  upon  an  apoplexy  are  never  abso- 
lutely recovered  from,  and  after  each  attack  they  are  more  marked  and 
permanent.  When  they  are  due  to  the  underlying  endarteritis  it  is  to 
be  expected  that  they  will  be  even  worse  after  than  before  the  attack. 
The  actual  obscuration  of  ideas  following  the  attack,  the  inability  to 
comprehend,  to  think,  to  judge,  and  to  act  slowly  subside.     The  flow 


506  CEBEBBAL   DISEASES    OF    VASCULAR    ORIGIN. 

of  ideas  becomes  clearer,  and  if  there  is  no  aphasia  it  may  be  possible 
for  a  man  to  make  a  will  within  two  weeks  of  the  attack.  But  sub- 
sequently to  an  attack  the  mind  may  be  permanently  enfeebled,  and 
when  an  emotional  state  develops,  and  the  memory  is  easily  confused, 
and  the  eifort  of  continuous  talking  or  reading  causes  distress  for  two 
months  after  an  attack,  it  is  a  question  whether  it  will  ever  be  possible 
for  the  man  to  resume  his  occupation.  Yet  it  is  often  amazing  to  see 
how  accurately  a  business  man  can  judge  of  his  affairs,  and  how  clearly, 
even  though  aphasic,  he  can  indicate  his  desires  in  regard  to  important 
matters  while  severely  paralyzed.  In  the  series  of  200  cases,  mental 
symptoms  were  permanent  in  63.  But  in  several  cases  where  more 
than  two  slight  attacks  occurred  it  was  these  symptoms  which  remained 
and  which  rendered  the  patient  incompetent  after  the  aphasia  and 
hemiplegia  had  entirely  subsided. 

A  very  rapid  improvement  in  all  the  local  symptoms  after  an  apo- 
plectic attack  should  not  invariably  be  regarded  with  favor,  as  this  is 
the  history  in  attacks  occurring  in  cases  of  general  paresis.  Such  a 
recovery  should  awaken  a  suspicion  of  this  affection,  which,  if  found, 
will  greatly  change  the  prognosis. 

It  is  always  to  be  remembered  that,  excepting  in  children,  one 
attack  of  apoplexy  predisposes  to  another.  Endarteritis  is  a  steadily 
progressive  disease.  No  prognosis  can  safely  be  given  as  to  the  length 
of  the  interval  between  attacks,  but  a  second  attack  is  reasonably 
sure. 

The  prognosis  in  cases  due  to  syphilitic  disease  of  the  arteries  is  not 
more  favorable  than  in  other  cases ;  for  no  amount  of  antisyphilitic 
treatment  can  affect  the  condition  of  softening  due  to  a  thrombus  in  an 
artery  the  subject  of  obliterating  endarteritis.  It  is,  therefore,  a  mis- 
take to  give  a  good  prognosis  in  syphilitic  cases  or  to  promise  any  more 
from  treatment  in  such  cases  than  in  others.  In  fact  these  patients  are 
more  liable  to  recurrence  than  others,  and  hence  the  prognosis  is  worse. 
An  obliterating  endarteritis  is  not  to  be  cured.  My  experience  in  this 
respect  is  quite  the  reverse  of  that  of  von  Monakow.^  The  eventual 
prognosis  after  the  end  of  the  sixth  month  is  much  worse  in  cases  of 
embolism  and  thrombosis  than  in  hemorrhage.  For  in  the  former  the 
symptoms  then  present  show  the  permanent  effect  of  softening,  while 
in  the  latter  a  shrinkage  of  the  clot  and  its  scar  or  cyst  may  go  on  for 
two  years. 

Treatment.  —  For  a  person  who  has  beginning  endarteritis  it  is 
essential  to  adopt  a  manner  of  life  which  will  arrest  the  progress  of 
the  affection  and  prevent  its  most  serious  result.  It  is  generally  agreed 
that  a  diet  which  is  simple  in  character,  without  rich  sauces  and  strong 
condiments,  and  which  does  not  contain  an  excess  of  nitrogenous  matter, 
is  the  one  least  likely  to  increase  the  arterial  disease  Patients  there- 
fore should  not  eat  a  great  deal  of  meat,  eggs,  or  cheese,  and  many  are 
better  for  avoiding  milk.  It  is  most  important  to  avoid  alcohol  in  any 
form,  and  the  use  of  wine,  beer,  and  spirits  should  be  forbidden.  Strong 
1  Gehlrnpathologie ;  Spec.  Path.  u.  Ther.  von  Nothnagel,  Bd.  ix.,  S.  870. 


TBEATMENT.  607 

coffee  and  tobacco  are  also  supposed  to  induce  endarteritis,  and  should 
be  used  in  moderation  only.  A  proper  care  of  the  digestion  is  there- 
fore imperative.  An  annual  visit  to  Carlsbad  for  a  mild  course  of 
treatment,  or  to  Saratoga  for  a  corresponding  cure,  is  most  advantage- 
ous. The  habitual  use  of  water  both  with  and  between  meals  is  to  be 
urged.  A  fair  amount  of  exercise  not  of  an  exhausting  character  is  to 
be  commended,  golf  and  horseback  riding  being  advisable  as  equally 
good  means  of  out-of-door  exercise,  and  billiards  or  Indian-club  swing- 
ing with  light  clubs  the  best  indoor  forms.  Tennis,  or  lifting  weights, 
or  rowing  "are  to  be  avoided,  as  they  put  too  severe  a  strain  upon  the 
heart  for  a  person  with  endarteritis.  Exercise  should  cause  a  mild 
perspiration,  but  if  followed  by  exhaustion  or  palpitation,  is  objection- 
able. Many  persons  live  more  comfortably  without  exercise,  but  if  the 
lack  of  it  induces  obesity  it  must  be  taken.  Tepid  or  cool  baths  are 
preferable  to  very  hot  ones,  and  the  sudden  shock  of  cold  is  to  be 
avoided.  A  climate  should  be  sought  without  great  extremes  of  heat 
or  of  cold,  and  chills  should  be  guarded  against.  As  the  accurate 
observations  of  Carter  ^  have  proven  that  iodide  of  potassium  does  not 
decrease  pulse  tension,  I  see  no  reason  to  believe  that  its  continued  use 
in  small  doses,  which  is  advised  by  many  authors,  can  in  any  way  pre- 
vent arterial  disease.  But  a  person  who  has  had  syphilis  should 
certainly  take  a  course  of  mercury  every  three  years  and  a  course  of 
iodide  of  potassium  every  six  months,  to  prevent  the  development  of 
endarteritis. 

Patients  who  have  any  arterial  disease  should  avoid  sudden  exertion, 
and  this  is  a  good  rule  for  every  one  over  forty-five  years  to  follow,  as 
many  cases  of  apoplexy  occur  after  exertion  in  apparently  healthy  per- 
sons. The  existence  of  miliary  aneurisms  is  often  unsuspected  until 
they  rupture  after  a  sudden  effort.  Thus  one  of  my  friends,  a  healthy 
woman  of  fifty  years,  lifted  a  trunk  and  immediately  had  a  cerebral 
hemorrhage  which  killed  her.  It  is  just  as  imperative  in  persons  with 
endarteritis  to  avoid  exhausting  effort  as  it  is  for  one  who  has  heart 
disease.  And  mental  or  emotional  excitement  of  an  intense  kind  is 
equally  to  be  avoided.  Contentment,  with  a  moderate  degree  of  success 
and  wealth,  is  better  than  fame  or  riches  bought  by  an  apoplexy. 
These  precautions  should  be  particularly  urged  upon  patients  who  are 
known  to  have  nephritis. 

The  treatment  of  an  attack  of  apoplexy  depends  upon  the  cause,  as 
hemorrhage,  or  softening,  require  exactly  opposite  measures. 

If  it  is  apparent  that  a  hemorrhage  has  occurred  the  patient  should 
not  be  carried  fer,  or  jolted  in  the  process,  but  should  be  placed  in  bed 
with  his  head  somewhat  higher  than  usual,  and  should  lie  upon  the 
non-paralyzed  side  rather  than  upon  the  other,  or  upon  his  back.  Res- 
piration is  always  hampered  by  the  accumulation  of  saliva,  by  a  falling 
backward  of  tlie  larynx,  and  by  paralysis  of  the  respiratory  muscles  on 
one  side.  If  the  patient  lies  on  the  non-paralyzed  side  the  saliva  runs 
out,  the  larynx  does  not  become  obstructed,  and  the  weak  side  expands 
'American  Journal  of  the  Medical  Sciences,  1901,  vol.  cxxii.,  p.  854. 


608  Ct:nEMAL   DISEASES   OF    VASCULAB    ORIGIN. 

better  than  if  it  is  held  down  by  the  weight  of  the  body.^  If  gravita- 
tion acts  at  all  on  the  clot,  it  should  not  favor  its  rupture  into  the  ven- 
tricle, hence  the  side  of  the  brain'  which  is  injured  should  be  against 
the  pillow.  The  full,  beating  pulse  which  tends  to  increase  the  bleed- 
ing is  often  the  result  of  carbonic  acid  poisoning  from  imperfect  res- 
piration. All  obstructions  to  breathing,  tight  clothing  or  neck-bands 
should,  of  course,  be  removed.  The  mouth  should  be  wiped  out  fre- 
quently with  an  alkaline  antiseptic  solution.  All  exertion,  such  as 
swallowing,  and  the  consequent  coughing  which  inevitable  choking 
produces,  are  to  be  avoided,  hence  no  attempts  at  medication  by  the 
mouth  are  to  be  tried  during  the  coma.  Vomiting  is  always  dangerous 
to  the  patient,  from  the  strain  and  the  possibility  of  inhaling  particles. 
It  may  be  arrested  by  a  mustard  leaf  on  the  epigastrium.  If  the  face 
is  red  or  cyanotic,  the  pulse  full,  the  coma  deep,  the  temperature  low, 
the  respiration  labored,  venesection  is  indicated,  and  from  twelve  to 
eighteen  ounces  of  blood  should  be  taken  from  the  arm  at  once.  It 
will  do  no  good  unless  done  within  a  few  hours  of  the  attack.  In  hos- 
pital cases  I  have  seen  this  cause  great  relief,  and  believe  I  have  seen 
it  save  life.  It  should  be  employed  much  more  freely  than  it  is  in  cases 
cerebral  hemorrhage.  That  it  is  a  harmless  measure  is  shown  by  its 
general  use  in  the  last  century  for  all  sorts  of  diseases.  The  reaction 
against  its  abuse  has  led  to  its  neglect  in  cases  where  it  is  indicated. 
The  cases  in  which  it  does  harm  are  cases  of  weak  heart,  small  pulse, 
and  general  anaemia.  Placing  a  tight  bandage  around  the  limbs  near 
the  body  will  prevent  venous  blood  from  returning,  and  thus  take  some 
blood  from  the  brain,  and  this  is  a  means  to  be  used  when  bleeding 
cannot  be  done.  Mustard  paste  to  the  legs  or  back  of  the  neck  ac- 
complishes similar  results,  but  serious  blisters  are  too  often  caused  in 
states  of  unconsciousness  by  these  means  and  by  hot  bottles  to  make  their 
use  advisable.  Applications  of  ice  to  the  head  are  usually  ordered,  but 
it  is  questionable  if  they  do  good.  They  are,  however,  agreeable  to 
some  patients  when  consciousness  returns  and  there  is  pain  in  the  head  ; 
hence  it  is  possible  that  they  relieve  congestion.  No  attempt  should 
be  made  to  arouse  the  patient  from  the  coma  or  to  feed  him,  and  when 
he  begins  to  recover,  all  excitement,  the  presence  of  several  persons, 
and  attempts  to  talk,  should  be  prevented ;  and  quiet  in  a  darkened 
room  should  be  preserved.  When  it  seems  certain  that  a  hemorrhage 
is  occurring  the  use  of  adrenalin  hypodermically  or  by  the  rectum  is 
advisable.  It  is  also  well  to  cause  free  purgation  by  giving  two  drops 
of  croton  oil  in  a  little  butter.  This  put  on  the  tongue  will  be  absorbed 
in  the  mouth,  and  is  not  likely  to  cause  choking  if  swallowed.  Con- 
vulsions may  be  controlled  by  chloroform  or  by  chloral  hydrate  given 
by  rectum  m  twenty-grain  doses. 

When    the  patient  recovers    consciousness  his    anxiety  should    be 
quieted  by  reassurance  that  the  danger  is  over.     No  attempt  at  feed- 
ing should  be  made  until  he  feels  hungry,  and  then  cold  milk  only 
should  be  given  for  the  first  three  or  four  days.     If  there  is  dilSiculty 
'  E.  L.  Bowles,  Stertor  and  Apoplexy.     London,  1891. 


TREATMENT.  509 

in  swallowing  a  small  tube  may  be  introduced  through  the  nose,  a 
spray  of  cocaine  being  first  used  in  the  throat,  and  milk  given  through 
this.  Water  may  be  given  freely  if  desired,  and,  in  cases  of  nephritis, 
should  be  urged  upon  the  patient.  In  such  cases,  if  there  is  not  a  free 
excretion  of  urine,  an  enema  of  salt  solution  will  be  advisable.  Heart 
stimulants  should  be  avoided,  and  wine,  coffee,  or  tea  are  not  to  be 
used  unless  a  condition  of  weakness  of  the  heart  becomes  very  mani- 
fest. Aconite  in  small  doses  frequently  repeated  is  of  service,  as  it 
quiets  the  increased  activity  of  the  heart.  Hypodermic  injections  of 
camphor,  two  grains,  dissolved  in  thirty  minims  of  olive  oil  is  the  best 
heart  stimulant  if  one  becomes  necessary.  If  the  patient  is  very  rest- 
less bromide  of  sodium  in  thirty-grain  doses  every  six  hours  may  be 
given,  and  if  sleepless,  trional  in  fifteen-grain  doses  may  be  used. 
Tepid  sponge  baths  with  alcohol  are  very  quieting  and  refreshing. 
Great  care  should  be  taken  to  keep  the  body  clean.  The  bladder 
should  be  emptied  by  catheter  every  three  hours  to  prevent  soiling  of 
the  bed,  and  a  daily  enema  should  be  used  to  clean  out  the  rectum. 
Castor  oil,  or  aloes,  or  cascara  may  be  given  to  keep  the  bowels  open. 
Pads  of  oakum  or  cotton  may  be  used  to  prevent  pressure,  and  the 
position  must  be  changed  every  little  while  to  relieve  restlessness  and 
prevent  bed-sores.  An  air-bed  or  water-bed  is  rarely  necessary,  but 
may  give  comfort. 

It  has  long  been  the  custom  to  give  five  or  ten-grain  doses  of  iodide 
of  potassium  three  times  a  day  to  assist  the  absorption  of  a  clot. 
Those  who  believe  that  it  will  accomplish  any  result  may  continue 
to  use  it. 

If  it  is  apparent  that  the  stroke  of  apoplexy  is  due  to  an  embolism, 
or  a  thrombosis,  and  the  patient  is  pale  and  the  heart  is  weak,  he 
should  be  laid  flat  in  bed,  and  heart  stimulants  with  vasodilators  should 
be  used  at  once.  A  hypodermic  injection  of  nitroglycerin,  one  one- 
hundredth  grain  in  whiskey  or  cognac  should  be  given,  and  the  alco- 
holic stimulant  repeated  or  given  with  hot  water  as  an  enema.  Ammonia 
may  be  inhaled  and  Hoffman's  anodyne  may  be  used.  Heat  should 
be  applied  to  the  body,  but  the  measures  recommended  in  hemorrhage 
—  venesection,  purgation,  ice  to  the  head,  and  adrenalin  —  are  con- 
traindicated.  If  convulsions  occur  chloroform  may  be  used  or  chloral 
in  large  dose  by  enema.  Absolute  rest  should  be  enforced,  no  effort 
allowed  as  the  patient  recovers  consciousness,  and  then,  when  he  is 
able  to  swallow,  supporting  food,  mild  stimulation  with  coffee,  hot 
drinks,  milk,  and  beef  tea  should  be  given.  These  measures  are  of 
great  value  in  the  slight  prodromal  attacks  which  precede  a  stroke  in 
cases  of  endarteritis.  I  have  seen  very  marked  and  favorable  effects 
from  the  use  of  caffeine  citrate  or  the  sodium  salicylate  of  caffeine, 
three-grain  doses  every  six  hours.  If  the  pulse  tension  is  high  nitrite 
of  sodium  in  two-  or  three-grain  doses  every  four  hours  may  be  used,  as 
it  is  less  sudden  and  more  prolonged  in  effect  than  nitroglycerin.  It 
may  be  given  with  the  caffeine.  If  an  inflammatory  reaction  follows 
the  embolism,  and  the  temperature  rises,  and  restlessness  ^nd  insomnia 


510  CEEEBEAL   DISEASES    OF    VASCULAE    OEIGIN. 

follow,  the  caffeine  must  be  stopped,  uud  bromide  of  sodium  or  chloral 
hydrate  used.  The  latter  in  five-grain  doses  every  four  hours  is  of 
much  service  in  these  cases,  as  it  does  not  increase  the  arterial  tension 
and  yet  quiets  the  brain.  Trional  is  also  of  service  to  cause  sleep.  A 
rapid  relief  from  the  coma  not  infrequently  follows  this  treatment  in 
cases  of  embolism  or  thrombosis  as  the  collateral  circulation  is  stimu- 
lated. In  the  cases  where  the  temperature  rises  and  active  encepha- 
litis occurs,  ice  to  the  head,  antipyretics,  such  as  phenacetin,  acetanilid, 
cool  sponging,  and  laxatives  are  indicated,  but  medication  accomplishes 
little. 

When  it  is  impossible  to  make  an  accurate  diagnosis  between  hemor- 
rhage and  thrombosis  or  embolism  it  is  not  safe  to  employ  active  medi- 
cation directed  to  either  condition,  lest  the  damage  to  the  brain  may 
be  increased.  In  such  cases  it  is  better  to  secure  absolute  rest ;  to  aid 
the  respiration  by  position  on  one  side  ;  to  see  that  the  condition  of  the 
heart  and  pulse  is  favorable,  using  either  mild  sedatives  like  aconite  ; 
or  mild  stimulants  like  camphor  and  caffeine,  as  they  are  indicated  ;  to 
apply  ice  to  the  head,  and  if  there  is  much  restlessness  to  use-  bromides 
freely.  When  consciousness  returns  quiet  should  be  ensured,  a  mild 
light  fluid  diet  ordered,  plenty  of  water  given,  the  skin  and  bladder 
kept  in  order,  the  bowels  evacuated  by  enemata  daily,  and  sedatives, 
bromide  or  trional,  used  if  needed. 

In  cases  of  mild  onset  without  loss  of  consciousness  the  same  degree 
of  rest  is  to  be  secured  as  in  severe  cases,  and  for  at  least  two  or  three 
weeks  after  the  attack,  no  matter  how  slight,  no  physical  or  mental 
exertion  should  be  allowed.  A  light  diet,  plenty  of  water,  and  mild 
laxatives  should  be  used,  and  stimulants  avoided.  The  subsequent 
treatment  should  depend  largely  upon  the  condition  which  is  suspected 
or  determined.  As  the  majority  of  cases  of  slight  attack  are  due  to 
thrombosis  in  diseased  arteries  rather  than  to  hemorrhage,  vascular 
dilators  and  caffeine,  with  or  without  a  small  amount  of  digitalis,  or 
spartein  may  be  cautiously  tried,  and  if  found  to  be  of  benefit  used 
more  freely.  If  they  cause  headache  or  other  premonitory  symptoms 
they  must  be  discontinued.  In  cases  where  spasm  or  convulsive  move- 
ments have  occurred  the  use  of  bromide  is  indicated. 

In  all  cases  the  treatment  of  the  paralytic  condition  following  an 
apoplexy  is  really  expectant.  It  is  impossible  to  hasten  the  process 
of  repair  in  the  brain,  and  time  alone  can  bring  about  a  spontaneous 
recovery. 

During  the  two  or  three  weeks  after  the  attack  the  patient  must  not 
be  allowed  to  sit  up  or  to  make  any  effort  for  any  purpose.  He  must 
be  fed,  his  bladder  emptied  by  catheter,  the  bedpan  used  for  his  evacua- 
tions, and  he  must  not  turn  himself  in  bed  when  bathed.  Cold  appli- 
cations to  the  head  should  be  kept  up  for  a  week  or  longer  if  they  are 
agreeable  to  the  patient.  By  the  end  of  a  week  the  diet  may  be  in- 
creased and  easily  digested  solid  food  may  be  given.  A  variety  of 
fluids,  such  as  lemonade  and  mineral  waters,  may  be  given,  and  weak 
tea  and  coffee  may  be  allowed,  but  no  stimulants,  unless  the  case  is  one 


TBEATMENT.  511 

of  thrombosis.  The  position  of  the  patieut  must  be  made  as  comfort-i 
able  as  po»ssible  by  the  use  of  cushions  supporting  the  arm  and  leg,  and 
their  position  may  be  changed  often.  Bathing  with  warm  water  and 
alcohol  is  very  soothing,  and  may  be  done  twice  a  day.  No  medicine 
is  needed  during  this  stage  unless  it  is  some  sedative  at  night.  Con- 
stant encouragement  is  needed,  and  the  hope  of  recovery  must  be  stimu- 
lated. At  the  end  of  two  or  three  weeks  in  light  cases,  and  at  the  end 
of  four  or  five  weeks  in  medium  cases,  the  patient  begins  to  draw  the 
leg  up  in  bed  or  to  straighten  it  out  when  it  has  been  passively  flexed. 
After  such  motions  have  been  possible  for  a  week,  but  not  sooner,  the 
patient  may  be  lifted  to  a  chair  and  allowed  to  make  attempts  at  stand- 
ing with  the  aid  of  two  persons  or  one  person  and  a  crutch.  It  is  not 
well  for  a  patient  to  exert  himself  in  this  way  too  soon  or  too  long,  for 
fear  of  a  relapse.  And  it  does  not  hasten  his  recovery  to  allow  him  to 
try.     The  paralyzed  arm  should  be  supported  in  a  sling. 

The  cases  are  few  where  it  can  be  accurately  determined  that  there 
is  a  surface  hemorrhage  which  can  be  removed  by  surgical  interference. 
When  this  is  the  case  there  should  be  no  hesitation  in  undertaking  the 
operation.  Thus  in  a  case  in  which  McBurney  trephined  for  a  trau- 
matic hemorrhage  causing  aphasia  and  right  hemiplegia,  the  subsequent 
progressive  recovery  would  undoubtedly  have  been  impossible  had  the 
clot  not  been  removed.^  In  subcortical  hemorrhage  where  the  arterial 
tension  is  high  and  increasing  to  the  fatal  degree,  as  shown  by  the 
Riva  Rocci  apparatus,  the  cranium  may  be  opened  and  the  intracranial 
tension  relieved.  This  measure  is  not  to  be  undertaken,  however,  with- 
out good  reason.^ 

The  treatment  of  the  hemiplegia,  which  should  be  begun  at  the  end 
of  the  second  week,  is  by  massage  of  the  limbs  daily  or  twice  a  day 
for  fifteen  minutes  at  a  time,  by  passive  movements  of  the  limbs,  and 
by  the  application  of  faradism  to  exercise  the  muscles,  especially  the 
extensors  of  the  wrist,  the  muscles  moving  the  fingers,  and  the  flexors 
and  extensors  of  the  ankle  and  knee.  These  muscles  should  be  exer- 
cised for  a  minute  at  a  time,  and  the  entire  application  should  take  half 
an  hour.  The  facial  muscles  do  not  require  electrical  treatment,  as  the 
instinctive  movements  exercise  them.  It  should  be  distinctly  under- 
stood that  the  effect  of  electricity  is  limited  to  exercise.  The  use  of 
galvanism  to  the  head  has  long  since  been  abandoned  as  useless  and 
possibly  harmful.  Any  device  which  will  enable  the  patient  to  make 
voluntary  motions,  and  thus  exercise  the  muscles  by  act  of  will,  is  far 
more  useful  than  electricity.  Thus  as  soon  as  any  power  appears  in  any 
muscle,  the  patient  is  to  be  urged  to  use  it.  He  can  do  this  if  the 
action  of  the  opposing  muscle  be  performed  passively  or  by  a  mechani- 
cal device.  Thus  grasping  a  bulb  which  contains  a  spring  will  exer- 
cise the  flexors  of  the  fingers  while  the  extensors  are  still  unable  to 
open  the  hand  ;  pressing  on  the  pedal  of  a  sewing  machine  will  exercise 
weak  flexors  of  the  ankle  while  the  extensors  are  still  unable  to  lift 

'  Brain  Surgery,  Case  XVIII. 

''See  Harvey  (Jusliiiifr,  American  Journal  of  tlie  Medical  Sciences,  June,  1903. 


512  CEBEBBAL    DISEASES    OF    VASCULAR    ORIGIN. 

the  toes.  Such  devices  not  only  serve  to  exercise  the  muscles  but  also 
to  occupy  the  patient's  mind,  which  is  usually  devoted  wholly  to  the 
consideration  of  his  state.  Muscle  beating  helps  some  patients,  and 
may  be  combined  with  massage.  Warm  applications  followed  by  cool 
affusions  sometimes  stimulate  the  circulation  in  the  limb  and  help  the 
strength  ;  and  as  soon  as  walking  is  possible  it  should  be  allowed,  and 
stepping  over  obstacles,  and  careful  voluntary  motions  of  the  leg  should 
be  practiced,  and  the  attention  should  be  directed  to  the  act  of  walking 
so  as  to  prevent  a  hemiplegic  gait  from  being  acquired.  The  same 
training  as  has  been  used  by  Fraenkel  in  the  treatment  of  locomotor 
ataxia  is  very  useful  in  cases  of  hemiplegia.      (See  page  382.) 

The  hemiausesthesia  may  occasionally  be  benefited  within  a  week  by 
the  application  of  a  faradic  current  to  the  limbs,  the  faradic  brush  being 
preferred.  If  it  has  no  effect,  however,  within  a  month  of  the  onset 
there  is  no  treatment  which  will  restore  sensation,  and  faradism  usually 
increases  the  subjective  numbness  which  annoys  the  patient. 

The  hemianopsia  cannot  be  treated  in  any  way,  and  it  is  often  well 
not  to  call  the  patient's  attention  to  it,  as  little  by  little  it  is  disregarded. 
I  have  seen  a  number  of  patients  who  had  bilateral  homonymous  hemi- 
anopsia but  were  not  aware  of  the  fact. 

The  aphasia  is  open  to  treatment  by  education  in  some  cases,  but 
not  in  all.  Practice  in  reading,  in  reading 'aloud,  in  naming  objects 
and  in  writing  is  sometimes  successful  in  recalling  the  lost  memories 
and  powers  of  expression,  I  have  had  patients  who  learned  to  read 
again  by  the  use  of  the  letters  of  the  alphabet  printed  on  little  cards. 
I  have  known  patients  who  acquired  a  new  language  before  they 
relearned  their  own  tongue.  I  have  known  patients  who  could  sing 
before  they  could  talk.  I  have  known  patients  to  carry  about  a  little 
dictionary  and  to  find  in  it  the  word  they  could  recall  by  sight  but 
could  not  utter,  and  thus  to  convey  their  ideas.  If  no  attempts  at  reedu- 
cation are  successful  the  deaf-and-dumb  language  should  be  acquired, 
and  it  may  interest  the  patient  to  teach  him  this  from  the  outset,  show- 
ing him  large  printed  letters  and  letting  him  form  a  new  association  of 
ideas  between  them  and  the  position  assumed  by  the  hands.  If  hemi- 
plegia attends  the  aphasia  the  well  hand  may  be  used.  The  cerebellar 
symptoms  usually  subside  spontaneously,  but  obstinate  vertigo  can  some- 
times be  relieved  by  the  use  of  bromide. 

Post-hemiplegic  tremor  or  athetosis  is  not  benefited  by  any  form  of 
treatment. 

The  mental  symptoms  cannot  be  treated.  But  the  patients  can  be 
kept  from  excitement,  from  emotional  strain,  from  mental  effort,  and 
must  have  cheerful  surroundings  and  mild  forms  of  amusement.  It  is 
better  for  the  general  welfare  of  educated  and  active  persons  to  allow 
them  some  definite  mental  occupation,  and  if  new  interests  cannot  be 
started  it  is  better  to  permit  some  apparent  resumption  of  professional 
or  business  life,  and  visits  of  former  associates,  than  to  allow  them  to 
drift  into  a  state  of  mental  depression  over  their  apparently  useless 
condition.     Cheerful  companionship,  entertaining  reading  by  a  good 


TMEATM.ENT.  513 

reader,  games,  the  dictation  of  correspondence,  and  the  general  inter- 
ests of  lif(^  may  afford  sufficient  occupation.  And  eventually  travel 
may  be  allowed.  It  is  well  for  a  person  who  has  had  one  attack  to 
avoid  extremes  of  climate  and  high  altitudes.  A  winter  in  a  dry,  mild 
climate  like  that  of  Bermuda,  Nassau,  California,  the  Nile,  or  the 
Riviera,  and  a  summer  in  the  mountains  or  by  the  sea,  not  exposed  to 
great  heat,  will  prolong  life. 


33 


CHAPTER   XXVIII. 

THE  CEREBRAL  ATROPHIES  OF  CHILDHOOD. 

Infantile  Hemiplegia,    Diplegia,    Feeble-raindedness,    Imbecility,    Idiocy,    Blindness, 

Deaf-mutism,  Epilepsy. 

MALDEVELOPMENT  OF  THE  BRAIN. 

The  brain  may  be  injured  or  diseased  in  foetal  life,  or  at  the  time  of 
birth,  or  during  infancy,  and  in  consequence  may  fail  to  develop  in  a 
normal  manner.  When  this  occurs  a  condition  of  cerebral  atrophy  is 
found  after  death.  The  atrophy  may  be  limited  in  its  extent  to  a  few 
convolutions,  or  may  involve  one  lobe  of  the  brain,  or  may  affect  the 
entire  brain  in  greater  or  less  degree.  The  symptoms  produced  by 
cerebral  atrophy  are  numerous,  but  patients  are  easily  assigned  to  one 
of  three  groups  of  cases,  in  accordance  with  the  chief  feature  presented, 
though  in  some  patients  the  symptoms  of  all  the  groups  are  found. 

Clinical  Groups.  —  The  first  group  of  cases  is  characterized  by 
spastic  paralysis,  which  may  be  hemiplegic  or  diplegic  in  type.  In 
the  diplegic  form  both  legs  may  be  affected,  or  both  arms  as  well  as 
both  legs.  The  paralysis  is  often  accompanied  by  aphasia  and  by 
athetosis,  and  not  infrequently  by  sensory  defects,  by  imbecility,  and 
by  epilepsy. 

The  second  group  of  cases  is  characterized  by  mental  defects  of 
greater  or  less  degree.  These  may  vary  from  absolute  idiocy  to 
feeble-mindedness,  or  the  patient  may  show  merely  some  peculiarity  of 
conduct,  indicating  a  lack  of  the  highest  powers  of  attention,  and  of 
reasoning,  and  of  self-control.     Epilepsy  may  develop  in  these  cases. 

The  third  group  of  cases  is  characterized  by  sensory  defects :  either 
hemianopsia,  or  total  blindness,  or  deafness,  and  consequent  mutism. 
In  these  cases  also  imbecility  and  epilepsy  may  develop. 

Frequency.  —  Cerebral  atrophy  is  not  rare.  I  have  records  of  400 
cases.  Of  these  274  belonged  to  the  first  group,  219  having  hemi- 
plegia, and  55  diplegia.  Among  these  patients  100  were  aphasic;  89 
had  athetosis,  and  90  had  epilepsy.  In  120  some  degree  of  mental 
defect  was  apparent.  To  the  second  group  111  patients  could  be 
assigned,  as  the  mental  defects  were  more  marked  than  other  symp- 
toms. Sixty  of  the  second  group  were  epileptics.  To  the  third  group 
15  patients  could  be  assigned,  7  being  blind,  3  having  hemianopsia, 
and  5  being  totally  deaf  from  birth.  Six  of  these  patients  were 
epileptics,  but  none  was  imbecile. 

Etiology.  —  In  some  cases  prenatal  influences  are  responsible  for 
the  maldevelopment  of  the  brain.     Osier  reports  a  case  of  hemorrhage 

514 


PATHOLOGY.  515 

found  lu  ^le  brain  of  a  foetus,  the  mother  having  died  of  typhoid  fever  at 
the  sixth  month  of  pregnancy.  Cotard  has  reported  a  similar  condition, 
the  mother  having  received  an  injury.  Any  severe  disease  in  the  mother 
may  affect  the  development  of  the  foetal  brain.  Syphilis  in  either  parent 
may  prevent  a  proper  evolution  of  the  child's  nervous  system.  Sachs 
holds  that  the  frequency  with  which  infants  of  neurotic,  epileptic,  alcoholic, 
and  insane  parents  present  cerebral  defects  is  proof  that  prenatal  influences 
are  potent  causes  of  cerebral  palsy  and  idiocy.  It  has  been  thought 
that  cerebral  thrombosis  and  encephalitis  may  occur  in  the  foetus. 

Many  cases  date  from  birth.  In  these  some  evidence  of  traumatism 
can  usually  be  obtained.  Thus  in  177  of  my  400  cases  there  was  a 
history  of  difficult  labor,  either  a  long  protracted  labor,  or  a  malposi- 
tion requiring  manual  delivery,  or  instrumental  delivery.  In  several 
cases  labor  was  premature,  and  the  child  was  kept  alive  with  difficulty. 
In  two  cases  the  patient  was  a  twin.  In  many  cases  the  child  was  born 
asphyxiated.  In  all  these  cases  it  is  probable  that  meningeal  or  cere- 
bral hemorrhage  occurred  during  delivery,  and  that  the  pressure  upon 
the  brain  or  some  injury  to  it  prevented  a  normal  development. 

In  some  cases  the  symptoms  appear  suddenly  in  a  normal  infant 
during  the  early  years  of  life,  the  first  three  years  being  the  period  of 
greatest  ability.  Among  my  patients  eighty-two  developed  symptoms 
during  the  first  year,  fifty-eight  during  the  second  year,  twenty-eight 
during  the  third  year,  the  remainder  later.  Some  cases  are  traumatic. 
Twenty-two  of  my  patients  had  suffered  from  severe  falls  during  in- 
fancy. Other  cases  were  due  to  cerebral  hemorrhage,  embolism,  or 
thrombosis,  or  to  an  acute  encephalitis.  Any  of  these  conditions  may 
occur  as  a  complication  of  the  infectious  diseases  of  childhood,  and 
hence  cerebral  palsies  or  idiocy  m.ay  date  from  an  attack  of  measles, 
diphtheria,  whooping-cough,  cerebro-spinal  meningitis,  pneumonia,  scar- 
let fever,  or  typhoid  fever.  There  was  a  history  of  one  of  these  dis- 
eases in  twenty  of  my  patients.  Meningitis  and  hydrocephalus  are 
common  causes.  In  a  great  many  cases  the  occurrence  of  a  convulsion 
is  the  cause  assigned  ;  but  whether  the  convulsion  is  the  first  symptom 
of  the  lesion,  or  whether  in  a  convulsion  some  vascular  rupture  causes 
a  lesion  of  the  brain,  is  never  a  matter  of  certainty.  Forty  per  cent, 
of  my  patients  had  an  initial  convulsion. 

There  are  also  cases  in  which  no  cause  can  be  found.  An  arrest  of 
development  of  the  brain  may  occur  at  any  time  during  the  first  twenty 
years  of  life,  and  then  produce  symptoms. 

Pathology.  —  Records  of  the  pathological  condition  found  in  all  the 
three  groups  of  cases  described  have  been  compiled,  collections  of  cases 
with  autopsies  having  been  made  by  numerous  writers. 

The  lesions  found  are  various  in  kind,  in  origin,  and  in  situation, 
but  a  careful  study  elicits  two  conclusions  :  First  the  difference  in  the 
clinical  types  is  due  to  the  varying  situation  of  the  lesion  rather  than 
to  its  varying  nature  ;  secondly,  tliat  the  various  processes  of  disease 
have,  as  a  fairly  uniform  result,  a  condition  of  atrophy  with  sclerosis 
of  the  brain,  which  we  may  terra  sclerotic  atrophy. 


516 


THE  CEBEBRAL   ATEOPHIES    OF   CHILDHOOD. 


In  the  first  clinical  type  the  sclerotic  atrophy  involves  the  motor 
area  of  the  brain  —  i.  e.,  the  central  convolutions  bordering  the  fissure 
of  Rolando  and  the  cortex  of  their  immediate  vicinity,  and  involves 
also  the  motor  tract  arising  from  this  part  of  the  cortex,  and  usually 
the  basal  ganglia  as  well.  (See  Fig.  226.)  Sometimes  the  lesion  is 
confined  to  the  basal  ganglia.  In  the  second  type  the  sclerotic  atrophy 
involves  the  anterior  portion  of  the  brain,  and  sometimes  the  entire 


Fig.  226. 


Sclerotic  atrophy  of  the  cortex  supplied  by  the  middle  cerebral  artery  in  a  case  of  diplegia, 
idiocy,  and  epilepsy.    Both  hemispheres  were  affected.     (Peterson.) 

hemisphere  to  a  greater  or  less  extent.  (See  Fig.  227.)  In  the  third 
type  the  sclerotic  atrophy  involves  the  posterior  and  lateral  parts  of 
the  hemispheres. 

It  is  not  surprising  that  the  variation  in  the  situation  should  pro- 
duce varying  symptoms  in  view  of  the  facts  of  the  localization  of  brain- 
functions  already  stated.  That  there  should  be  a  limitation  of  the 
sclerotic  atrophy  to  certain  lobes  or  regions  —  to  the  frontal,  or  central, 
or  occipital,  or  parieto-temporal  regions  —  in  various  cases,  has  led  to 
the  conclusion  that  the  origin  of  the  disease  lay  in  some  interference 
with  the  blood  supply  of  the  part  affected,  since  it  has  often  been  evi- 
dent that  the  atrophy  was  limited  to  the  region  nourished  by  blood 
reaching  it  through  one  arterial  trunk.  And  this  theory  has  been 
rightly  supposed  to  explain  the  pathogenesis  of  these  cases.  Whether 
thrombosis  or  hemorrhage  has  occurred  is  not  always  easy  to  determine. 

It  has  been  stated  that  the  various  processes  of  disease  have,  as  a 
uniform  result,  a  condition  of  sclerotic  atrophy.  This  fact  is  borne  out 
by  the  following  resume  of  pathological  findings  in  343  cases  which 
have  been  gathered  from  the  records  of  Kundrat,  Audry,  Wallenberg, 
Osier,  Wilmarth,  Fere,  Henoch,  Hirt,  Fowler,  Schultze,  Sachs,  Rich- 
ardiere,  Bourneville,  Fisher,  and  from  recent  American  and  foreign 
journals.     The  conditions  found  were  as  follows  : 


MALDEFELOFMENT    OF    THE   BBAIN. 


517 


Porencephalus,  a  localized  atrophy  or  agenesis,  leaving  a  cavity  in 
the  cerebi^l  hemisphere,  which  may  be  deep  enough  to  open  into  the 
ventricle,  132  cases.     Figs.  228  and  229  show  this  condition. 


Fig.  227. 


Congenital  maldevelopment  of  the  right  hemisphere  of  the  brain,  with  sclerotic  atrophy.     (Bailey.) 

Sclerotic  atrophy,  an  atrophic  condition  of  the  brain  with  an  increase 
of  connective  tissue  and  disappearance  of  the  nervous  elements  ;  affect- 
ing both  hemispheres,  or  one  only,  or  a  part  of  one  only,  or  limited  to 
small  areas  in  various  parts,  97  cases.  This  is  the  terminal  result  of 
encephalitis  (q.  v.)  and  often  results  from  maldevelopment  from  unknown 
causes  acting  on  the  foetus. 

Maldevelopment  and  apparent  atrophic  condition  of  the  minute 
structures  of  the  hemisphere,  chiefly  cortical,  the  cells  resembling  those 
of  a  newborn  child,  but  with  no  apparent  gross  defects  in  the  brain, 
32  cases. 

Atrophy,  consequent  upon  the  condition  of  softening  produced  by 
embolism  or  thrombosis,  and  limited  in  extent  to  certain  arterial  dis- 
tricts of  the  brain,  23  cases.     Fig.  226  shows  this  condition. 

Meningo- encephalitis,  a  condition  shown  by  thickening  and  adhesion 
between  the  pia  and  the  brain,  with  destruction  of  the  cerebral  cells 
and  atrophy  of  the  cortex,  21  cases. 

Cysts  lying  on  the  brain  and  producing  atrophy  by  pressure,  or 
associated  with  atrophy  due  to  the  original  lesion  of  which  the  cyst 


5i8 


TEE   CEBEBBAL   ATROPHIES    OF   CIIILDHOOD. 


remains  as  a  trace,  14  cases.     Figs.  231  and  232    show  this  condi- 
tion. 

Hemorrhage  on  or  in  the  brain,  as  shown  by  the  remains  of  a  clot, 
or  by  hseraatin  staining  of  a  cyst,  of  the  pia,  or  of  the  sclerotic  tissue, 

18  cases. 

Fig.  228. 


PoTcncephalus.     (Lloyd.) 

Fig.  229. 


Frontal  section  through  a  porencephalic  brain.  The  left  hemisphere  is  normal.  The  right  hemi- 
sphere is  atrophied  in  lo/o,  and  has  a  cavity  in  the  motor  region  which  extends  downward  Into  the 
ventricle.    The  basal  ganglia  are  atrophied.    This  condition  Is  always  congenital.     (Shattenberg.) 

Hydrocephalus  with  extreme  dilatation  of  the  ventricles,  so  that  the 
brain  tissue  is  reduced  to  a  mere  wall  about  the  cavity,  five  cases. 


MALDEVELOPMENT    OF    THE    BRAIN. 


519 


Hydrocephalus  is  an  accumulation  of  serum  in  large  amount  in  the 
cranium.  If  the  serum  collects  in  the  meninges  it  compresses  the 
brain  and  is  associated  with  atrophy.     This  is  termed  external  hydro- 


FiG.  230. 


t/ppercavofy' 


Fossa  Sylvil 


J-ovrer- pari 
of  Ccuvvfy 


^     -Lower part 
t|    ofCcwUy 


Lower  horn, 
ofyintricie 

Extensive  bilateral  porencephalus.    The  cavity  within  the  brain  communicated  with  the 
external  surface  at  A.     (Richter,  Arch.  f.  Psych.,  xxxii.) 


Fig.  231. 


Superior  surface  of  a  brain  of  a  congenital  iiuljccile  who  had  hcmii>lcgia  and  epilepsy.  The  entire 
right  liemispherc  is  atroi)hiod.  C  is  the  arachnoid,  which  was  thickened  and  fonucd  thq  wall  of  a  cystic 
cavity  in  the  hemisphere.     (Fcrraro.) 


520 


THE  CEBEBBAL  ATBOPEIES   OF   CHILDHOOD. 


cephalus.  It  is  a  rare  condition,  secondary  to  meningitis  or  to  mal- 
development  of  the  brain,  and  not  infrequently  the  fluid  is  poured  out 
to  fill  the  space  left  between  an  atrophic  brain  and  the  cranium.  The 
common  form  of  hydrocephalus  is  inter aal  hydrooephalus,  that  is  a  dis- 
tention of  the  ventricles  of  the  brain  by  an  exudation  of  serum  into 
them. 

Internal  hydrocephalus  may  be  acute  or  chronic.  The  acute  form 
is  always  due  to  meningitis,  either  simple  or  tuberculous  (see  Chapter 
XLI,).  The  chronic  form  may  be  secondary  to  a  mild  meningitis  or 
ependymitis,  the  original  disease  resulting  in  an  adhesion  within  the 
ventricles,  which  closes  one  of  the  foramina  and  prevents  the  free 

Fig.  232. 


Superior  surface  of  the  brain  of  a  congenital  imbecile.  The  arachnoid  being  removed,  the  poren- 
cephalic cavity  is  displayed.  The  cortex  is  wholly  defective  over  the  upper  frontal  and  parietal  lobes, 
and  the  cavity  in  the  hemisphere  opens  into  the  lateral  ventricle  at  D,  in  which  the  choroid  plexus  E 
is  seen.     (Ferraro.) 


flow  of  serum  from  the  lateral  ventricles  outward  through  the  aqueduct 
of  Sylvius  and  the  fourth  ventricle  and  the  foramen  of  Magendie  into 
the  space  about  the  brain.  It  may  also  be  secondary  to  any  disease 
in  or  outside  of  the  brain  which  compresses  the  base  and  obstructs  the 
free  exit  of  serum  from  the  ventricles.  Thus  tumors  of  the  brain 
usually  cause  hydrocephalus.  It  may  also  be  secondary  to  syphilitic 
disease  in  the  brain  or  meninges,  and  many  congenital  cases  are  due 
to  inherited  syphilis,  with  closure  of  the  aqueduct  or  foramina.  Chronic 
internal  hydrocephalus  is  in  many  cases  a  congenital  condition,  and  is 


MALDEVELOPMENT    OF    THE   BHAIN. 


521 


very  freqi^gntly  associated  with  rickets.  It  is  considered  a  primary 
condition  in  cases  wliere  no  cause  can  be  ascertained,  and  it  is  in  these 
cases  that  the  most  extreme  distention  of  the  brain  is  observed.  The 
fluid  appears  to  be  secreted  by  the  ependyma  of  the  lateral  ventricles, 
and  may  vary  in  amount  from  a  few  ounces  up  to  five  or  six  pints.  It 
is  identical  with  the  cerebrospinal  fluid.  It  gradually  distends  the 
ventricles,  dilates  the  passages  between  them,  and  compresses  the  brain. 
.The  brain  becomes  anaemic  and  atrophies,  so  that  finally  a  mere,  thin 
capsule  of  brain  tissue  is  left  about  the  ventricles.  The  gradual  com- 
pression and  atrophy  of  the  brain  interfere  with  the  function  of  the 
tracts  passing  through  it,  and   hence  gives  rise   to  spastic  diplegia,  to 


Fig.  233. 


Congenital  absence  of  one-half  of  the  cerebellum.    Atrophy  of  the  opposite  olivary  body  and 
of  the  superficial  transverse  fibres  of  the  pons.     (Kindness  of  Dr.  Oliver  S.  Strong.) 

sensory  defects,  to  mental  deterioration,  and  imbecility.  The  disten- 
tion of  the  brain  causes  a  distention  of  the  skull,  giving  rise  to  the 
characteristic  hydrocephalic  skull,  in  which  the  sutures  are  separated, 
the  frontal  and  parietal  bosses  bulge  outward,  and  the  head  is  of  enor- 
mous size.  The  softening  of  the  bones  may  be  the  earliest  sign  of  a 
beginning  hydrocephalus.  Holt  states  that  the  average  head  at  birth 
measures  14  inches  in  circumference,  and  at  one  year  measures  18  to 
19  inches.  A  rapid  enlargement  beyond  these  limits  during  infancy 
is  suggestive  of  hydrocephalus.  The  enlargement  may  progress  slowly 
for  several  years,  or  may  cease  at  any  time,  spontaneous  arrest  of  the 


52^  THE  cxsi:^nAL  ATHovmns  OV  cmLDSOOB, 

condition  being  observed  in  about  six  per  cent,  of  the  patients.  Kinety- 
four  per  cent,  of  these  patients  die  before  the  age  of  seven  years. 
Attempts  to  drain  the  ventricles  do  not  result  in  an  arrest  of  the  secre- 
tion, and  hence  are  almost  uniformly  unsuccessful.' 

Unilateral  hydrocephalus  was  found  in  one  case  in  the  pathological 
records. 

It  is  evident  that  the  common  condition  in  all  these  cases  was  an 
atrophy  of  the  brain. 


Table  XII.     Measurements 

OF  THE  Skull. 

(Peterson.) 

Average  of  normal 

Limits  of  Physiological 

male  skull. 

variation. 

Circumference     .... 

52.0  cm. 

48.5  to 

57.4  cm. 

Volume  (rou.2:h  approximation) 

1500.0    " 

1201.0  " 

1751.0  " 

Nasooccipital  arc 

32.0    " 

28.0  " 

38.0  " 

Nasobregmatic  arc      . 

12.5    " 

10.9  " 

14.9  " 

Bregmato-Iambdoid  arc 

12.5    " 

9.1   " 

14.4  " 

Binauricular  arc 

32.0    " 

28.4  " 

35.0  " 

Antero-posterior  diameter  . 

17.7    " 

16.5  " 

19.0  " 

Greatest  transverse  diameter 

14.6    " 

13.0  " 

16.5  " 

Length-breadth  index 

82.2    " 

76.1   " 

87.0  " 

Binauricular  diameter 

12.4    " 

10.9  " 

13.9  " 

Facial  length      .... 

12.37  " 

10.5  " 

14.4  " 

The  maldevelopment  of  the  brain  is  usually  accompanied  by  a  state 
of  microcephalus,  all  the  measurements  of  the  skull  being  reduced  as 
compared  with  a  normal  head.  In  many  such  children  great  asym- 
metry of  the  skull  is  found.  In  other  cases  the  head  is  unusually 
large.  A  large  head  may  contain  a  microcephalic  brain,  the  space 
about  it  being  filled  with  fluid.  The  asymmetry  becomes  more  notice- 
able as  the  child  grows  up.  The  table  on  page  518  shows  the  measure- 
ments of  the  average  normal  skull  and  the  limits  of  physiological  varia- 
tion in  an  adult.      In  cases  of  cerebral  atrophy  these  limits  are  passed. 

1.  Symptoms  of  Cerebral  Spastic  Paralysis.  —  The  first  group 
of  cases  present  symptoms  of  paralysis.  This  may  be  unilateral  or 
bilateral. 

Hemiplegia.  —  In  the  cases  which  develop  a  unilateral  paralysis  the 
characteristic  features  of  hemiplegia  as  seen  in  adults  are  evident.  This 
may  date  from  birth  or  it  may  develop  later.  If  it  develops  in  infancy 
it  usually  begins  with  a  series  of  convulsions,  which  are  often  general, 
but  may  be  unilateral,  and  are  attended  by  high  fever,  102°  to  105° 
F.,  and  nausea,  vomiting,  and  headache,  with  delirium.  The  onset  is 
followed  by  a  period  of  unconsciousness  of  varying  duration,  from  an  hour 
to  two  or  three  days,  the  average  being  twelve  hours.  The  child  may 
die  in  such  an  attack.  But  there  is  usually  a  gradual  improvement  in 
the  paralysis  after  the  active  manifestations  of  the  onset  have  subsided ; 
and,  finally,  a  stationary  condition  remains,  in  which  the  face  is  but 
slightly  affected  in  its  voluntary  or  automatic  movements  ;  the  speech 
is  acquired  very  slowly,  or  is  slowly  regained,  if  it  had  been  lost ;  the 
arm  is  quite  seriously  paralyzed,  the  fingers  being  stiff*  and  awkward, 
and  sometimes  being  in  constant  slow  involuntary  motion  which  is 

J  See  Brain  Surgery,  Chapter  VII. 


MALDEVELOFMENT    OF    THE   BBAIN. 


523 


called  athetosis ;  the  leg  is  weak,  and  the  knee  and  ankle  are  held 
rigid,  so  that  the  child  limps  in  walking,  and  sometimes  has  a  club- 
foot. A  slow  improvement  is  the  rule.  As  time  goes  on  the  child 
learns  to  help  himself  in  many  ways,  and  it  is  possible  for  the  gradual 


Fig.  234. 


Fig.  235. 


Hemiplegia  with  contractures.    The  patient  had  suf- 
fered since  the  age  of  two  years.     (Curschmann.) 


Spastic  hemiplegia  from  angiolithic 
sarcoma  of  the  brain,  the  symptoms 
dating  from  five  years  of  age.  (Der- 
cum.) 


growth  of  the  limbs  to  be  attended  by  increasing  power.  In  all  cases 
the  paralyzed  limbs  are  found  to  be  affected  in  their  growth  and  de- 
velopment, so  that  they  are  smaller,  colder,  stiffer,  and  weaker  than 
the  others  ;  they  are  often  blue  and  cyanotic.  The  reflexes  are  exag- 
gerated on  the  paralyzed  side.  The  electrical  reactions  are  not  quali- 
tatively changed.  The  sensation  is  normal.  This  condition  remains 
through  life  as  a  permanent  defect,  and  although  the  division  of  con- 
tractured  muscles  or  tendons,  and  the  application  of  ingenious  apparatus, 
may  correct  deformities  and  make  the  paralyzed  parts  fairly  useful,  and 
although  the  application  of  electricity  to  the  muscles  chiefly  affected 


624  THM  CEHEBMAL  ATMOPEIES   OF  CHILDHOOD. 

may  increase  their  nutrition,  and  thus  prevent  contractures  which  come 
from  the  unbalanced  strain  between  the  various  muscles,  yet  a  perfect 
recovery  does  not  occur.  The  degree  of  spontaneous  recovery  possible 
in  any  case  cannot  be  determined  for  several  years.  But  in  many  cases 
while  some  trace  of  the  hemiplegia  remains  there  is  so  much  improve- 
ment that  the  patient  is  capable  of  earning  his  living.  I  have  several 
such  patients  who,  though  unable  to  use  the  hand  freely,  and  always 
obliged  to  limp  in  walking,  are  able  to  occupy  places  as  clerks  or  mes- 
sengers, or  have  been  able  to  go  through  college,  and  even  to  study  a 
profession.  It  is  only  in  the  cases  in  which  weak-mindedness  and  epi- 
lepsy occur  that  the  condition  is  a  hopeless  one.  But  even  when  the 
final  state  is  not  one  of  helplessness  these  patients  are  extremely 
liable  to  other  nervous  diseases.  Thus  several  such  persons  under  my, 
•  observation  have  suffered  from  chronic  neurasthenia  and  hysteria.  One 
of  them  developed  attacks  of  mania  of  short  duration,  but  frequent 
recurrence,  not  unlike  the  psychical  epileptic  equivalent,  and,  though 
an  educated  lawyer,  has  required  asylum  care  for  several  years  past.  ^ 

Spastic  Rigidity;  Athetosis. — The  especial  characteristics  of  this 
form  of  hemiplegia  are  the  spastic  rigidity  of  the  affected  limbs  with 
marked  contracture,  and  the  athetosis.  The  contractures  lead  to  vari- 
ous deformities  about  the  ankle  and  wrist,  especially  to  club-foot.  Fig. 
234  shows  these  conditions.  Athetosis  may  occur  when  the  hand  or 
foot  are  quiet.  When  the  child  attempts  to  move  the  hand  or  leg  un- 
expectedly involuntary  slow  motions  occur  which  prevent  the  intended 
result,  or  the  limb  is  thrown  into  a  sudden  state  of  rigid  immobility. 
Athetoid  movements  on  the  paralyzed  side  are  set  up  by  any  voluntary 
act  on  the  well  side.  These  athetoid  motions  begin  soon  after  the  onset 
as  a  rule.  In  one  case  of  birth  palsy  they  did  not  appear  until  the 
fourth  year  and  increased  until  the  seventh  year,  remaining  when  I  saw 
the  child  at  the  age  of  twelve  years.  If  the  athetoid  movements  affect 
the  face  and  tongue,  grimaces  are  constant  and  speech  is  interfered 
with.  Athetosis  has  been  present  in  30  per  cent,  of  my  cases.  (See 
Fig.  225.) 

In  some  cases  the  athetosis  is  the  most  marked  symptom.  Thus  in 
the  case  of  a  boy,  aged  eleven  years,  who  had  been  born  asphyxiated 
after  a  face  presentation,  and  was  revived  with  difficulty,  a  peculiar 
type  of  athetosis  had  been  present  since  the  age  of  ten  months.  The 
motion  was  limited  to  the  left  side  and  was  present  in  the  face,  in  the 
hand,  and  in  the  foot.  It  was  not  constant  in  this  case,  nor  was  it 
started  by  any  effort.  But  it  came  in  attacks,  twelve  or  more  in  a  day, 
each  lasting  several  minutes.  The  motion  was  a  quick,  involuntary 
athetoid  movement,  which  he  could  not  control.  It  was  not  attended 
by  a  loss  of  consciousness,  or  by  a  sense  of  alarm,  or  by  any  numbness. 
He  had  no  paralysis  whatever,  no  nystagmus,  no  disturbance  of  vision, 
no  headaches,  and  was  bright  and  active  mentally.  The  attacks  were 
first  noted  during  sleep,  but  as  he  grew  older  became  more  frequent  in 
the  daytime,  and  no  treatment  had  any  effect  upon  them. 

In  another  case,  also  dating  from  birth,  the  athetosis  affected  the 


MALDEVELOPMENT    OF    THE    BBAIN.  525 

entire  body,  so  that  any  effort  was  attended  by  contortions  of  the  face 
and  by  peduliar  crowing  sounds  which  interfered  with  talking  and  eat- 
ing ;  by  constant  violent  movements  of  the  arms  and  legs,  so  that  no 
voluntary  act  could  be  performed,  and  walking  was  impossible.  The 
boy,  at  the  age  often  years,  was  perfectly  intelligent  and  not  paralyzed, 
but  was  in  a  constant  state  of  active  motion  without  any  volitional 
power.  It  was  impossible  for  him'  to  sit  upright  in  a  chair  or  to  stand. 
He  could  only  lie  in  bed  supported  by  pillows,  and  was  even  fed  with 
difficulty.  The  athetosis  ceased  during  sleep.  I  have  recently  seen  a 
second  similar  case  equally  distressing  in  a  young  man,  aged  eighteen 
years,  whose  entire  life  had  been  spent  in  bed. 

Choreic  movements  more  sudden,  rapid  and  violent  than  athetosis 
may  appear  in  the  paralyzed  limbs  and  remain  as  a  permanent  symp- 
tom. Such  a  condition  was  noticed  in  twenty-one  of  my  two  hundred 
and  seventy -four  cases. 

In  one  of  my  cases  there  was  a  total  paralysis  of  the  tongue  with 
right  hemiplegia,  and  the  child  at  the  age  of  twelve  years  was  still 
unable  to  articulate.  Food  was  pushed  back  into  the  throat  by  the 
finger,  and  then  swallowed.  This  child  had  learned  to  read,  but  was 
weak  mentally  and  epileptic. 

Aphasia  is  a  common  accompaniment  of  right  hemiplegia.  In  my 
274  patients,  130  had  right  hemiplegia,  and  in  100  of  these  aphasia 
was  present.  It  is  usually  of  the  motor  type.  The  children  under- 
stand what  is  said  to  them,  can  acquire  a  knowledge  of  the  names  of 
objects,  and  may  learn  to  read,  but  cannot  be  taught  to  talk,  or  if  they 
acquire  language,  do  so  very  slowly  and  imperfectly.  I  have  known 
children  of  the  age  of  twelve  years  to  talk  like  a  child  of  three  years. 
There  is,  however,  as  a  rule,  gradual  recovery  of  speech,  and  it  seems 
likely  that  in  these  cases  the  right  hemisphere  takes  the  place  of  the 
left  in  developing  this  function.  This  is  often  aided  by  training  the 
child  to  write  with  the  left  hand. 

Diplegia  —  Little's  Disease.  —  In  many  cases  dating  from  birth  a  con- 
dition of  bilateral  paralysis  of  arms  and  legs,  or  of  the  legs  alone,  is 
found  as  soon  as  the  child  is  born.  This  is  termed  diplegia.  It  becomes 
more  apparent  after  the  first  few  weeks  when  attempts  at  voluntary 
motion  are  made.  It  is  attended  by  a  rigidity  of  the  muscles  which 
is  noticed  in  the  legs,  and  there  is  an  increase  of  the  reflexes.  The 
infant  may  have  difficulty  in  sucking  and  may  have  to  be  fed  with  a 
spoon. 

Diplegia  may  however  develop  slowly,  and  not  become  apparent  for 
some  months.  In  some  cases,  especially  after  the  infectious  diseases, 
it  may  not  appear  till  the  age  of  three  years.  In  these  cases  as  the 
baby  grows  its  body  is  found  to  be  stiff,  especially  in  its  back  and  legs, 
the  latter  tending  to  be  adducted  and  extended.  This  is  noticed  in 
adjusting  the  diapers.  Later  when  attempts  at  walking  are  made  the 
adduction  and  inward  rotation  of  the  thighs  may  cause  an  overlapping 
of  the  knees  and  feet,  there  may  develop  a  pointing  of  the  toes  which  . 
gradually  becomes  a  fixed  equino-varus,  so  that  the  heels  can  not  be 


526 


TEE  CEBEBRAL  ATEOPEIES   OF   CHILDHOOD. 


put  on  the  ground,  and  the  inability  of  the  child  to  balance  becomes 
more  noticeable.  Any  attempt  at  standing  increases  the  stiffness  of 
the  back  and  legs.  Sometimes  the  back  is  so  rigid  that  the  child  can- 
not sit  up  on  a  chair,  but  has  to  be  kept  in  a  lying  position.  As  the 
child  grows  the  condition  becomes  more  noticeable,  and  sometimes  the 
child  is  bedridden  and  never  learns  to  walk.      In  other  cases  the  child 


Fig.  236. 


Fig.  237. 


Marked  spastic  paraplegia  ;  walking  or  stand- 
ing alone  impossible.     (Dercum.) 


Spastic  paraplegia ;  crossed-legged 
progression.     (Dercum.) 


acquires  a  spastic  gait  and  helps  itself  by  its  hands,  as  they  are  often 
not  affected,  and  so  is  able  to  get  about.  Others  improve  slowly  but 
steadily,  and  finally  walk  without  a  cane,  but  stif&y. 

In  the  severe  cases  the  arms  are  rigidly  adducted  and  flexed,  and 
the  hands  are  coutractured  and  useless.  (See  Fig.  236.)  In  such  a 
condition  athetoid  movements  are  universal.  The  tendon  reflexes  are 
always  exaggerated,  the  muscles  of  the  limbs  are  poorly  developed,  but 
there  are  no  bed-sores,  and  there  is  no  loss  of  control  of  the  sphincters. 
There  are  no  sensory  symptoms.  These  children  frequently  have 
mental  defects,  and  epilepsy  is  likely  to  develop. 

Strabismus  is  a  not  uncommon  symptom,  and  difficulty  of  articula- 
tion, or  of  swallowing  may  be  present.     Oppenheim  has  called  attentiou 


MALDEVELOPMENT    OF    THE    BRAIN.  527 

to  the  timidity  of  these  children  and  to  the  fact  that  sudden  noises  cause 
them  to  ju'fiap  or  even  throw  them  into  convulsions. 

In  a  few  of  my  cases  the  symptoms  have  been  exclusively  cerebellar 
in  type.  The  child  has  been  unable  to  sit  up,  to  balance  itself  on  its 
feet,  or  to  learn  to  walk.  In  one  such  case  dating  from  birth,  the  child 
was  still  absolutely  helpless  at  the  age  of  nine  years,  although  intelli- 
gent and  not  paralyzed.  In  another  the  same  condition  was  present 
at  the  age  of  five  years,  but  was  attended  by  feeble-mindedness.  This 
child  died  of  measles,  and  Fig.  233  shows  the  unilateral  atrophy  of  the 
cerebellum  found. 

In  many  of  the  cases  difficulty  in  learning  to  talk  has  been  noticed, 
the  child  acquires  words  slowly,  and  sometimes  even  at  the  age  of  six 
years  is  unable  to  frame  a  sentence  or  to  speak  clearly. 

Epilepsy.  —  In  the  majority  of  cases  both  of  hemiplegia  and  of 
diplegia  epileptic  attacks  begin  soon  after  the  paralysis ;  though  some- 
times the  interval  is  several  months  or  even  several  years.  Two-thirds 
of  the  cases  eventually  develop  epilepsy. 

The  fits  in  organic  epilepsy  are  more  frequent  and  severe  than  in 
idiopathic  epilepsy.  I  have  one  patient  who  has  had  as  many  as 
twenty  convulsions  daily  for  weeks  at  a  time.  These  fits  do  not  de- 
stroy life,  but  of  course  render  life  a  burden.  They  are  different  from 
the  fits  of  idiopathic  epilepsy  in  being  of  a  character  which  indicates 
local  irritation  of  the  cerebral  cortex.  (Seepage  91.)  They  usually 
begin  in  one  hand  with  closure  of  the  fist,  or  pronation  of  the  hand, 
attended  by  a  sensation  of  tingling ;  the  contraction  of  the  muscles  is 
clonic  and  the  spasm  extends  up  to  the  elbow,  and  finally  the  shoulder- 
joint  is  thrown  into  motion,  the  entire  arm  moving  in  the  convulsion. 
The  spasm  then  extends  either  to  the  neck  and  face,  which  is  drawn  to 
the  paralyzed  side,  or  it  spreads  down  the  trunk  and  involves  the  leg, 
which  shakes  or  is  violently  extended  with  clonic  spasms  of  the  toes. 
Such  a  spasm  may  last  for  a  few  minutes,  and  then  gradually  cease, 
leaving  the  muscles  paralyzed  and  the  side  numb  for  several  hours.  Or 
it  may  extend  to  the  other  side  of  the  body,  in  which  case,  as  a  rule, 
consciousness  is  lost  for  some  minutes,  the  patient  falls,  bites  the 
tongue,  froths  at  the  mouth,  and  subsequent  to  the  attack  sleeps  heavily 
for  an  hour  or  more,  waking  dazed,  and  often  unable  to  talk  or  to  think 
for  some  hours.  In  many  cases  the  attack  begins  in  the  leg  and 
extends  to  arm  and  face.  In  other  cases  the  reverse  is  the  order  of 
extension  of  the  spasm ;  the  point  of  greatest  irritation,  in  the  cor- 
tex being  indicated  by  the  part  of  the  body  first  aifected.  In  some 
cases  head  and  eyes  turn  toward  the  paralyzed  side  at  the  beginning 
of  the  fit. 

In  other  cases  the  epileptic  attack  begins  with  sensory  rather  than 
with  motor  symptoms,  the  initial  numbness  and  tingling  always  accom- 
panies and  may  precede  the  local  spasm.  But  sometimes  it  is  a  visual 
aura,  a  light,  or  color,  or  figure  seen  with  which  the  attack  begins.  In 
other  cases  it  is  a  sound,  a  noise,  or  a  bell,  or  a  voice  which  is  heard. 
In  other  cases  it  is  an  odor  or  a  taste  which  is  perceived.     In  all  these 


528  TRE   CEBEBRAL   ATROPHIES    OF   CHILDHOOD. 

conditions  a  local  spasm  or  a  general  convulsion  follows  the  aura,  indi- 
cating that  the  irritation  of  the  brain  which  has  started  in  a  sensory 
areaj  the  chief  seat  of  disease,  has  radiated  outward  and  has  invaded 
the  motor  centres.  (See  page  91.)  Mental  aurae  are  also  observed, 
consisting  of  a  dreamy  state,  a  sort  of  second  consciousness,  when  the 
mind  seems  to  watch,  its  own  processes  without  being  able  to  modify 
them.  The  mental  aura  may  be  a  sudden  fear,  or  fright,  or  distress, 
pleasant  ideas  being  rarely  present,  though  not  unknown.  Attacks  of 
an  active  maniacal  kind  or  of  double  consciousness  may  occur  without 
a  convulsion  ;  these  are  the  so-called  "  psychical  epileptic  equivalents " 
of  an  attack. 

All  these  forms  of  attack  are  of  very  great  interest,  as  they  indicate 
quite  faithfully  the  area  of  brain  in  which  the  disease  is  located,  and 
form  the  guide  to  the  surgeon  in  cases  which  are  open  to  operation. 

II.  Symptoms  of  Mental  Defects. — The  second  group  of  cases 
presents  mental  defects  rather  than  physical  symptoms.  There  may 
be  an  entire  lack  of  mental  development  from  the  outset.  This  is 
termed  congenital  idiocy,  and  is  due  to  congenital  defects  of  the  brain 
or  to  some  arrest  of  growth  soon  after  |)irth.  There  may  be  an  acquired 
idiocy,  from  some  disease  occurring  during  infancy  or  childhood,  such 
as  injury  of  the  brain  from  trauma,  or  after  convulsions,  or  due  to 
some  of  the  infectious  diseases.  Idiots  have  been  classified  as  micro- 
cephalic, hydrocephalic,  paralytic,  epileptic,  meningitic,  or  amaurotic, 
according  to  the  predominant  cause. 

A  maldevelopment  of  the  thyroid  gland  leading  to  cretinism  and 
mental  weakness  is  not  attended  by  brain  lesions,  and  should  not  be 
classed  with  idiocy.     It  may  be  cured  by  thyroid  treatment. 

Idiots  are  wholly  lacking  in  the  power  of  acquiring  ideas  or  concepts, 
in  the  power  of  fixing  the  attention  and  in  the  capacity  of  remembering, 
and  cannot  be  trained  to  keep  themselves  clean.  The  sensory  powers 
are  defective  in  many,  but  they  are  in  constant  motion,  a  rhythmical 
swaying  being  often  observed. 

If  the  degree  of  mental  defect  is  less  marked  the  mental  state  is 
described  as  one  of  imbecility.  An  imbecile  can  acquire  simple  ideas, 
can  recognize  persons  and  objects,  can  say  a  few  words,  but  is  incapable 
of  acquiring  any  of  the  higher  conceptions  which  are  the  basis  of  knowl- 
edge and  of  conduct.  Imbeciles  are  usually  helpless  if  left  alone,  and 
are  often  ugly  in  disposition,  destructive  and  filthy.  The  difference 
between  an  idiot  and  an  imbecile  is  one  of  degree  only,  and  no  sharp 
line  can  be  drawn  between  them. 

There  are  other  patients  with  mental  defects,  of  a  higher  type  of 
mind  than  imbeciles,  but  nevertheless  abnormal.  These  are  classed  as 
weak-minded.  The  child  may  be  slow  in  learning  to  walk ;  may  seem 
unable  to  fix  its  attention  upon  anything  continuously ;  may  be  exceed- 
ingly active,  in  constant  motion  —  the  activity  being,  however,  aimless; 
may  throw  things  about,  or  tear  things  up,  or  put  everything  into  its 
mouth  ;  may  be  very  difficult  to  manage  because  of  its  inability  to  retain 
and  combine  impressions  with  sufficient  power  to  reason  upon  them. 


MALDEVELOPMENT    OF    THE    BBAIN.  529 

and  many  therefore  be  incapable  of  appreciating  the  meaning  of  punish- 
ment, if  th^  be  inflicted.  Such  children  may  have  good  powers  of 
perception,  may  recognize  persons  and  objects,  show  pleasure  at  bright 
colors,  or  music,  or  caresses,  but  fail  to  show  evidence  of  thought  in 
the  sense  of  reasoning  power,  judgment,  or  self-control.  Some  patients 
constantly  drool  at  the  mouth,  cannot  be  taught  cleanly  habits,  and  are 
manifestly  imbecile.  Other  patients  are  quite  bright  in  many  direc- 
tions, may  even  be  precocious,  show  talents  in  music,  or  drawing,  or 
fondness  for  mathematics,  designing,  and  languages,  yet  are  apparently 
unable  to  appreciate  moral  ideas,  cannot  be  taught  to  tell  the  truth,  are 
cruel  and  bad,  will  not  control  any  of  their  impulses,  and  so  are  the  dis- 
tress and  despair  of  parents  and  teachers.^  It  is  those  mental  qualities 
which  are  the  product  of  the  highest  evolution  that  have  failed  to 
develop  in  this  class  of  cases.  The  final  result  is  that  they  have  to 
be  taken  care  of  all  their  lives,  either  at  home  or  by  attendants,  being 
incapable  of  supporting  themselves  or  directing  their  conduct.  Many 
of  them  have  epilepsy. 

III.  Sensory  Defects.  —  The  third  group  of  cases  show  sensory 
defects,  usually  blindness  or  deafness,  although  a  loss  of  taste  and  smell 
is  occasionally  observed.  These  symptoms  are  likely  to  escape  obser- 
vation until  the  child  reaches  the  age  of  two  or  three  years.  Patients 
belonging  to  this  class  may  present  no  motor  •  or  mental  defects, 
though  they  may  be  the  subject  of  epilepsy.  On  several  occasions  I 
have  been  consulted  by  parents  who  have  brought  children  to  me  sup- 
posedly the  subjects  of  epilepsy  in  whom  I  have  detected  one  or  more 
of  these  sensory  defects.  Thus,  in  three  cases  a  condition  of  homony- 
mous hemianopsia  was  discovered  which  was  undoubtedly  congenital, 
each  child  having  grown  up  without  any  appreciation  of  the  defect  of 
vision.  In  all  of  these  cases  there  was  a  corresponding  flattening  of 
the  skull  over  the  occipital  lobe,  and  all  the  patients  were  suffering 
from  epilepsy.  Moeli  ^  has  described  three  such  cases  in  which  poren- 
cephalus  was  found  in  the  occipital  lobe,  and  Henschen  ^  and  Freund  * 
have  also  observed  such  cases.  In  several  patients  whom  I  have  seen 
total  blindness  has  been  found.  It  is  not  always  easy  to  determine 
whether  this  is  due  to  a  primary  atrophy,  or  lack  of  development  in 
the  optic  nerve,  or  whether  it  is  due  to  atrophy  of  the  occipital  convo- 
lutions. Both  conditions  have  been  found  post-mortem  in  many  cases. 
If  the  condition  is  due  to  atrophy  of  the  optic  nerve  an  ophthalmo- 
scopic examination  will  reveal  this  condition. 

There  are  other  cases  in  which  the  patients  are  deaf.  If  a  child  has 
never  heard,  or  lost  its  hearing  before  it  has  learned  to  talk,  it  becomes 
mute.  The  majority  of  cases  of  deaf-mutism  are  undoubtedly  due  to 
lesions  in  the  ears  or  to  lesions  of  the  auditory  nerves  upon  the  base  of 
the  l^rain.  There  are  a  few  cases,  however,  on  record  in  which  an 
atrophy  of  the  temporal  convolutions  has  been  attended  by  deafness. 

'See  F.  Peterson.     Idiots  Savants.     Popular  Science  Monthly,  Dec,  1896. 
^Archiv.  fiir  Psych.,  xxii.,  2.     ^  Kensclien,  Path,  des  Gehirns,  cases 32 and 35. 
*  Freund,  Wien.  med.  Woch.,  1888,  No.  32, 

34 


530  THE  CESEBRAL  ATROPHIES   OF  CHILDBOOD.      ^ 

Loss  of  smell  and  taste  have  been  produced  by  atrophic  conditions 
of  the  uncinate  convolutions  upon  the  base  of  the  brain.  These  are 
usually  traceable  to  basilar  meningitis.  They  very  often  escape  obser- 
vation, though  in  any  case  where  the  child  is  known  to  put  all  sorts  of 
objects  into  the  mouth  and  be  willing  to  eat  offensive  things,  a  sus- 
picion of  a  loss  of  taste  is  awakened. 

A  localized  atrophy  in  the  convolutions  of  the  brain  may  also  occur 
from  disuse.  Thus,  in  a  number  of  cases  recorded  where  patients  have 
lost  the  sense  of  hearing  and  of  sight  in  early  life,  as  for  example,  from 
basilar  meningitis,  cerebro-spinal  meningitis,  or  lesions  of  the  ear,  or 
eye,  the  function  of  the  corresponding  portion  of  the  brain  has  not  been 
awakened,  and  as  a  result  of  absolute  disuse  an  atrophy  of  these  con- 
volutions has  been  discovered  when  the  patients  have  died  late  in  life. 
This  was  the  condition  found  in  the  brain  of  Laura  Bridgman,  the 
temporal  and  occipital  convolutions  being  rudimentary  in  size,  while 
the  sensorimotor  convolutions  about  the  fissure  of  Rolando  were  enor- 
mously developed.  This  girl  lost  sight  and  hearing  at  the  age  of  two 
years  from  a  basilar  meningitis,  but  developed  remarkable  powers  of 
observation  through  the  sense  of  touch ;  and  of  expression  through 
motor  action.  In  two  other  cases  reported  by  foreign  observers  the 
same  condition  has  been  found.  Hence  the  localized  atrophy  due  to 
failure  of  development  in  limited  areas  of  the  brain  after  disuse  of  the 
corresponding  organs  is  proven  in  man  as  well  as  established  by  the 
experiments  of  von  Gudden. 

Epilepsy  is  a  common  condition  in  these  cases  of  sensory  defect. 
The  attack  not  infrequently  begins  by  an  aura  of  a  sensory  nature.  It 
may  be  limited  to  an  attack  of  petit  mal,  or  it  may  go  on  to  an  attack 
of  grand  mal.  If  the  atrophy  of  the  brain  is  unilateral  the  epileptic 
attack  may  be  of  a  Jacksonian  nature. 

Prognosis.  —  The  prognosis  in  cerebral  atrophy  is  most  unfavorable. 
The  underlying  pathological  condition  is  permanent,  even  though  it  is 
not  progressive,  and  there  is  no  hope  of  improvement  or  of  recovery. 
The  paralysis,  the  mental  defects,  and  the  sensory  defects  cannot  pos- 
sibly be  relieved.  The  epilepsy  may  be  held  in  check  to  some  extent 
by  the  use  of  bromides,  the  number  of  fits  being  reduced,  their  severity 
somewhat  mitigated.     But  they  cannot  be  stopped. 

Treatment.  —  The  treatment  of  an  acute  attack  of  apoplexy  in 
childhood  does  not  differ  in  any  way  from  that  occurring  in  the  adult. 
The  same  care  must  be  taken  in  diagnosis,  and  the  same  measures 
adopted  as  in  the  conditions  of  hemorrhage  or  thrombosis  already 
considered. 

The  treatment  of  the  condition  of  infantile  paralysis  and  of  idiocy 
consists  in  rendering  the  life  of  these  patients  as  happy  and  comfort- 
able by  proper  surroundings  and  agreeable  attendants  as  possible.  This 
can  best  be  done  by  securing  for  them  a  country  home  away  from  ex- 
citement and  away  from  the  family.  Parents  too  often  expend  more 
care,  attention,  and  money  upon  these  chronic  invalids  than  upon  their 
healthy  children  who  suffer  in  consequence.    It  should  be  made  clear  to 


MALDEVELOPMENT    OF    THE    BRAIN.  531 

them  that  in  doing  so  they  accomplish  little  and  are  unjust  to  those  who 
will  bette/ repay  their  care.  Education  by  those  who  are  skilled  in  the 
training  of  the  weak-minded  accomplishes  something,  and  is  to  be  recom- 
mended if  parents  can  afford  the  expense.  The  treatment  of  the  epi- 
leptic condition  is  the  same  as  that  of  ordinary  epilepsy  by  the  use  of 
bromides  ;  but  in  these  cases  the  effect  of  the  drug  soon  wears  off",  and 
I  prefer  not  to  use  any  medical  treatment. 

Surgical  Treatment.  —  When  a  patient  belonging  to  one  of  these 
clinical  types  is  presented  to  the  neurologist  and  the  question  is  asked, 
can  surgical  treatment  benefit  him?  it  is  evident  that  a  serious  problem 
is  opened.  In  these  cases  the  disease  is  at  a  standstill  and  does  not 
threaten  life,  and  yet  is  hopeless  from  the  medical  standpoint.  Surgi- 
cal treatment  is  not  free  from  danger,  but  if  it  offers  any  relief  almost 
any  risk  is  justifiable.  The  brain  is  still  capable  of  great  develop- 
ment in  infancy  and  youth.  Can  such  development  be  aided  by  an 
operation?  In  many  cases  the  epileptic  attacks  are  of  such  frequent 
occurrence  that  any  risk  might  well  be  taken  if  they  could  be  surely 
stopped. 

Any  solution  of  the  problem  of  operative  treatment  must  be  based 
upon  two  considerations :  first,  the  pathology  of  the  cases ;  secondly, 
the  results  of  experience  when  such  operations  have  been  done. 

First,  from  the  records  of  the  pathology  already  given  in  343  cases 
it  is  evident  that  a  state  of  cerebral  atrophy  was  present  in  the  majority 
which  nothing  could  remove,  and  it  is  apparent  that  many  of  the  con- 
ditions were  of  such  a  nature  as  to  be  wholly  unaflFected  by  any  opera- 
tive interference.  A  porencephalic  cavity  filled  with  cerebrospinal 
fluid  is  not  likely  to  be  benefited  by  any  enlargement  of  the  intra- 
cranial space  or  by  the  abstraction  of  the  fluid.  In  several  cases 
operated  upon  the  withdrawal  of  this  fluid  has  been  followed  by  sudden 
collapse  and  death. ^  On  the  other  hand,  there  are  conditions,  such  as 
maldevelopment  of  the  cortex  without  gross  lesion,  in  which  it  is  pos- 
sible that  anything  which  may  stimulate  latent  powers  of  growth,  or 
may  remove  those  influences  which  interfere  with  development,  might 
result  in  improvement.  It  is  to  be  remembered  that  the  brain  is  cap- 
able of  growth  and  development  until  the  age  of  fourteen  years,  if  not 
longer,  and,  granting  that  a  stimulus  to  its  growth  may  be  given  dur- 
ing childhood,  the  arrest  of  development  in  many  cases  might  perhaps 
have  been  prevented.  And  there  are  cases  of  cysts  lying  upon  the 
cortex  whose  removal  may  relieve  pressure  and  allow  a  proper 
growth. 

The  study  of  the  pathological  conditions,  therefore,  does  not  abso- 
lutely contraindicate  operative  interference,  although  it  makes  it  clear 
that  the  lesion  in  the  majority  of  the  cases  is  one  that  cannot  be 
improved  by  any  means.  If  we  admit  that  porencephalus,  atrophy 
from  vascular  lesion,  meningo-encephalitis,  and  hydrocephalus  are 
incurable,  and  that  hemorrhages  cannot  be  diagnosed  early  enough  to 

^  Cases  of  Bullard,  Boston  Medical  and  Surgical  Journal,  February  16,  1888.     Ham- 
mond, New  York  Medical  Journal,  August  12,  1890.     One  of  my  own. 


532  TEE   CEREBBAL   ATROPHIES    OF   CHILDHOOB. 

warrant  the  removal  of  the  clot  before  it  has  caused  atrophy  from  pres- 
sure, we  have  193  cases  out  of  343  in  which  operation  would  have 
been  futile.  This  leaves  150  cases  of  sclerotic  atrophy,  maldevelop- 
ment  of  the  cortex,  and  cysts,  in  which  it  is  barely  possible  that  an 
operation,  if  it  relieved  pressure  or  stimulated  brain  growth  might  have 
had  some  effect.  It  is  unfortunate  that  we  cannot  make  an  exact  patho- 
logical diagnosis  from  the  clinical  symptoms.  Therefore,  one  cannot 
affirm  that  in  any  particular  case  a  removable  cyst  is  present  or  a  con- 
dition of  pressure  which  may  be  relieved.  The  operation  must  in  all 
cases,  therefore,  be  exploratory,  and  this  should  always  be  understood 
by  all  concerned. 

There  is  one  further  fact  which  requires  a  word  of  explanation.  In 
many  of  these  cases  inspection  shows  a  very  decided  irregularity  of  the 
skull.  It  has  been  thought  by  some  observers,  especially  Lannelongue, 
in  France,  that  an  early  closure  of  the  sutures  and  fontanelles  in 
infancy  may  prevent  a  proper  expansion  of  the  skull,  and  thus  pro- 
duce a  pressure  on  the  brain,  preventing  its  growth.  These  authors 
regard  the  atrophy  as  secondary  to  compression.  This  view  I  consider 
erroneous.  The  skull  does  not  unite  as  long  as  there  is  any  pressure 
within  it.  It  is  the  cerebral  defect  due  to  disease  which  prevents  the 
brain  from  developing  and  arrests  its  normal  growth  and  internal 
pressure.  The  bones  close  because  the  natural  expansion  has  ceased. 
The  skull  does  not  cause  pressure  on  the  brain.  This  is  evident  in 
cases  operated  upon,  for  no  signs  of  compression  of  the  brain  have 
been  found  in  these  cases.  The  theory,  therefore,  upon  which  opera- 
tions of  craniectomy  have  been  done  is  not  borne  out  by  the  facts. 

Secondly.  The  results  of  experience  of  surgeons  in  the  treatment  of 
these  cases  by  operation  has  not  been  very  favorable. 

The  operation  of  craniectomy  has  been  done  by  many  surgeons 
during  the  past  few  years,  and  the  results  have  been  reported  by  several 
of  them,  notably  by  Lannelongue,  Keen,  Bullard,  Oppenheim,  Frank, 
Hammond,  Horsley,  Agnew,  and  McBurney.  Many  other  operators 
have  reported  single  cases,  and  my  own  experience  in  connection  with 
McBurney  is  extensive.^ 

Lannelongue  reported,  in  1891,  that  he  had  operated  on  25  patients, 
chiefly  microcephalic  or  epileptic  children,  with  but  one  fatal  result,  and 
that  there  was  an  improvement  in  many  cases.  Lannelongue's  operation 
is  to  make  a  long,  curved  incision  through  the  scalp  and  skull  from  the 
temporal  ridge  backward  for  six  to  ten  inches,  and  crack  the  side  of  the 
skull  outward,  leaving  an  interspace  of  an  inch  or  more  in  width  which 
fills  up  with  fibrous  connective  tissue.  Thus  he  relieves  the  supposed 
compression  of  the  brain.  It  is  unfortunate  that  these  cases  were  re- 
ported soon  after  the  operation,  so  that,  although  the  surgical  result  was 
not  fatal,  we  know  nothing  of  the  effects  of  the  operation  on  their  brain 
development.  In  a  recent  article  by  one  of  Lannelongue's  assistants 
the  intimation  is  given  that  little  was  accomplished. 

'  For  an  analysis  of  these  operations  see  the  Medical  Record,  January  23,  1892 ; 
Brain  Surgery,  Chapter  III, 


MALDEVELOPMENT  OF  THE  BRAIN.  533 

In  my  own  collection  of  cases,  50  in  number,  including  15  in  my 
own  experience,  the  result  cannot  be  said  to  be  very  encouraging.  Of 
50  cases  16  died  during  or  soon  after  the  operation.  Death  was  due 
in  11  cases  to  shock  from  sudden  evacuation  of  fluid  from  a  large 
cavity  in  the  brain.  Thus,  in  one  case  operated  upon  for  me  by  Mc- 
Burney,  in  which  the  child,  a  girl  of  eleven  years,  presented  only  a 
slight  degree  of  right  hemiplegia,  with  severe  attacks  of  hemiepilepsy 
and  no  imbecility  or  aphasia,  the  entire  left  frontal  lobe  was  deficient, 
its  place  being  occupied  by  a  cyst.  The  exposure  of  this  on  opening 
the  dura  was  attended  by  laceration  of  its  wall,  as  it  was  adherent  to 
the  dura,  and  the  flow  of  serous  contents  was  followed  by  immediate 
collapse  and  death.  In  10  cases  reported  by  others  a  similar  result 
occurred.  In  some  cases  death  has  been  due  to  excessive  hemorrhage 
from  large  brittle  veins  in  the  pia  or  in  the  walls  of  a  porencephalic 
cavity.  Here  ligature  was  impossible,  and  compression  equally  so. 
In  some  cases  death  has  been  due  to  exhaustion  following  the  opera- 
tion, which  has  necessarily  been  long.  It  is  evident,  therefore,  that 
these  operations  are  much  more  dangerous  than  the  ordinary  operation 
for  trephining,  because  of  the  existence  of  pathological  conditions 
which  cannot  be  foreseen  or  provided  against. 

In  the  thirty-four  patients  who  have  survived  the  operation  a  certain 
amount  of  improvement  has  been  reported  in  twenty-five,  and  in  nine 
no  apparent  result  was  noticed.  The  improvement  has  consisted  in  a 
relief  of  the  paralysis,  but  not  in  its  disappearance ;  a  marked  relief 
from  the  athetoid  movements,  but  no  cessation ;  an  improvement  in  the 
gait ;  an  improvement  in  the  mental  capacity,  and  a  change  from  imbe- 
cility to  a  condition  of  weak-mindedness,  but  no  complete  recovery  or 
normal  development  of  the  mind  ;  a  diminution  in  the  frequency  and  a 
mitigation  in  the  severity  of  the  epileptic  attacks,  but  no  absolute  cure. 

In  several  of  my  own  cases  nothing  abnormal  has  been  found  in  the 
brain  at  the  operation,  and  it  has  been  supposed  that  the  lesion  was  of 
a  microscopic  character.  In  two  of  these  cases  the  subsequent  history 
has  shown  considerable  improvement,  which  must  have  been  wholly 
independent  of  the  operation,  and  awakens  the  suspicion  that  such  im- 
provement of  a  spontaneous  kind  may  have  been  erroneously  ascribed 
to  the  operation  by  partial  observers. 

In  three  of  my  cases  a  thin  organized  blood  clot  containing  fine  con- 
nective-tissue filaments  adherent  to  the  dura  in  one  case  and  to  the 
brain  and  cortex  in  two  cases  was  discovered.  It  was  possible  to 
sponge  and  scrape  away  this  clot  in  all  these  cases,  though  some 
hemorrhage  followed  from  the  rupture  of  the  new  capillaries  in  the 
organized  mass.  These  were  all  cases  of  several  years'  duration,  hence 
the  clot  was  not  recent  in  any.  In  all  these  cases  there  was  an 
apparent  marked  improvement.  In  a  number  of  my  cases  cysts  have 
been  found  —  sometimes  lying  on  the  cortex,  in  the  pia,  and  causing  a 
depression  of  the  cortex ;  in  other  cases  within  the  brain,  being  in  part 
covered  by  the  cortex;  in  some  cases  deep  in  the  white  matter,  being 
discovered  only  by  the  exploratory  needle.     It  has  usually  been  pos- 


534  TBE  CEREBRAL  ATROPHIES   OF   CHILDHOOD. 

sible  to  remove  these  cysts,  the  walls  being  carefully  dissected  away 
without  rupture.  It  was  found  that  if  they  were  merely  evacuated 
the  fluid  soon  returned,  and  a  second  operation  was  necessary.  This 
occurred  in  two  cases.  When  the  cyst  lay  deep  in  the  white  matter 
and  could  only  be  discovered  by  puncture  it  has  not  been  always  pos- 
sible to  remove  the  wall,  and  in  these  cases  they  probably  refilled,  as  no 
improvement  followed.  The  best  result  has  been  obtained  in  the 
cases  where  a  cyst  could  be  removed  as  a  whole.  In  one  case  a  cystic 
mass  exactly  like  honeycomb  occupied  the  entire  lower  two-thirds  of 
the  anterior  central  convolution,  and  could  not  be  removed  entirely. 

In  some  cases  thick,  firm,  connective-tissue  scars,  or  porous  bands 
have  been  found  in  the  cortex,  in  one  case  as  firm  as  cartilage,  in 
another  case  calcified  in  part.  These  were  probably  the  result  of 
hemorrhages  or  of  an  area  of  softening.  Their  removal  was  attended 
by  severe  hemorrhage,  in  one  case  making  it  necessary  to  postpone 
twice  a  completion  of  the  operation.  In  neither  of  these  cases  were 
the  mental  symptoms  and  epilepsy  for  which  the  operation  was  under- 
taken at  all  improved. 

This  experience  leads  me  to  be  very  cautious  about  urging  an  operation 
in  this  class  of  cases.  The  dangers  are  many.  The  prospect  of  relief 
is  very  small.  Cure  cannot  be  promised.  Improvement  in  a  small  pro- 
portion of  the  cases  only  is  the  best  result  which  can  be  expected. 

Harvey  Gushing  has  recently^  advocated  an  operation  immediately  after 
the  appearance  of  the  symptoms,  especially  in  the  cases  developing  at  birth. 
He  has  had  several  successful  cases,  in  which,  within  a  few  days  of  birth 
or  of  the  appearance  of  hemiplegia,  he  has  opened  the  skull  by  making 
incisions  along  the  lines  of  the  sutures  in  the  parietal  bone,  has  turned 
down  the  parietal  bone,  exposed  the  brain,  found  a  large  surface  clot 
and  has  removed  it.  I  heartily  approve  this  operation  in  a  case  where 
the  diagnosis  of  hemorrhage  can  be  made  in  a  positive  way,  but  think 
it  should  be  done  only  by  a  surgeon  skilled  in  cerebral  surgery. 

*  American  Journal  of  the  Medical  Sciences,  July,  1905. 


CHAPTER  XXIX. 

ENCEPHALITIS. 

Acute  Hemorrhagic  Cortical  Encephalitis.     Polio-encephalitis  Superior  and  Inferior. 

Cerebellar  Encephalitis. 

Etiology.  —  That  an  acute  inflammation  of  the  brain  may  occur  as 
an  independent  affection  has  been  established  by  Striimpell,  Leichten- 
stein,  and  Oppenheim.  It  is  probably  an  acute  infectious  disease,  as 
it  has  all  the  characteristic  signs  of  one.  It  has  been  known  to  occur 
in  the  course  of  scarlet  fever,  measles,  influenza,  pneumonia,  erysipelas, 
whooping-cough,  mumps,  diphtheria,  ulcerative  endocarditis,  otitis 
media,  and  septicaemia.  It  is  not  necessarily  accompanied  by  purulent 
meningitis,  though  it  may  be.  In  most  of  these  conditions  the  char- 
acteristic organism  of  the  original  disease  has  been  found  in  the  foci  of 
inflammation  in  the  brain.  Ptomaine  poisoning  has  been  ascribed  as 
a  cause,  also  alcoholism  and  poisoning  by  carbon  dioxide.  It  is  still  a 
matter  of  dispute  whether  an  injury  of  the  head  not  attended  by  frac- 
ture or  meningitis  can  set  up  an  acute  inflammation  of  the  brain. 
Bruises  in  soft  tissues  are  rarely  followed  by  inflammation,  but  may 
predispose  an  organ  to  the  invasion  of  bacteria.  I  have  seen  abscess 
to  occur  under  these  conditions,  and  believe  it  to  have  resulted  from 
such  an  inflammation.  The  disease  occurs  chiefly  in  children  and 
young  people,  but  may  develop  in  adults. 

Varieties.  —  The  inflammatory  process  is  usually  limited  in  its  extent 
in  the  brain,  and  is  not  diffuse.  It  is  sometimes  only  to  be  found  by 
microscopic  investigation.  It  is  sometimes  wholly  within  the  domain 
of  one  arterial  branch.  In  other  cases  it  has  been  known  to  affect 
symmetrical  parts  of  both  hemispheres,  and  in  a  few  cases  disseminated 
foci  of  inflammation  have  been  found.  The  inflammation  may  be  found 
in  both  gray  and  white  matter,  and  is  not  limited  to  either.  It  may 
affect  any  part  of  the  brain.  A  number  of  different  forms  have  been 
described,  depending  upon  the  location. 

Thus,  Striimpell's  first  cases  were  chiefly  cortical  in  the  motor  area, 
and  were  called  acute  infantile  cerebral  palsy,  or  acute  hemorrhagic 
cortical  encephalitis.  Later  this  condition  was  found  to  occur  in 
adults  and  even  in  old  age  as  in  a  case  of  Mills.^ 

In  the  cases  collected  by  Wernicke  and  named  by  him  polioencepha- 
litis superior  the  lesion  was  located  in  the  gray  matter  lining  the 
aqueduct  of  Sylvius.  These  cases  are  not  different  from  acute 
ophthalmoplegia. 

'C.  K.  Mills.  Review  of  Neurology  and  Psychiatry,  Feb.,  1907,  where  full  refer- 
ences may  be  found. 

635 


536  E'NCEPEALITIB. 

There  is  a  form  limited  to  the  nuclei  of  the  motor  cranial  nerves  of 
the  tongue  and  face,  termed  polio-encephalitis  inferior,  or  acute  bul- 
bar paralysis,  and  causing  all  the  symptoms  of  chronic  bulbar  paralysis 
(Chapter  XXXIV.),  but  with  acute  onset  and  rapid  course.^  Such 
cases  have  occurred  as  a  complication  of  acute  anterior  poliomyelitis, 
in  several  epidemics  ;  see  Chapter  XIV. 

Lastly,  a  form  limited  to  the  cerebellum  has  also  been  described  as 
a  separate  disease. 

Pathology.  —  The  lesion  in  all  these  cases  is  the  same,  and  the  dif- 
ference in  the  symptoms  is  dependent  entirely  upon  the  different  func- 
tions of  the  parts  of  the  brain  affected.  Such  a  multiplication  of 
diseases  is  an  unfortunate  weakness  of  authors  who  lack  the  generaliz- 
ing faculty.  The  disease  has  its  exact  homologue  in  acute  anterior 
poliomyelitis,  and  the  changes  present  are  the  same.  The  two  diseases 
have  been  known  to  occur  together.  The  changes  found  in  these  cases 
in  the  acute  stage  are  (1)  an  acute  hypersemia  with  distention  of  the 
bloodvessels,  rupture  of  their  walls,  capillary  hemorrhages  in  the  tis- 
sues, and  an  emigration  of  leucocytes  and  small  cells.  (2)  Various 
stages  and  degrees  of  degeneration  of  the  neurones,  both  cell  body, 
axones,  and  dendrites  being  affected,  with  subsequent  secondary  changes 
of  wide  extent.  The  exact  microscopic  changes  are  identical  with  those 
already  described' in  the  chapter  on  anterior  poliomyelitis,  but  are  located 
in  the  cortex  or  bulbar  nuclei. 

After  an  acute  stage  the  hypersemia  subsides,  the  clots  or  diffusely 
infiltrated  red  blood  cells  may  be  absorbed,  and  if  the  degeneration  of 
neurones  has  not  led  to  the  destruction  of  a  large  number  controlling 
any  special  function  a  gradual  recovery  ensues  and  no  trace  is  left.  In 
this  respect  the  disease  resembles  anterior  poliomyelitis,  where  recovery 
in  many  of  the  muscles  at  first  paralyzed  is  the  rule.  But  if  a  number 
of  neurones  in  any  system  are  destroyed  there  are  permanent  symptoms 
remaining  whose  character  depends  on  the  function  of  the  neurones 
affected.  Thus,  if  the  frontal  lobes  are  affected,  imbecility  results.  If 
the  motor  cortex  is  destroyed  a  state  of  hemiplegia  remains.  In  a  case 
examined  by  Hochhaus,  in  which  cerebral  hemiplegia  developed  in  the 
course  of  measles  in  an  infant,  a  cortical  encephalitis  with  some  local- 
ized meningitis  was  found.  If  the  ocular  motor  nuclei  are  affected 
strabismus  and  paralysis  of  ocular  movement  results.  If  the  facial  and 
lingual  nuclei  are  paralyzed  some  defect  in  speech,  swallowing,  and 
facial  expression  remains.  If  the  cerebellum  is  affected  a  permanent 
defect  of  co5rdination  and  of  gait  ensues.  If  the  cord  is  affected  with 
the  brain,  some  type  of  poliomyelitis  attends  the  encephalitis. 

The  permanent  result  of  the  pathological  process  is  the  formation  of 
small  or  large  areas  of  sclerotic  tissue  or  of  small  cysts.  The  glia  cells 
are  multiplied,  connective-tissue  elements  are  increased,  and  a  small 
patch  of  sclerosis  or  a  state  of  sclerotic  atrophy  of  an  entire  lobe  of  the 
hemisphere,  or  any  degree  between  these  extremes,  may  be  left  as  a 

^For  a  review  of  these  rare  cases  see  Judson  and  Carncross,  Amer.  Jour.  Med.  Sci., 
Dec,  1908. 


SYMPTOMS.  537 

permanent  condition.  Thus,  disseminated  encephalitis  of  infectious 
origin  is  tne  starting  point  of  many  cases  of  so-called  multiple  sclerosis. 
It  is  not  possible  that,  as  time  goes  on  or  as  other  infections  occur  these 
sclerotic  patches  may  extend,  but,  as  a  rule,  they  are  latent.  These 
pathological  changes  in  the  brain  when  on  the  surface  are  often  at- 
tended by  the  lesions  of  meningitis  in  the  pia  and  dura.  Hence,  in 
the  terminal  stage  an  adhesion  of  the  membranes  to  the  cortex  is  fre- 
quently found.  In  a  few  fatal  cases  of  recent  origin  thrombosis  of  the 
venous  sinuses  has  been  observed  The  occurrence  of  leucocytosis 
during  life  will  distinguish  this  class  of  cases  from  those  due  to  vascu- 
lar lesions  only. 

Symptoms.  —  The  symptoms  of  encephalitis  may  be  divided  into 
two  classes  :  first  the  general  symptoms  of  the  infectious  disease,  and 
secondly  the  local  symptoms  of  the  particular  region  of  the  brain 
affected. 

The  general  symptoms  develop  acutely.  After  a  day  or  two  of  in- 
definite feelings  of  malaise,  attended  by  vertigo  and  headache,  and  in 
children  by  extreme  fretfulness,  there  is  a  sudden  chill,  attended  some- 
times by  vomiting  and  by  a  convulsion,  followed  by  a  state  of  stupor 
or  coma.  The  temperature  rises  rapidly  to  102°  or  104°  F.,  the  pulse 
is  rapid  but  regular,  the  respiration  normal,  rarely  of  the  Cheyne- 
Stokes  type.  In  the  state  of  stupor  there  is  restlessness  and  delirium. 
This  stupor  may  deepen  into  a  coma  which  may  never  be  recovered 
from.  If  the  patient  does  not  die  in  the  coma  he  gradually  recovers 
consciousness,  but  delirium  and  even  acute  maniacal  excitement  are 
not  uncommon  for  a  week  or  more,  and  have  been  known  to  continue 
with  fever  for  twenty  days.  The  recovery  of  consciousness  is  slow, 
and  the  mind  is  not  clear  for  some  days.  The  fever  subsides  by  remis- 
sions in  the  morning  and  its  attendant  symptoms  of  general  constitu- 
tional disturbance  gradually  pass  away.  Leucocytosis  is  observed 
throughout.  A  day  or  two  after  the  onset  some  local  symptoms  become 
evident,  and  then  the  diagnosis  of  hemiplegia,  or  of  ophthalmoplegia, 
or  of  bulbar  palsy,  may  be  made. 

The  local  symptoms  in  the  first  type  of  case  are  aphasia,  or  hemi- 
plegia, or  monoplegia,  and  these  are  not  infrequently  combined  at  the 
outset.  The  aphasia,  as  a  rule,  passes  away,  but  some  degree  of  hemi- 
plegia or  monoplegia  remains.  Thus,  in  one  of  my  patients,  while 
total  aphasia  and  right  hemiplegia  occurred  at  the  onset,  a  paralysis 
with  athetosis  of  the  right  arm  was  the  only  permanent  symptom.  In 
another  case  which  developed  suddenly  during  convalescence  from 
cerebro-spinal  meningitis,  a  left  hemiplegia  with  mental  weakness 
remains. 

In  another  type  of  case  the  local  symptoms  may  be  sensory  rather 
than  motor,  some  form  of  word-blindness  or  word-deafness  being  a  per- 
manent result,  or  hemianopsia,  as  in  a  case  of  Furbringer.  It  is  prob- 
able that  this  is  the  origin  of  deaf-mutism  in  some  cases. 

In  other  cases  a  state  of  imbecility  develops,  and  the  child  naturally 
born,  and  bright  before  the  attack,  grows  up  deficient  in  intelligence 
and  in  the  higher  powers  of  the  mind. 


53S  EVCSPBALtTlS. 

In  some  rare  cases,  as  in  one  of  my  own,  the  local  symptoms  pomt 
to  the  cerebellum  as  the  site  of  the  inflammation.  A  child  of  three 
years,  able  to  walk  and  feed  itself,  was  left  after  such  an  acute  attack, 
which  came  on  after  measles,  with  a  great  degree  of  ataxia  in  the  hands 
and  an  inability  to  balance  itself  on  its  feet,  to  stand  or  to  walk  or  to 
sit  up  without  the  aid  of  its  hands.  In  one  such  case  of  Bethe  cere- 
bellar lesions  were  found. 

In  all  these  types  of  cases  epilepsy  of  the  cortical  variety  is  a  com- 
mon sequel,  and  may  be  the  only  permanent  symptom  remaining.  In 
Mills'  case  Jacksonian  epilepsy  was  the  chief  symptom.  It  is  my 
belief  that  many  cases  of  so-called  idiopathic  epilepsy,  especially  those 
that  are  characterized  by  a  uniform  sensory  or  motor  aura,  originate  in 
acute  encephalitis  during  infancy  or  childhood. 

In  another  type  of  cases  (polio-encephalitis  superior  of  Wernicke) 
the  local  symptoms  are  confined  to  the  eye  muscles ;  there  is  ptosis  of 
one  or  both  eyes ;  the  eyeballs  cannot  be  moved  voluntarily  together ; 
there  is  some  form  of  strabismus;  there  is  nystagmus,  and  in  a  few 
cases  optic  neuritis  has  been  observed.  There  is  always  intense 
vertigo  with  this  condition,  and  hence  a  staggering  gait  is  sometimes 
noticed.  I  have  seen  but  one  such  case.  The  patient  was  a  physi- 
cian, and  recovered  entirely  within  a  year.  Weakness,  tremor,  and 
ataxia  of  the  limbs,  facial  paralysis,  difficulty  in  speech  and  in  swal- 
lowing have  occurred  in  the  severer  cases.  In  adults,  especially  in 
patients  who  are  alcoholics,  this  type  is  more  common  than  the  hemi- 
plegic  type.  It  may  develop  with  few  constitutional  symptoms  and 
even  without  fever.  In  this  respect  it  resembles  acute  anterior  polio- 
myelitis in  adults. 

In  still  another  group  of  cases  the  local  symptoms  are  those  of  an 
acute  bulbar  palsy.  Speech,  swallowing,  and  respiration  are  affected, 
the  various  characteristic  signs  of  glosso-labio-laryngeal  paralysis 
appear  simultaneously,  and  in  these  cases  a  fatal  result  is  more  com- 
mon than  recovery.  These  have  been  termed  polio-encephalitis 
inferior.  But  the  lesion  is  not  always  limited  to  the  gray  matter  of 
the  pons  and  medulla,  and  when  it  extends  to  the  tracts  passing 
through  them  the  symptoms  may  be  widespread.  Thus  various  forms 
of  alternating  hemiplegia  and  hemiansesthesia  or  general  paralysis  may 
be  the  result. 

The  combination  of  cerebral  with  spinal  symptoms  has  already  been 
mentioned. 

The  course  of  the  case  in  all  these  various  types  is  usually  one  of 
gradual  improvement,  and  a  final  recovery  with  a  few  local  symptoms 
remain.  Such  are  monoplegia,  sensory  defects  in  the  visual  field, 
paralysis  of  one  or  two  ocular  muscles,  or  epilepsy.  In  some  cases 
all  the  local  symptoms  disappear,  and  then  it  is  evident  that  few 
neurones  were  actually  destroyed  by  the  inflammation.  In  other  cases 
a  most  irregular  combination  of  symptoms  such  as  nystagmus,  defective 
articulation,  tremor,  and  some  form  of  paralysis,  remains,  and  the 
terminal  state  is  identical  with  that  of  multiple  sclerosis. 


TBEATMENT.  539 

Prognosis. — The  prognosis  during  the  acute  onset  must  be  guarded, 
as  death  sometimes  occurs.  It  will  depend  upon  the  severity  of  the 
symptoms,  especially  on  the  occurrence  of  convulsions  and  upon  the 
depth  of  the  coma,  the  degree  of  temperature,  the  condition  of  the 
pulse  and  respiration,  and  the  power  of  the  individual  to  combat  an 
acute  infection.  The  prognosis  after  the  acute  stage  has  passed  is 
favorable  for  recovery,  and  even  serious  local  symptoms  may  pass 
away.  The  prognosis  is  better  than  in  cerebral  hemorrhage,  embo- 
lism or  thrombosis,  as  the  focus  of  disease  is  usually  smaller  and  there 
is  not  such  a  gross  lesion.  Recurrence  of  the  disease  has  been  seen  in 
.three  cases  only  (Dinken,  Wiener,  and  Oppenheim). 

Treatment.  —  The  treatment  divides  itself  into  the  care  of  the 
patient  during  the  acute  stage  and  the  treatment  of  local  symptoms 
later.  At  the  outset  purgatives  may  be  given  and  ice  applied  to  the 
head,  and  antipyretics  may  be  used  freely,  antipyrine,  quinine,  salicin. 
Leeches  to  the  mastoid  region  are  to  be  applied  excepting  in  very 
anaemic  persons,  and  may  be  repeated  daily  during  the  febrile  stage. 
Hot  baths  with  mustard  may  be  given  to  children,  and  hot  foot  baths 
to  adults.  When  the  stage  of  onset  is  over  the  various  local  symptoms 
are  to  be  treated  in  the  same  manner  as  if  the  case  were  one  of  apo- 
plexy, or  of  ophthalmoplegia,  or  of  bulbar  palsy,  or  of  infantile  spinal 
paralysis. 


CHAPTEH  XXX. 

MENINGO-ENCEPHALITIS.     PAEESIS.     DEMENTIA  PARALYTICA. 

Paresis  or  dementia  paralytica  is  a  diffuse  degenerative  disease  of 
the  cerebral  cortex  characterized  by  mental,  motorj  sensory,  and  vaso- 
motor symptoms,  of  progressive  course  and  fatal  ending. 

Etiology.  —  Males  are  much  more  liable  to  paresis  than  females.^ 
Inherited  weakness  of  the  nervous  system  predisposes  the  individual 
to  the  development  of  paresis.  It  is  a  disease  of  early  adult  life,  the 
majority  of  cases  developing  between  the  ages  of  thirty  and  forty-five 
years  ;  but  no  age  is  exempt,  as  cases  have  been  reported  both  in  child- 
hood and  in  old  age.  Mott^  has  recently  reported  22  cases  of  juvenile 
general  paresis,  and  has  collected  75  other  cases.  It  is  evident,  there- 
fore, that  the  disease  may  develop  in  children.  In  the  majority  of 
these  cases  hereditary  syphilis  was  found  to  be  the  cause.  It  is  a  post- 
syphilitic disease  in  about  60  per  cent,  of  the  cases,  though  statistics 
gathered  from  54  different  authorities  by  Mott^  gave  a  varying  per- 
centage from  11  per  cent,  to  94  per  cent.  The  German  statistics  show 
80  per  cent,  of  the  cases  to  be  syphilitic,  and  recent  tests  of  the  spinal 
fluid  and  of  the  blood  have  demonstrated  the  presence  of  syphilis  in 
90  per  cent,  of  the  cases  according  to  Nonne.  But,  as  in  locomotor 
ataxia,  it  is  not  due  to  an  active  syphilitic  process,  since  the  patho- 
logical lesions  are  not  peculiarly  syphilitic,  and  it  is  not  arrested  by 
antisyphilitic  treatment.  The  interval  between  the  syphilitic  infection 
and  the  onset  of  paresis  is  usually  a  considerable  one  —  from  five  to 
twenty  years.  Mental  strain  and  anxiety  are  the  chief  exciting  causes 
of  the  disease.  The  tremendous  efforts  to  attain  financial  and  social 
success,  the  enormous  responsibilities  undertaken  by  ambitious  men, 
the  effort  to  carry  through  large  projects,  and  the  ceaseless  work  day 
and  night,  with  no  rest  and  little  sleep,  which  are  characteristic  of  life 
at  the  present  time  in  our  large  cities,  are  the  chief  factors  in  the  rapid 
increase  in  the  number  of  cases  of  paresis.  It  is  admitted  by  all  that 
it  is  a  disease  of  the  more  highly  civilized,  the  more  mentally  active 
type  of  men.  Hence,  the  higher  classes  are  more  liable  than  the 
lower,  and  those  who  lead  an  agricultural  life  are  almost  exempt. 
Peterson  states  that  in  Egypt  the  disease  is  very  rare,  though  syphilis 
is  prevalent  there.     The  Mohammedans  do  not  use  alcohol.     Alco- 

^  The  statistical  statements  in  this  chapter  are  based  on  the  analysis  of  3454  cases  in 
the  Berlin  Asylum  by  Junius  and  Amdt,  Arch.  f.  Psych.,  Bd.  XLR^.,  1908.  See  also 
Jolly,  Arch.  f.  Psych.,  Bd.  XLIV.,  p.  259,  and  upon  3,000  cases  analyzed  by  Clark  and 
Atwood,  Jour.  Nerv.  and  Ment.  Dis.,  Sept.,  1907. 

^Archives  of  Neurology  of  London  County  Asylum,  1899,  vol.  i.,  p.  250. 

^Loc.  cit.,  p.  169. 

540 


PATHOLOGY.  541 

holism  and  excess  in  sexual  indulgence  are  certainly  additional  factors 
in  its  prodijction.  Alcoholism  is  present  in  a  third  of  the  cases.  A 
certain  number  of  cases  seem  to  be  traceable  to  injuries  of  the  head 
and  to  sunstroke. 

Pathology.  —  The  disease  begins  with  changes  in  the  vessels, 
hypersemia  of  the  pia  and  small  cortical  vessels,  increase  of  nuclei  in 
the  vessel  walls,  occasional  stasis  in  the  capillaries,  and  exudation  of 
serum  into  the  lymph  spaces.  As  it  advances,  a  formation  of  fusi- 
form dilatations  of  the  vessels  and  the  development  of  fibrils  of  con- 
nective tissue  between  the  vessel  walls  and  the  surrounding  neuroglia 
occur.  In  the  neuroglia  at  the  same  time  a  marked  increase  of  the 
nucleated  cells,  with  numerous  branching  processes  is  in  progress, 
numerous  spider  cells  developing  throughout  the  cortex,  around  the 
vessels  and  lymph  spaces,  and  about  the  nerve  cells.  This  increase 
of  neuroglia  goes  on  rapidly,  producing  a  diflPuse  sclerosis  of  the  cortex, 
which  is  followed  by  retraction  of  the  tissue  leading  to  atrophic  shrink- 
ing. The  entire  brain  takes  part  to  a  lesser  degree  in  this  process.  In 
the  cerebral  tissue  at  the  same  time  there  is  in  progress  a  degeneration 
of  the  finest  nerve  fibrillse  and  of  the  branching  processes  of  the  nerve 
cells.  This  begins  in  the  tangential  fibres  of  the  first  layer  of  the  cor- 
tex, but  later  involves  all  the  layers  of  cells.  A  swelling  chromatoly- 
sis,  with  hyaline  or  fatty  degeneration,  vacuolization,  pigmentation, 
and  final  atrophy  of  the  cell  bodies  takes  place.  The  formation  of 
cystic  cavities  throughout  both  gray  and  white  matter  is  often  observed. 

Mott,^  whose  investigations  are  most  reliable,  believes  that  the  start- 
ing point  of  paresis  is  a  degeneration  in  the  neurones  of  the  cortex. 
This  is  attended  by  the  production  of  choline  (see  page  33)  which  can 
be  found  in  the  cerebro-spinal  fluid  in  paretics.  Choline  is  a  toxic 
substance  causing  a  depression  of  the  heart  and  a  lowering  of  arterial 
pressure.  Mott  believes  that  its  presence  causes  a  venous  stasis  in  the 
veins  of  the  brain,  especially  those  of  the  convexity  opening  into  the 
longitudinal  sinus,  in  which  the  blood  flows  contrary  to  gravity.  (See 
Fig.  214,  page  465.)  He  points  out  the  fact  that  it  is  in  the  domain 
of  these  veins  that  the  thickening  of  the  pia  arachnoid  occurs.  The 
choline  accumulates  in  the  perivascular  lymph  spaces  about  the  veins, 
causing  stasis  and  inflammation,  which  are  intensified  by  the  action  of 
gravity  in  the  veins  that  empty  into  the  longitudinal  sinus.  (Edema 
of  the  brain  follows,  and  this  is  always  present  in  paretic  brains.  The 
cerebro-spinal  fluid  contains  leucocytes  in  much  greater  number  than 
in  health. 

The  final  result  of  these  pathological  processes  in  the  vessels,  neu- 
roglia, and  cerebral  substance  is  a  gradual  atrophy  of  the  brain,  so 
that  it  weighs  much  less  than  normal,  sometimes  only  two-thirds  of  the 
normal,  the  left  hemisphere  being  more  atrophied  than  the  right  one ; 
it  appears  shrunken,  the  convolutions  being  narrow  and  the  sulci  open; 
the  brain  tissue  is  hard,  pale,  friable,  pigmented  and  on  the  summit  of 
the  convolutions  adherent  to  the  pia,  which  is  opaque  and  thickened. 
'  Aicliivurf  of  JNeuroiogy  of  London  County  Asylums,  vol.  i.,  p.  396. 


542  MENINGO-ENCEPEAIJTIS.     PARESIS. 

When  the  pia  is  stripped  oif,  portions  of  the  cortex  come  away  with  it, 
The  thickness  of  the  cortex  is  seen  to  be  much  reduced.  A  thickened 
condition  of  the  ependyma  of  the  ventricles  is  uniformly  found.  There 
is  usually  an  increase  of  fluid  within  the  ventricles  and  beneath  the  pia. 
As  a  complication  the  lesions  of  pachymeningitis  with  hsematoma  are 
not  infrequently  present.  All  of  the  changes  in  the  cortex  are  more 
marked  in  the  frontal  lobes  and  motor  area  and  about  the  fissures  of 
Sylvius,  but  in  the  later  stages  of  the  disease  no  part  of  the  cortex  is 
normal.  In  about  10  per  cent,  of  the  cases  spinal  lesions  are  found  in 
addition  to  the  cerebral  lesions.  These  consist  of  sclerosis  in  the  pos- 
terior and  lateral  columns  of  the  cord.  There  are  some  cases  in  which 
the  posterior  sclerosis  is  the  first  lesion  to  appear,  and  in  these  the 
lesions  of  locomotor  ataxia  are  well  marked,  as  already  described,  page 
339.  In  other  cases  they  appear  after  the  condition  of  paresis  has 
developed,  and  then  they  are  not  so  complete.  The  sclerosis  in  the 
lateral  columns  is  usually  due  to  secondary  degeneration  in  the  motor 
tracts  after  changes  in  the  motor  cells  of  the  cortex. 

Symptoms.  —  Paresis  begins,  as  a  rule,  very  gradually,  so  that 
many  cases  are  overlooked  and  others  are  considered  as  neurasthenia 
or  dyspepsia  for  some  months,  there  being  present  some  of  the  symp- 
toms of  one  or  both  of  these  diseases.  After  a  time  the  friends  of  the 
patient  begin  to  notice  that  he  is  not  acting  naturally,  that  he  is  unusu- 
ally irritable  or  excitable,  is  offended  or  delighted  at  slight  provoca- 
tion, is  variable  in  his  moods,  and  appears  at  times  inattentive,  forget- 
ful, and  careless  of  the  proprieties.  Soon  it  is  found  that  his  aifairs 
are  in  confusion,  his  accounts  not  properly  kept,  and  that  it  is  impos- 
sible to  keep  his  attention  concentrated  upon  important  business  or 
upon  any  train  of  thought,  so  that  his  conversation  becomes  noticeably 
fragmentary.  He  cannot  do  his  work,  and  yet  resents  any  interference 
with  it,  maintaining  that  he  is  perfectly  well.  He  will  admit  some 
confusion  of  thought,  some  cerebral  sensations  of  discomfort,  irritability 
and  sleeplessness.  His  memory  for  recent  occurrences  then  begins  to 
fail.  He  neglects  engagements ;  he  disregards  obligations,  sleeps  in 
company,  loses  his  temper,  and  uses  bad  language  at  home  or  in  public, 
neglects  his  family,  and,  though  previously  moral  in  conduct,  indulges 
in  alcoholic  and  sexual  excesses.  By  this  time  it  is  noticed  that  his 
speech  is  a  little  thick,  indistinct,  and  hesitating,  and  a  fine  tremor  of 
the  tongue  and  lips  and  possibly  of  the  hands  may  be  detected.  The 
affection  is  one  of  fine  co5rdmated  movements  —  a  cortical  ataxia  rather 
than  gross  paralysis.  The  pupils  are  often  unequal,  or  contracted,  or 
dilated,  and  react  very  slowly  to  the  light  in  the  early  stage  ;  and  soon 
fail  to  react  to  light  at  all,  though  they  react  in  accommodation.  The 
Argyll-Robertson  pupil  is  present  in  95  per  cent,  of  cases. 

During  the  period  of  invasion  headache  and  sleeplessness  are  some- 
times prominent  symptoms,  and  a  gradual  loss  of  weight  goes  on. 
From  the  outset  restlessness  is  noticeable,  and  after  a  time  it  seems 
impossible  for  the  patient  to  keep  quiet,  he  wants  to  take  all  kinds  of 
exercise,  to  walk  for  miles^  to  go  about  even  when  exhausted  by  fatigue. 


SYMPTOMS.  543 

His  intellectual  powers  are  notably  weakened,  he  is  no  longer  attentive, 
logical,  seif-controlled,  cautious,  or  careful  in  conduct,  and  the  change 
of  character  becomes  apparent  both  to  his  family  and  to  his  acquaint- 
ances. This  period  of  invasion  varies  from  one  to  three  years  and 
may  be  interrupted  by  periods  of  subsidence  of  many  of  the  symptoms. 
It  usually  goes  on  to  the  full  development  of  the  disease. 

The  prominent  symptoms  during  the  period  of  invasion  differ  some- 
what in  different  cases,  and  this  has  led  German  authors  to  distinguish 
between  various  types  of  the  disease,  (a)  They  find  the  demented 
type  the  most  common,  37  per  cent,  of  their  cases  belonging  to  it.  In 
this  type  there  is  a  slowly  advancing  mental  failure  with  imperfect 
power  of  attention  and  defects  of  memory  and  an  indifference  and 
apathy  as  the  more  prominent  mental  symptoms.  (6)  The  expansive 
type  is  next  most  common,  27  per  cent,  of  their  cases  being  thus 
classified.  In  this  type  the  patients  are  constantly  boasting  of  their 
physical  or  financial  well-being,  are  confident  of  their  own  capacity  and 
undertake  all  sorts  of  absurd  schemes.  Such  patients  if  wealthy  are 
liable  to  ruin  themselves  by  foolish  financial  schemes,  and  if  in  places 
of  power  are  able  to  do  much  harm  to  others  as  well  as  themselves. 
The  cause  of  many  failures  in  the  business  and  banking  world,  and  the 
wrecking  of  several  railway  systems  can  be  traced  to  beginning  paresis 
m  the  man  in  power,  (c)  The  depressed  type  is  the  third  in  frequency, 
17  per  cent,  belonging  to  it.  In  this  type  the  neurasthenic  symptoms 
are  attended  by  much  mental  depression  ;  self-accusation  and  suicidal 
impulses  being  common.  The  patient  is  distrustful  of  his  capacity,  is 
depressed  in  his  spirits,  takes  a  dark  view  of  his  affairs  and  is  reluctant 
to  undertake  responsibility,  appreciating  his  own  growing  incapacity. 
He  IS  worried  over  his  affairs,  even  without  cause,  and  is  apprehensive 
of  his  own  condition.  Many  such  cases  are  at  first  supposed  to  be 
cases  of  simple  melancholia  and  the  diagnosis  is  difficult  until  the 
physical  signs  of  paresis  appear,  {d)  The  fourth  type  is  the  agitated 
paretic  who  is  restless,  uneasy,  physically  overactive  and  mentally 
irritable.  Eight  per  cent,  belong  to  this  class.  These  patients  wear 
themselves  out  by  their  constant  activity,  (e)  The  remaining  11  per 
cent,  of  patients,  not  included  in  these  types,  present  a  mixture  of 
symptoms  which  prevent  their  classification.  And  however  the  disease 
may  have  begun,  it  is  not  uncommon  for  each  type  eventually  to  pre- 
sent many  symptoms  prominent  in  the  other  types. 

Clark  and  Atwood,'  in  an  analysis  of  3000  cases,  found  the  grandiose 
or  exalted  type  in  70  per  cent.,  the  demented  type  in  20  per  cent.,  and 
the  depressed  type  in  10  per  cent.  This  would  indicate  a  racial  dif- 
ference in  the  process  of  the  disease  in  the  different  countries. 

When  paretic  dementia  is  fully  developed  numerous  symptoms  pre- 
sent themselves  —  both  mental  and  physical. 

The  power  of  sensory  perception  is  not  impaired,  but  the  patient  is 
often  so  preoccupied  or  indifferent  that  he  does  not  notice  fully  or 
remember  the  subjects  to  which  attention  is  directed.     His  mind  passes 
'Jour.  Nerv.  and  M^nt.  Dis.,  Sept.,  1907, 


544  MENINGO-ENCEPHALITIS.     PARESIS. 

rapidly  from  one  thing  to  another,  and  he  does  not  perceive  anything 
accurately.  Thus  a  patient  will  forget  that  he  has  dined,  or  neglect  to 
put  on  some  of  his  clothes,  will  expose  his  person,  or  will  commit 
openly  small  thefts,  will  make  serious  mistakes  in  his  accounts  or  ap- 
pointments, merely  from  a  lack  of  careful  perception  and  consequent 
impairment  of  memory.  Illusions  and  hallucinations  occur  only  toward 
the  close  of  the  disease,  and  are  followed  by  a  marked  loss  of  power  of 
sensory  perception  when  the  dementia  becomes  extreme. 

The  power  of  logical  thought  is  impaired  very  early.  The  patient 
shows  impaired  judgment  from  the  outset,  takes  little  notice  of  impor- 
tant matters,  dwells  upon  trivial  things,  acts  without  consideration,  and 
indulges  in  extravagant  schemes,  reckless  expenditures,  and  excesses  of 
many  kinds  which  his  better  judgment  would  condemn.  He  may  be- 
come exceedingly  immoral,  all  restraints  of  society,  religion,  and  mor- 
ality being  wholly  neglected.  He  becomes  profane,  obscene,  and  tipsy. 
He  very  frequently  develops  a  delusion  of  grandeur,  says  that  he  never 
was  so  well  in  his  life,  believes  that  he  is  the  strongest,  the  brightest, 
the  most  powerful,  the  most  wealthy  of  men.  And  he  acts  at  times  in 
accordance  with  his  delusion,  undertaking  the  most  extravagant  and 
impossible  schemes  without  any  regard  for  his  actual  circumstances. 
Many  a  patient  involves  himself  and  others  in  financial  difficulties  by 
undertaking  colossal  combinations  and  extensive  business  organizations 
far  beyond  his  powers  or  means,  before  it  is  realized  that  these  schemes 
are  the  product  of  an  unbalanced  mind.  Yet  the  delusions  are  not 
carried  out  fully  in  his  actions,  and  he  mixes  ordinary  affairs  with 
them  in  an  incongruous  manner.  The  delusions  are  not  as  fixed  or 
as  systematized  as  in  paranoia.  The  development  of  such  delusions 
is  a  sure  sign,  in  Meynert's  opinion,  that  an  atrophy  of  the  brain  has 
begun. 

His  emotional  state  is  unstable.  He  has  little  control  over  its  mani- 
festations, can  be  moved  to  tears  or  to  laughter  in  the  course  of  an 
ordinary  conversation,  can  be  excited  or  depressed  by  suggestions.  He 
is  sometimes  depressed  for  days,  and  feels  discomfort,  but  does  not 
blame  himself,  and  in  the  midst  of  the  depression  may  become  excited. 
He  is  usually  sanguine  and  hopeful,  and  his  emotion  is  usually  in 
accordance  with  his  delusion,  one  of  exaltation.  At  times  or  on  pro- 
vocation the  excitement  may  become  maniacal,  and  outbursts  of  rage 
or  of  frenzy  are  not  infrequent  in  the  course  of  the  disease.  Hence 
a  paretic  dement  is  never  a  harmless  member  of  society,  but  requires 
to  be  watched.     He  rarely  shows  any  tendency  to  suicide. 

Voluntary  action  and  conduct  are  affected  from  the  outset ;  indeed 
it  is  by  change  in  conduct  that  the  changes  in  thought  and  character 
are  chiefly  betrayed,  and  the  weak  logical  power  made  manifest.  In- 
consistency is  evident  in  speech  and  conduct.  Acts  are  done  without 
consideration  ;  thus  impulsive  acts,  such  as  stealing,  forgery,  enarmous 
expenditures,  even  murder  may  be  performed  under  sudden  excitement 
or  under  the  influence  of  a  delusion,  or  in  a  fit  of  frenzy.  All  the 
ordinary  restraints  to  conduct  seem  to  be  removed,  and  instinct  rather 
than  morality  is  the  guide,  self-control  being  greatly  weakened. 


SYMPTOMS.  545 

Self-consciousness  appears  to  be  very  imperfect.  The  patient  does 
not  appreciate  the  inconsistency  between  his  previous  personality  and 
his  present  acts  ;  he  does  not  notice  the  growing  anxiety  of  his  family ; 
he  regards  with  indifference  loss  of  means,  or  even  his  confinement  in 
an  asylum ;  he  is  usually  so  engaged  in  his  imaginary  undertakings 
under  the  influence  of  his  delusions  that  he  is  contented  wherever  he 
is.  His  personality  may  be  changed,  and  he  may  imagine  himself  a 
prince,  a  millionaire,  a  deity.  Gradually  the  consciousness  becomes 
obscured,  he  recollects  little  regarding  his  illness,  and  as  the  deeper 
dementia  ensues  he  becomes  indifferent,  dull,  stupid,  and  finally  almost 
stuporous.  In  the  final  condition  of  dementia  all  mental  action  is 
suspended. 

The  physical  symptoms  are  as  marked  as  the  mental  symptoms,  and 
in  many  cases  are  the  first  to  appear. 

Motor  disturbances  appear  early.  There  is  restlessness,  a  tendency 
to  be  in  constant  motion.  The  patient  takes  long  walks  or  rides, 
indulges  in  unusual  exercise,  wishes  to  be  going  out  to  theatres  every 
night,  to  visit  friends  all  the  time,  and  is  not  content  to  lead  an  ordi- 
nary quiet  life,  as  before  his  illness.  He  has  a  sense  of  physical  power, 
and  is  eager  to  show  his  strength,  though  this  may  really  be  impaired 
He  is  very  talkative,  discussing  subjects  of  which  he  knows  little,  or  talk- 
ing at  random.  Tremor  of  the  muscles  appears  early,  first  in  the 
tongue,  then  in  the  face  and  hands,  and  finally  any  motion  is  attended 
with  some  trembling.  This  tremor  is  followed  by  incoordination, 
which  shows  itself  in  thickness  and  indistinctness  of  speech,  in  irregu- 
larity of  handwriting,  in  clumsiness  in  handling  things,  and  an  awk- 
ward, unsteady,  stumbling  gait.  The  speech  is  quite  characteristic, 
letters  being  slurred  and  words  mispronounced,  so  that  any  long  word 
or  combination  of  difficult  syllables,  like  "  third  artillery  brigade," 
cannot  be  said.  In  writing  the  patient  will  omit  letters  from  words, 
later  may  write  wholly  unintelligible  phrases,  or  even  merely  make 
marks  on  the  paper,  and  yet  not  appreciate  these  defects  of  writing. 
From  the  first  his  handwriting  is  changed,  lines  being  irregular,  letters 
too  small  or  too  large,  and  badly  written.  The  facial  expression  be- 
comes blank  and  inane  —  no  appearance  of  thought  or  interest  being 
manifest,  but  when  he  talks  an  excessive  play  of  the  facial  muscles  is 
noticeable.  Paresis  shows  itself  after  a  time  in  the  limbs,  and  pro- 
gresses until  in  the  final  stage  there  is  total  paralysis,  with  increased 
reflex  action  and  loss  of  all  control  over  bladder  and  rectum.  Finally 
speech  may  become  impossible.  The  knee-jerks  are  either  much 
increased  or  are  lost  from  the  outset.  In  60  cases  observed  in  my 
clinic  the  knee-jerks  were  increased  in  53  per  cent,  and  lost  in  30  per 
cent.^  which  corresponds  exactly  with  the  statistics  of  the  German 
authors  based  on  992  cases. 

In  the  course  of  the  disease  epileptiform  attacks,  attacks  of  severe 

vertigo,  attacks  of  sudden  loss   of  consciousness  without  convulsions, 

and  attacks  of  monoplegia  or  hemiplegia,  temporary  or  permanent, 

often  occur.     They  may  be  the  initial  symptoms  of  the  disease.     They 

36  ^  E.  L,  Hunt,  New  York,  Medical  Keeord,  January,  1905. 


546  PARESIS.    DEMENTIA  PARALYTICA. 

occur  in  one  half  of  the  patients  at  some  time  in  the  course  of  the 
affection. 

Vasomotor  disturbances  are  also  present.  The  face  flushes  or  pales 
frequently,  there  are  sudden  attacks  of  vertigo  and  of  headache,  and 
of  feeling  of  fulness  in  the  head ;  the  pulse  is  usually  slow,  large,  and 
of  low  tension.  In  the  late  stages  venous  congestions  in  various  organs 
are  found,  and  hsematoma  of  the  ears. 

Lumbar  puncture  reveals  leucocytosis  of  the  spinal  fluid,  as  many  as 
100  leucocytes  being  found  in  3  c.c.  of  fluid.  This  is  an  early  symp- 
tom and  of  importance  in  diagnosis. 

Sensory  symptoms  are  less  marked  than  motor  symptoms,  but  as  the 
disease  advances  anaesthesia  or  analgesia  of  the  limbs  may  be  found, 
especially  in  connection  with  hemiplegia. 

In  the  last  stage  trophic  disturbances  occur,  for  in  the  stupid,  dirty, 
helpless  state  of  the  patient  cleanliness  is  diflicult  and  the  liability  to 
bed-sores  and  to  cystitis  is  great. 

Some  cases  are  complicated  by  the  development  of  spinal  sclerosis, 
either  in  the  form  of  posterior  or  of  lateral  sclerosis,  with  its  attendant 
symptoms,  and  in  a  few  cases  the  paresis  follows  the  spinal  disease. 

It  is  evident  that  the  mental  and  physical  symptoms  are  very  numer- 
ous. Many  cases  show  only  some  of  them  ;  others  present  all  the 
symptoms  during  the  course.  In  some  cases  the  mental  symptoms 
appear  early  and  are  more  prominent  throughout  than  the  physical 
symptoms  ;  in  other  cases  the  reverse  is  observed.  A  few  cases  begin 
with  epileptiform  attacks.  In  other  cases  the  symptoms  begin  with  a 
hsematoma  of  the  dura,  causing  an  attack  of  apoplexy  with  aphasia,  or 
hemiplegia,  or  hemianopsia.  These  symptoms  subside  rapidly,  so  that 
in  a  week  or  two  the  patient  may  appear  to  have  recovered.  But  later 
the  mental  and  physical  symptoms  of  paresis  appear,  and  then  it  is 
evident  that  the  apoplexy  was  the  first  sign  of  paresis. 

The  course  of  the  disease  is  slowly  progressive,  though  remissions 
of  one  or  two  years  are  not  uncommon.  Its  average  duration  is  about 
three  years,  though  rapid  cases  may  terminate  within  a  year,  and  some 
are  known  to  have  lasted  five  or  six  years.  According  to  the  German 
statistics  19  per  cent,  die  in  the  first  year  of  the  disease ;  26  per  cent, 
in  the  second  year;  27  per  cent,  in  the  third  year;  12  per  cent,  in  the 
fourth  year ;  6  per  cent,  in  the  fifth  year  and  7  per  cent,  in  the  sixth 
or  following  years.  No  cases  outlived  the  tenth  year.  In  private 
practice  the  duration  of  the  disease  is  longer  than  in  asylums  ;  as  great 
care  can  be  taken  of  patients  and  fatal  accidents  avoided.  The  patients 
die  of  exhaustion  or  of  some  complicating  disease,  such  as  cystitis,  or 
pneumonia,  or  obstruction  of  the  bowels. 

Diagnosis. — The  diagnosis  of  paresis  is  to  be  made  from  the  combi- 
nation of  physical  signs  and  mental  and  physical  symptoms.  The  loss 
of  pupil  reflex  to  light,  unequal  pupils,  tremor  of  the  face  and  tongue, 
indistinct  speech,  tremor  of  the  hands,  exaggeration  or  loss  of  the  knee- 
jerks  and  spinal  leucocytosis  are  objective  physical  signs  of  importance. 
The  mental  irritability,  excitement  with  expansive  ideas,  defects  of 
memory  and  of  self-control  are  the  most  important  early  mental  symp- 


PROGNOSIS.  547 

toms.    Attacks  of  epileptic  or  apoplectic  nature  are  the  most  important 
physical  symptoms. 

Neurasthenia  may  be  present  in  the  early  stage  of  paresis,  and  any 
or  all  of  its  symptoms  may  then  be  apparent.  But  a  neurasthenic 
patient  always  notices  every  symptom  minutely,  describes  it  fully,  and 
discusses  its  significance,  while  a  paretic  is  usually  not  aware  of  the 
fact  that  he  is  ill,  and  does  not  care  to  talk  about  his  health  as  much 
as  about  his  projects  and  success.  Defects  of  memory  and  of  speech 
are  rare  in  neurasthenia,  and  the  neurasthenic  presents  none  of  the 
physical  signs  of  paresis.  In  a  doubtful  case  it  is  on  the  appearance 
of  these  signs  only  that  the  diagnosis  of  paresis  can  be  made. 

There  are  some  cases  of  cerebral  syphilis  which  resemble  paresis 
and  it  is  often  difficult  in  the  early  stage  to  differentiate  these  diseases. 
Cerebral  syphilis  usually  causes  pain  in  the  head,  worse  at  night.  If 
it  produces  disturbance  of  speech  it  is  rather  in  the  form  of  true  aphasia 
not  the  tremulous  indistinct  speech  of  paresis.  There  is  never  any 
tremor  of  face  and  hands,  and  hence  writing  is  clear  and  not  impaired 
unless  there  is  agraphia  with  aphasia,  and  this  differs  from  the  omis- 
sion of  letters  and  words  in  writing  present  in  paresis.  Optic  neuritis 
is  often  present  in  cerebral  syphilis,  but  very  rarely  in  paresis.  The 
emotional  state  is  usually  depressed  and  the  patient  anxious  in  syphilis, 
while  it  is  one  of  excitement  and  elation  in  paresis.  Delusions  are 
much  less  common  in  syphilis,  and  are  never  as  extreme  as  in  paresis. 
The  signs  of  dementia  are  more  marked  in  an  early  stage  than  they  are 
in  paresis  ;  the  lack  of  memory,  lack  of  power  of  reasoning,  and  lack 
of  self-control  appearing  early  in  syphilis  and  late  in  paresis.  In 
syphilis  there  are  often  physical  signs  of  single  or  multiple  localizable 
lesions,  while  paresis  is  a  diffuse  disease.  The  course  of  the  case  in 
syphilis  is  variable,  with  much  improvement  under  treatment,  while  in 
paresis  it  is  usually  progressive. 

Multiple  sclerosis  can  hardly  be  mistaken  for  paresis  ;  as  the  nystag- 
mus, tremor  of  an  increasing  kind  on  effort,  tremor  of  the  head  and 
trunk,  and  absence  of  delusions  even  when  some  dementia  is  present 
are  characteristic  of  multiple  sclerosis. 

The  dementia  of  old  age  and  the  dementia  of  chronic  alcoholism  are 
not  attended  by  delusions  of  exaltation  and  grandeur. 

Prognosis.  —  The  prognosis  in  paresis  is  absolutely  bad.  Krafft- 
Ebing  declares  that  he  has  never  seen  a  recovery  in  2500  cases.  It 
must,  however,  be  remembered  that  remissions  in  the  symptoms  occur, 
especially  in  the  early  stage.  I  have  known  many  patients  who  have 
had  all  the  symptoms,  to  recover  for  a  time  under  quiet  rest  in  the 
country,  and  be  able  to  return  to  business.  Such  remission  may  last 
for  a  year  or  two,  during  which  time  the  only  evidence  of  the  disease 
may  be  the  inactive  pupil,  and  the  slight  tremor  of  the  hands,  and  the 
exaggerated  or  lost  knee-jerk.  I  have  never  known  these  physical 
signs  to  subside,  and  I  have  never  known  a  remission  to  last  more  than 
two  years.     Hence,  the  prognosis  as  to  recovery  is  bad. 

The  prognosis  as  to  the  duration  is  different  in  the  different  types  of 
the  disease.     In  the  agitated  type  it  runs  a  rapid  course  and  death 


548  PARESIS.    DEMENTIA  PARALYTICA. 

occurs  within  the  second  year.  The  duration  is  somewhat  longer  in 
the  depressed  type.  In  the  expansive  type  the  chance  of  life  is  better 
than  in  either  of  these.  The  demented  paretic  lives  the  longest,  often 
for  four  or  five  years  after  the  disease  is  fully  declared.  The  prognosis 
is  also  better  in  the  mixed  type  than  in  the  three  first  mentioned.  It  has 
been  noticed  that  those  who  were  predisposed  to  the  disease  by  a  bad 
inheritance  lived  longer  when  the  disease  developed  than  those  whose 
inheritance  was  good. 

Treatment. — There  is  no  form  of  treatment  which  will  arrest 
paresis.  As  the  majority  of  patients  are  syphilitic  a  course  of  treat- 
ment by  salvarsan,  or  by  mercury  and  iodide  of  potassium  is  advisable, 
this  treatment  being  assisted  by  the  use  of  daily  hot  (106°  F.)  baths  for 
half  an  hour.  In  some  cases  I  have  seen  material  benefit  as  a  result,  and 
a  remission  of  some  months  in  the  symptoms  usually  follows  such  a 
course.  It  is  absolutely  necessary  for  the  patient  to  stop  all  business, 
to  rest  and  to  avoid  emotional  excitement.  Hence,  it  is  well  to  remove 
him  from  his  home  surroundings,  and,  as  he  is  not  fit  to  travel,  it  is 
better  to  send  him  to  a  sanitarium,  or  to  some  health  resort,  or  some  one 
of  the  hot  springs,  where  water  treatment  can  be  given.  Some  forms 
of  hydrotherapy  are  of  service  in  these  cases,  but  cold  batlis  and  douches 
are  to  be  avoided.  A  tub  bath,  or  a  hot-air  box,  followed  by  a  shower 
not  below  80°  F.,  and  massage  daily  appears  to  exert  a  favorable  effect 
upon  the  circulation  and  to  quiet  many  nervous  symptoms.  When  the 
patient  becomes  uncontrollable  by  his  family  or  nurses  it  is  better  to 
commit  him  to  an  asylum  where  he  may  improve,  so  that  in  a  period 
of  remission  he  may  be  released  and  allowed  to  travel.  The  quiet 
routine  life  of  an  institution,  without  excitement  or  responsibility,  seems 
favorable  to  the  subsidence  of  the  symptoms  of  irritation. 

In  the  later  stages  of  the  disease,  when  dementia  is  well-marked, 
care  in  an  asylum,  or  in  a  home  well  secluded  and  provided  with  skilled 
nurses,  may  prolong  life  for  many  months. 

Some  of  the  symptoms  require  active  treatment.  Sleeplessness  must 
be  treated  by  hot  baths  at  night,  or  massage,  or  by  the  use  of  paralde- 
hyd  in  60-grain  dose,  or  trional  20  grains,  or  sulphonal  15  grains.  It 
is  well  to  give  hypnotics  with  hot  milk,  as  they  are  more  rapidly 
absorbed  and  more  efficacious.  The  motor  restlessness  leads  to  over- 
fatigue and  heart  failure  ;  hence  very  many  short  walks  in  a  day,  each 
followed  by  a  short  rest,  are  better  than  the  long  tramps  which  these 
patients  are  proud  of  taking.  When  the  mental  excitement  becomes 
very  intense,  and  the  patients  cannot  be  controlled  by  argument,  active 
purgatives,  such  as  compound  cathartic  pills,  aloes,  or  croton  oil,  which 
cause  a  temporary  anaemia  of  the  brain,  will  quiet  them  better  than 
sedatives.  An  occasional  use  of  bromide,  or  bromide  and  chloral,  or 
even  a  hypodermic  injection  of  morphine  ^,  or  hydrobromate  of 
hyosciney^-g-  grain,  may,  however,  be  necessary  to  allay  excitement. 
A  single  dose  of  fluid  extract  of  ergot,  one  drachm,  may  have  the  same 
effect,  but  the  continued  use  of  ergot  is  to  be  avoided. 


CHAPTER   XXXI. 


ABSCESS  OF  THE  BEAIN. 


Etiology. — Abscess  of  the  brain  occurs  as  a  sequel  to  injuries  of 
the  head,  with  and  occasionally  without  fracture.  In  cases  of  com- 
pound fracture  or  of  wounds  in  which  a  septic  infection  occurs  at  the 
time  of  the  injury  such  abscess  formation  is  to  be  expected.  This  is 
particularly  true  if  a  small  bit  of  bone  or  a  foreign  body  remains  in 
the  brain.  In  cases  where  the  scalp  is  only  bruised  it  is  difficult  to 
understand  the  source  of  infection.  Von  Bergman  declares  that  a 
simple  contusion  of  the  head  is  not  capable  of  causing  an  abscess,  but 
I  have  seen  it  in  several  cases  where  the  scalp  was  not  broken,  though 
the  bone  was  fractured.  In  some  cases  the  abscess  follows  soon  after 
the  injury;  in  other  cases  the  symptoms  do  not  develop  for  many 
weeks  or  months ;  and  cases  are  on  record  where  the  only  explanation 
for  the  presence  of  a  latent  abscess  found  unexpectedly  at  the  autopsy 
was  an  injury  received  years  before. 


Fig.  238. 


Large  abscess  in  the  inferior  parietal  region  secondary  to  fracture  of  the  skuU.    The  thick 
capsule  of  the  abscess  can  be  seen. 

In  the  case  of  an  infant  seen  with  Poore  at  St.  Mary's  Hospital,  a 
fall  upon  the  left  parietal  bone,  causing  a  laceration  of  the  scalp  and 
an  indentation  of  the  soft  bone,  was  followed  within  two  weeks  by  the 
development  of  right  hemiplegia  with  hemianopsia.  At  the  autopsy  a 
large  cerebral  abscess  was  found  beneath  the  site  of  the  injury  within 

549 


550 


ABSCESS  OF  THE  BRAIN. 


the  white  matter  of  the  brain,  there  being  no  apparent  affection  of  the 
cortex  for  one-fourth  of  an  inch  above  the  abscess.  The  abscess  had 
a  thick  capsule  and  was  well  defined.  (See  Fig.  238.)  An  attempt 
was  made  to  open  it,  but  the  operation  had  to  be  suspended  on  account 
of  the  collapse  of  the  patient. 


Fig.  239. 


Abscess  of  the  temporal  lobe  after  otitis  media.     (Larkin.) 


In  a  second  case,  seen  with  McBurney  at  Roosevelt  Hospital,  a 
compound  fracture  of  the  superior  portion  of  the  left  parietal  bone, 
which  had  healed,  had  been  followed  three  months  later  by  the  gradual 
development  of  paralysis  of  the  right  leg.  At  the  operation  a  large 
abscess  was  found  involving  the  superior  parietal  lobule  and  adjacent 
portion  of  the  posterior  central  convolution.  In  the  midst  of  the 
abscess  a  considerable  piece  of  bone  was  found,  which  evidently  had 
been  driven  in  at  the  time  of  the  fracture.  The  operation  was 
successful.  The  patient  recovered  and  gradually  regained  power 
in  the  leg. 


PLATE   XXIV 


Abscess  of  Cerebellum  Secondary  to  Chronic  Suppurative  Otitis  Media. 

(Bacon.) 

The  right  cerebellar  hemisphere  has  been  divided  and  the  lower  half  removed, 
exposing  the  abscess  cavity  and  its  wall  {a)  and  the  area  of  hemorrhagic  softening 
posterior  to  the  abscess  {b). 


ETIOLOGY. 


551 


,  In  a  third^case,  also  seen  with  McBuj-ney  at  Roosevelt  Hospital,  a 
similar  compound  depressed  fracture,  with  spicula  of  bone  in  the  brain, 
had  led  to  the  development  of  an  abscess  within  two  weeks  of  the  time 
of  fracture.  The  abscess  cavity  invaded  the  middle  third  of  the  motor 
area,  and  caused  hemiplegia  with  paralysis  chiefly  marked  in  the  arm, 
and  here,  too,  at  the  time  of  operation,  pieces  of  bone  had  to  be 
removed  as  well  as  the  purulent  accumulation  around  them.  The 
operation  was  successful  and  the  boy  recovered. 

In  a  fourth  case,  seen  with  McCosh  at  the  Presbyterian  Hospital,  a 
fracture  of  the  skull  over  the  left  occipital  region  had  produced  well- 
marked  right  homonymous  hemianopsia.  Trephining  resulted  in  the 
successful  evacuation  of  the  abscess,  but  the  hemianopsia  remained 
permanently. 

In  22  cases  of  abscess  of  the  brain  observed  at  the  Presbyterian 
Hospital,  12  have  been  due  to  trauma. 

The  second  and  probably  the  most  frequent  cause  of  brain  abscess 
is  chronic  otitis  media.  A  very  thin  layer  of  bone  separates  the  cavity 
of  the  middle  ear  from  the  dura  and  brain.  It  is  easy  to  conceive 
that  a  slight  degree  of  caries  in  this  bone  will  open  a  way  for  the 


Fig.  240. 


1.  Primary  abscess  cavity  connected  with  sinus  in  tympanic  roof.    2.  Secondary  abscess  cavity  in 
temporo-sphenoidal  lobe.    Both  abscess  cavities  found  free  from  pus  at  autopsy.     (Bacon, ) 

entrance  of  microorganisms,  and  their  development  will  lead  to  either 
purulent  meningitis  or  to  the  formation  of  an  abscess.  Such  an  ab- 
scess may  develop  in  the  temporal  lobe  of  the  brain  or  in  the  lateral  lobe 
of  the  cerebellum.     Fig.  240  and  Plate  XXIV.  show  such  abscesses. 

The  first  attack  of  otitis  media  is  rarely  followed  by  a  brain  abscess. 
It  is  in  the  chronic  cases,  where  as  time  has  gone  on  the  bone  has  been 
slowly  eroded,  that  a  sudden  attack  of  inflammation  of  the  ear  goes 
on-to  the  formation  of  the  brain  abscess. 


552  ABSCESS  OF  THE  BRAIN. 

Many  cases  in  adult  life  are  traced  to  infectious  otitis  media  occur- 
ring in  childhood,  the  frequency  of  this  complication  of  scarlatina  and 
measles  being  well  known. 

In  all  such  cases  there  is  a  history  of  a  chronic  purulent  discharge 
from  the  ear,  which  from  time  to  time  ceases,  but  recurs,  and  then 
suddenly  an  acute  attack  of  pain  in  the  ear  occurs,  which  is  soon  fol- 
lowed by  the  cerebral  symptoms  of  abscess.  In  other  cases  the  acute 
attack  of  otitis  subsides,  but  the  patient  does  not  feel  quite  well,  and 
after  some  weeks  the  signs  of  a  brain  abscess  occur. 

Korner  has  shown  that  such  abscesses  are  more  common  on  the 
right  side.  Barr  in  a  collection  of  76  such  abscesses  found  55  in  the 
temporal  lobe,  13  in  the  cerebellum,  2  in  the  pons,  and  1  in  the  crus. 
Poulsen  collected  13  cases  of  abscess,  of  which  9  were  in  the  temporal 
lobe  and  4  in  the  cerebellum.  Between  1900  and  1905  82  abscesses 
secondary  to  otitis  media  have  been  published  ;  of  these  54  were  in  the 
temporal  lobe,  25  in  the  cerebellum  and  2  in  the  occipital  lobe,  and  1 
in  the  third  frontal  convolution. 

A  third  cause  of  brain  abscess  is  a  carious  process  starting  in  the 
nasal  cavity  or  orbit,  or  an  infection  after  some  operation  upon  these 
parts.  Thus,  some  years  ago,  before  the  importance  of  aseptic  surgery 
was  appreciated,  I  saw  a  case  of  abscess  of  the  frontal  lobe,  with 
sudden  death,  which  followed  within  a  week  of  an  operation  for  the 
removal  of  an  exostosis  of  the  septum.  I  have  known  of  several 
similar  cases,  and  Kahnt  and  Dreyfuss  have  collected  a  number. 

Abscess  of  the  brain  has  been  known  to  follow  erysipelas  of  the 
face  and  head. 

Metastatic  abscess  in  the  brain  after  abscess  and  gangrene  of  the 
lung  is  not  very  common,  but  occurs.  Nahter  has  collected  8  such 
cases  in  a  study  of  100  cases  of  pulmonary  gangrene.  It  may  also 
follow  bronchiectatic  abscess,  empyema  and  pyaemia,  ulcerative  endo- 
carditis, and  abscess  in  any  organ.  In  these  cases  there  are  small 
multiple  abscesses  in  the  brain,  whose  existence  is  usually  overlooked, 
as  the  general  symptoms  of  the  original  disease  obscure  the  cerebral 
symptoms. 

It  has  been  supposed  that  in  some  cases  of  unknown  origin  the  cere- 
bral abscess  is  the  result  of  some  infection,  and  many  forms  of  infec- 
tious disease  —  typhus,  typhoid,  cerebro-spinal  meningitis,  measles, 
influenza,  and  tuberculosis  have  been  considered  the  starting  point  of 
cerebral  abscess  in  various  reported  cases. 

Pathology. — Abscess  in  the  brain  is  the  result  of  a  purulent  enceph- 
alitis. Streptococcus  pyogenes,  staphylococcus  pyogenes  aureus,  albus 
and  citreus,  and  pneumococcus  have  been  found  in  such  abscesses.  It 
may  present  several  different  appearances.  There  may  be  a  small, 
localized,  softened  condition  of  the  brain  tissue,  in  which  on  micro- 
scopic examination  leucocytes,  pus  cells,  and  microorganisms  are  found. 
This  is  the  condition  in  metastatic  abscesses.  Von  Bergman  reports 
a  case  in  which  100  such  little  collections  of  pus  were  found  in  one 
brain  in  a  patient  who  had  pyaemia  after  gangrene  of  the  leg. 


SYMPTOMS.  553 

There  may  be  a  collection  of  pus  within  an  irregular  cavity  without 
distinct  wall,  but  surrounded  by  a  more  or  less  pulpy,  broken  down, 
and  hemorrhagic  area  of  brain  tissue.  The  pus  is  green  or  brown  and 
fetid.  Such  abscesses  extend  rapidly  and  lead  to  a  fatal  termination. 
Even  if  evacuated  and  drained  they  are  likely  to  go  on,  and  very  often 
set  up  a  meningitis. 

There  may  be  a  collection  of  pus  enclosed  in  a  thick  connective- 
tissue  capsule  lying  in  the  white  matter  of  the  brain,  and  quite  distinct 
from  the  brain  tissue.  This  form  occasionally  shows  a  tendency  to 
rapid  progress,  but  is  usually  of  slow  growth.  It  usually  lies  in  or  on 
the  brain  like  a  tumor,  causing  symptoms  of  pressure  ;  or  it  may  cause 
no  symptoms  whatever,  and  surprise  the  pathologist  at  the  autopsy. 
This  form  is  probably  a  terminal  result  of  the  second  form  described, 
nature  forming  a  protecting  capsule  to  the  disintegrated  tissue  and 
shutting  up  the  pus  in  the  thick  wall ;  but  such  a  process  may  go  on 
with  some  rapidity,  as  I  have  seen  a  thick  wall  in  a  case  of  abscess  of 
but  three  weeks'  duration.  The  existence  of  the  wall  does  not  pre- 
clude the  possibility  of  an  extension  of  the  abscess,  and  in  such  cases 
an  infiltration  of  the  brain  tissue  about  it  with  pus,  leucocytes  and 
bacteria  has  been  found.  The  size  of  such  an  abscess  varies.  It  is 
usually  about  as  large  as  a  walnut,  but  may  be  much  larger.  The 
rupture  of  such  a  capsule  may  cause  sudden  death,  as  in  one  of  my 
patients.  It  may  rupture  inward  into  the  ventricle  or  outward  on  to 
the  surface.     It  is  not  possible  for  an  abscess  to  be  absorbed. 

In  many  cases  of  abscess  there  occurs  a  complicating  meningitis  or 
thrombosis  of  the  cerebral  sinuses  just  prior  to  death. 

Symptoms. — The  general  symptoms  of  cerebral  abscess  are,  in  the 
order  of  their  importance  :  1 .  Headache,  general  but  sometimes  located 
over  the  seat  of  the  abscess.  2.  A  change  in  mental  characteristics, 
of  the  nature  of  irritability,  alternating  with  dulness,  imperfect 
attention,  slowness  of  thought,  and  defects  of  memory  —  a  semicoma- 
tose condition  and  appearance  of  illness  out  of  proportion  to  the  other 
symptoms.  3.  Abnormal  temperature.  4.  Changes  in  the  blood. 
5.  Tenderness  of  the  head  to  percussion,  with  change  in  the  percussion 
note  over  the  site  of  the  abscess.  6.  Facial  palsy  of  the  peripheral 
type  upon  the  side  effected.  7.  Optic  neuritis.  8.  General  febrile 
symptoms  with  occasional  chills.  The  development  of  these  symptoms 
in  a  patient  who  had  had  an  injury  of  the  head,  or  is  the  subject  of 
chronic  otitis  media,  or  who  has  been  exposed  to  any  of  the  causes 
already  mentioned,  should  awaken  grave  anxiety. 

It  will  be  noticed  that  none  of  these  symptons  are  distinctly  diag- 
nostic of  brain  abscess,  for  all  of  them  may  occur  in  meningitis  or  in 
thrombosis  of  the  lateral  sinus,  and  all  of  them  may  vary  in  severity  in 
any  given  case.  I  have  seen  such  a  combination  of  symptoms  in  patients 
with  otitis  media  purulenta  who,  by  proper  surgical  treatment,  have 
gone  on  to  recovery  without  any  subsequent  evidences  of  brain  abscess. 

Thus  in  two  of  the  cases  of  traumatic  abscess  of  the  brain  already 
mentioned  headache  was  not  present.     In  a  case  of  abscess  of  the  cere- 


554  ABSCESS  OF  THE  BRAIK. 

bellum  which  I  saw  with  Gorham  Bacon/  headache  was  not  a  very 
marked  symptom  until  late  in  the  disease,  some  time  after  the  begin- 
ning of  the  abscess  ;  and  in  a  case  of  abscess  subsequent  to  ear  disease, 
seen  with  McBurney,  headache,  though  present,  was  never  very  severe. 
In  other  patients,  however,  I  have  known  it  to  be  intense,  so  that  it 
formed  the  chief  complaint.  It  is  evident,  therefore,  that  from  the 
degree  of  headache  no  conclusion  can  be  arrived  at  that  is  of  diagnostic 
value.  It  is  usually  stated  that  the  headache  in  meningitis  is  more 
severe  and  constant  than  it  is  in  abscess ;  but  if  all  degrees  of  headache 
may  occur  in  abscess,  it  is  evident  that  this  symptom  cannot  be  relied 
upon  in  diagnosis. 

It  is  equally  difficult  to  estimate  the  real  importance  of  mental  symp- 
toms. In  the  first  place,  the  mental  capacity  and  characteristics  of 
different  individuals  are  so  diiFerent  that  it  is  necessary  to  have  known 
the  person  prior  to  his  illness  in  order  to  reach  any  definite  judgment. 
A  condition  of  excitement  and  talkativeness  in  a  person  who  is  ordi- 
narily reserved  or  stolid  is  of  much  more  importance  than  in  an  excit- 
able and  loquacious  individual ;  and,  vice  versa,  a  condition  of  apathy 
and  stupor  in  a  man  of  bright,  active  intelligence  is  of  much  greater 
diagnostic  value  than  in  a  dull  or  stupid  person.  Anyone  who  watches 
a  large  class  going  through  gymnastic  movements  under  the  leadership 
of  an  instructor  will  notice  that  a  certain  proportion  are  quite  percep- 
tibly behind  the  others  in  their  motions,  and  even  in  a  class  of  fifty  it 
is  rare  to  ^nd  any  two  who  are  moving  exactly  in  time,  especially  when 
the  exercise  performed  is  a  novel  one.  This  fact  illustrates  the  varia- 
bility of  the  personal  equation  —  the  diiFerent  rate  that  obtains  in  the 
thinking  process  of  different  individuals — and  unless  this  fact  is  kept 
in  mind  in  judging  of  the  mental  state,  of  the  slowness  of  thought,  or 
of  the  defects  of  attention  in  a  person  who  is  ill,  very  erroneous  con- 
clusions may  be  reached  regarding  their  significance.  The  effect  of 
illness  upon  mental  processes  is  not  always  appreciated,  and  varies 
much  with  the  individual,  some  persons  becoming  restless,  irritable,  and 
more  active  mentally  when  ill,  others  becoming  lethargic,  morose,  and 
apparently  dull.  Inasmuch  as  these  variations  are  observed  in  all 
forms  of  illness,  it  is  obviously  erroneous  to  attach  much  differential 
diagnostic  value  to  the  mental  state  in  distinguishing  between  three  con- 
ditions—  abscess,  meningitis,  and  sinus  thrombosis — so  closely  allied. 

The  course  of  the  temperature  in  abscess  of  the  brain  is  quite 
variable.  The  statement  has  been  made  by  MacEwen,^  and  seems  to 
be  borne  out  by  his  cases,  that  it  is  common  in  abscess  of  the  brain  to 
find  a  persistently  low  temperature  with  little  variation.  MacEwen 
states  that  during  the  preliminary  period  the  temperature  has  been 
slightly  above  normal.  During  the  period  of  full  development  of  the 
abscess  the  temperature  is  about  normal  or  slightly  subnormal,  from 
97°  to  99°  F.;  and  that  in  the  terminal  stage  if  the  abscess  bursts  the 
temperature  rises  within  a  few  hours  with  a  bound  to  104°  or  105°  F., 

1  American  Journal  of  the  Medical  Sciences,  August,  1895. 

^Pyogenic  Infective  Diseases  of  the  Brain,  Macmillan  &  Cb.,  London,  1893. 


SYMPTOMS.  555 

but  if  the  ajbscess  is  evacuated  by  operation  the  temperature  rises  to 
100|°  or  101°  F.,  and  in  a  few  hours  comes  down  to  below  100°  F., 
remaining  near  the  normal  until  recovery  ensues.  He  contrasts  sharply 
this  run  of  temperature  with  that  found  in  infective  thrombosis  of  the 
lateral  or  cavernous  sinuses,  in  which  very  high  temperatures  with  very 
great  remissions,  resulting  in  a  most  irregular  curve  between  106°  and 
97 J °  F.,  have  been  observed.  He  also  contrasts  it  with  the  tempera- 
ture curve  of  meningitis,  in  which  the  temperature  is  high,  between 
103°  and  104°  F.  constantly,  without  the  great  remissions  occurring 
in  thrombosis.  Okada  found  a  marked  rise  of  temperature  and  a 
febrile  course  in  46  of  88  cases  of  abscess  of  the  cerebellum;  in  15 
the  temperature  was  normal ;  in  15  it  was  subnormal ;  in  8  there  was 
a  rise  of  temperature  only  at  the  onset,  in  4  only  at  the  very  end.  A 
study  of  the  temperatures  occurring  in  three  successive  cases  of  cerebral 
abscess  shows  that  any  conclusion  from  a  study  of  temperature  is  not 
reliable.  Thus  in  a  case  seen  with  Bacon,  ^  the  curve  was  one  which 
suggested  thrombosis  of  the  sinuses,  there  being  very  high  temperatures, 
followed  by  sudden  remissions  two  or  three  times  a  day  for  several 
days  prior  to  the  operation.  In  this  case  the  abscess  was  successfully 
evacuated  and  the  patient  recovered. 

In  a  second  case  the  temperature  chart  corresponded  pretty  nearly 
to  that  described  by  MacEwen  ;  the  temperature  was  not  above  98  J °  F. 
or  below  98°  F.  during  the  week  of  illness. 

In  a  third  case  the  temperature  chart  was  much  more  suggestive  of 
meningitis,  varying  from  101°  to  104°  F.  during  the  entire  illness  of 
eight  days.  Hence  the  conclusion  must  be  reached  that  the  course 
,of  temperature  is  not  to  be  relied  upon  as  a  diagnostic  sign. 

The  changes  in  the  blood  are  important  from  a  diagnostic  and  prog- 
nostic standpoint.  There  is  an  increase  in  the  relative  proportion  of 
polynuclear  cells  on  differential  count  above  85  per  cent,  and  the 
higher  the  proportion  the  more  active  the  inflammation  in  the  brain. 
Sondern  ^  has  shown  that  this  is  associated  with  a  greater  or  lesser 
degree  of  leucocytosis ;  and  the  higher  the  leucocytosis  with  a  given 
percentage  of  polynuclear  cells,  the  better  ordinarily  is  the  body  resis- 
tance toward  the  infection  at  the  time  the  blood  examination  was  made. 
A  slight  polynuclear  increase  with  pronounced  leucocyte  increase  indi- 
cates slight  infection  and  well  marked  body  resistance.  A  pronounced 
polynuclear  increase  associated  with  a  pronounced  leucocytosis  indi- 
cates a  severe  infection  and  good  body  resistance.  A  pronounced 
polynuclear  increase  and  little  or  no  leucocytosis  indicate  a  severe  in- 
fection and  little  or  no  body  resistance.  An  absence  of  leucocytosis  in 
severe  inflammatory  lesions  is  a  bad  prognostic  sign.  If  a  differential 
count  is  made  the  high  relative  polynuclear  percentage  will  also  be 
found.  An  increasing  polynuclear  percentage  and  stationary  or  de- 
creasing leucocytosis  indicates  an  increasing  degree  of  infection  and 

'  New  York  Medical  Journal,  August  15,  1896. 

''Sondern,  Blood  Count  in  Sepsis.     N.  Y.  Med.  Jour.,  June  16,  1906. 


556  ABSCESS  OF  THE  BHAin. 

decreasing  body  resistance.  A  decrease  both  in  the  polynuclear  per- 
centage and  in  the  leucocytosis  denotes  improvement. 

In  some  cases  of  brain  abscess  these  changes  are  not  found.  When 
pus  is  confined  by  dense  pyogenic  or  fibrous  membrane,  in  other 
words,  when  no  absorption  of  toxine  occurs,  there  is  no  leucocytosis 
and  no  polynuclear  increase.  Hence  in  some  abscesses  the  blood  count 
does  not  help  us. 

Sometimes  the  rapid  emaciation  of  the  patient,  his  cachectic  appear- 
ance, with  yellow  skin  and  general  evidences  of  a  septic  state,  are  so 
marked  as  to  suggest  a  latent  abscess,  even  when  the  temperature  is  low. 

MacEwen  was  the  first  to  call  attention  to  the  diiFerence  in  the  per- 
cussion note  over  the  side  of  the  head  upon  which  the  abscess  lies.  It 
is  somewhat  difficult  to  elicit  this  physical  sign.  The  stethoscope  is  to 
be  placed  upon  the  forehead  in  the  median  line,  or  upon  some  bald 
spot  upon  the  head,  as  any  contact  with  the  hair  vitiates  the  test.  The 
head  is  to  be  struck  with  a  percussion  hammer  tipped  with  rubber. 
The  note  elicited  varies  very  much  in  different  skulls,  it  being  very 
dull  and  flat  in  a  baby,  where  the  bones  are  very  thin,  and  of  higher 
pitch  and  clearer  in  the  adult.  The  patient's  head  should  not  rest  upon 
the  pillow  while  the  test  is  applied.  MacEwen  believes  that  as  the 
intracranial  pressure  increases  the  percussion  note  becomes  higher  in 
pitch  and  of  greater  resonance,  and  that  this  resonance  increases  as  the 
disease  advances.  In  four  cases  of  brain  tumor  I  have  been  able  to 
confirm  this  statement,  the  note  elicited  being  of  greater  resonance  and 
higher  pitch  over  the  side  of  the  tumor  than  upon  the  opposite  side. 
In  all  these  cases  there  was  a  great  distention  of  the  lateral  ventricles 
with  fluid,  a  condition  which  MacEwen  also  mentions  as  affecting  the 
percussion  note.  In  two  other  cases  of  brain  tumor,  and  in  two  cases 
of  cerebral  abscess,  no  essential  difference  could  be  determined  between 
the  two  sides.  It  is  evident,  therefore,  that  this  sign  is  one  which 
requires  further  study  before  it  can  be  said  to  be  diagnostic,  though  it 
appears  to  be  of  considerable  value,  and  should  be  more  widely  known 
and  more  commonly  noticed. 

Tenderness  to  percussion  or  to  pressure  is  a  symptom  emphasized  by 
Horsley  as  occurring  in  brain  tumors,  and  it  has  been  observed  in  brain 
abscess.  It  appears  to  depend  in  both  cases  upon  the  nearness  of  the 
tumor  or  abscess  to  the  surface  or  upon  a  thinning  of  the  cranial  bones 
in  the  vicinity  of  the  tumor  or  abscess.  Such  a  thinning  of  the  crani  il 
bones  occurs  in  some  cases  in  a  very  remarkable  manner.  Thus  in  a 
patient  suffering  from  tumor  of  the  brain,  seen  at  the  New  York  Hos- 
pital with  Peabody,  the  symptoms  pointed  to  a  tumor  located  in  the 
posterior  central  convolution  at  the  junction  of  its  upper  and  middle 
thirds,  there  being  a  paralysis  with  anaesthesia  of  arm  and  leg  of  the 
opposite  side,  and  over  this  region  there  was  great  tenderness  to  per- 
cussion and  to  pressure  and  a  marked  difference  in  the  percussion  note. 
At  the  autopsy  in  this  case  the  bone  was  so  much  reduced  in  thickness 
over  this  region  as  to  be  translucent,  affording  a  sharp  contrast  to  the 
skull  in  other  parts.     The  tumor,  however,  did  not  lie  in  contact  with 


SYPMTOMS.  557 

the  skull  nor  upon  the  cortex,  but  was  at  least  one  inch  below  the 
level  of  the  cortex,  within  the  superior  parietal  lobule  and  nearer  to 
the  falx  than  to  the  dura  of  the  convexity. 

Facial  palsy  is  a  symptom  which  has  been  observed  in  abscess  of  the 
cerebellum  from  pressure  upon  the  pons  at  the  exit  of  the  seventh 
nerve.  It  is  not  to  be  forgotten,  however,  that  in  ear  disease  the  facial 
nerve  is  frequently  afiPected  in  its  passage  through  the  middle  ear,  and 
therefore  this  intracranial  symptom  must  not  be  overestimated  as  indi- 
cating either  meningitis  or  abscess. 

Optic  neuritis  is  a  sign  of  great  value  in  the  diagnosis  of  cerebral 
abscess  and  especially  in  its  differentiation  from  meningitis.  In  Okada's 
cases  two-thirds  of  the  patients  developed  optic  neuritis  in  one  or  both 
eyes.  It  often  occurs  in  thrombosis  of  the  lateral  sinus,  and  therefore 
is  of  less  value  in  differentiating  abscess  from  this  condition.  It  is  to 
be  remembered,  however,  that  many  abscesses  of  the  brain  run  their 
course  without  the  development  of  this  sign. 

It  seems  evident  from  these  considerations  that  in  the  diagnosis  of 
cerebral  abscess  very  little  reliance  is  to  be  placed  upon  any  one  symp- 
tom. It  is  the  combination  of  these  symptoms,  however,  and  a  certain 
order  of  their  development,  which  makes  the  diagnosis  clear.  It  is 
possible  to  divide  the  course  of  the  disease  into  three  distinct  periods. 
These  are  not  sharply  separated  from  one  another,  but  in  looking  back 
over  a  case  it  is  quite  evident  that  they  are  distinct.  The  history  of 
the  following  case  will  illustrate  the  course  of  the  disease  following 
otitis  media  : 

Male,  aged  thirty-six  years.  Had  had  an  attack  of  scarlet  fever  at 
the  age  of  fifteen  years,  which  was  attended  by  an  inflammation  of  the 
middle  ear,  leaving  a  chronic  discharge  from  the  right  ear  which  had 
varied  in  intensity  and  severity  from  time  to  time  during  twenty  years. 
This  otitis  media  had  not  produced  any  pain  and  was  not  attended  with 
any  degree  of  deafness.  Occasionally  the  discharge  increased  consid- 
erably and  then  the  ear  required  some  treatment.  In  March  he  caught 
cold,  suffered  from  considerable  pain  in  the  ear  and  from  a  sudden 
increase  in  the  discharge  of  pus.  The  onset  of  this  acute  attack  was 
attended  by  a  rise  of  temperature,  which  lasted  three  or  four  days  and 
then  subsided.  The  discharge  was  quite  profuse  and  was  treated  by 
antiseptic  washings.  It  lasted  about  one  month  and  then  gradually 
dried  up,  so  that  the  specialist  under  whose  care  he  was  discharged  him 
cured  of  the  otitis  media,  the  last  week  in  April.  This  attack  had 
differed  in  no  respect  from  numerous  preceding  attacks,  and  was  not 
considered  of  any  particular  importance  by  himself  or  his  family,  but 
during  this  month  of  treatment  he  suffered  from  a  constant  headache 
over  the  right  side  of  the  head,  the  side  upon  which  the  ear  was  affected  ; 
and  although  this  headache  was  only  occasionally  intense,  yet  he  was 
never  free  from  it  entirely.  During  the  month  he  became  more  and 
moi-e  nervous  and  irritable,  also  unduly  apprehensive  as  to  his  own 
condition,  and  at  times  he  acted  in  a  markedly  hysterical  manner.    He 


558  ABSCESS  OF  THE  BRAIN. 

also  became  gradually  more  and  more  depressed  and  somewhat  dull, 
and  on  two  occasions  during  the  month  he  vomited  suddenly  without 
any  nausea  or  digestive  cause.  He  also  suffered  occasionally  from 
dizziness,  and  on  several  occasions  felt  faint  when  the  ear  was  being 
treated. 

On  April  29th,  a  few  days  after  the  discharge  from  the  ear  had 
ceased,  his  headache  suddenly  became  intense  and  he  vomited  twice. 
He  became  very  much  disturbed  in  his  mind,  was  nervous,  apprehen- 
sive and  irritable,  felt  faint  and  dizzy.  His  temperature  was  98°  F. 
and  his  pulse  60.  His  tongue  was  coated  thickly,  his  breath  was  foul, 
and  he  had  the  appearance  of  a  man  suffering  from  some  septic  condi- 
tion, but  there  was  no  discharge  from  the  ear,  there  was  no  tenderness 
about  the  mastoid,  there  was  no  tenderness  of  the  head  to  percussion, 
the  optic  disks  were  clear,  the  pupils  reacted  promptly  and  were  equal, 
the  knee-jerks  were  normal,  there  was  no  disturbance  of  his  gait,  and 
although  he  was  complaining  he  was  able  to  be  up  and  about.  This 
condition  continued  on  April  30th  and  on  May  1st,  but  on  May  2d, 
after  brisk  purgatives  had  acted  he  seemed  to  be  better,  though  he  was 
still  apprehensive  and  still  suffering  from  headache,  nausea,  and  som- 
nolence. His  temperature  had  remained  constantly  at  98°  F.  and  his 
pulse  had  varied  from  60  to  80.  The  only  new  symptom  which  had 
appeared  was  a  dilatation  of  the  right  pupil. 

On  May  4th  his  condition  had  changed  markedly  for  the  worse. 
He  had  suffered  intensely  from  headache,  the  somnolence  had  increased, 
and  he  had  become  more  dull  and  was  aroused  with  some  difficulty, 
and  when  aroused  was  fretful  and  irritable,  and  did  not  talk  spontane- 
ously, but  only  in  response  to  questions.  This  somnolent  condition 
alternated  with  a  state  of  great  restlessness,  in  which  he  turned  con- 
stantly from  side  to  side,  complaining  greatly  of  general  discomfort 
and  of  pain  in  the  right  side  of  the  head.  His  right  pupil  was  still 
dilated,  and  though  both  pupils  reacted  to  light,  the  optic  disk  of  the 
right  eye  was  distinctly  congested  and  slightly  swollen ;  but  careful 
examination  failed  to  reveal  any  paralysis,  any  change  in  the  reflex 
activity,  any  disturbance  of  sensation,  and  there  was  still  no  tenderness 
over  the  mastoid  and  no  tenderness  to  percussion  of  the  head.  The 
temperature  was  still  98°  F.  and  the  pulse  52  to  60,  and  occasionally 
irregular.  At  this  time  a  diagnosis  of  abscess  of  the  brain  was  made, 
and  an  operation  was  advised  and  agreed  to.  It  was  thought  best, 
however,  to  defer  it  twenty-four  hours.  On  May  5th  the  general  con- 
dition had  become  somewhat  worse.  The  temperature  was  still  98° 
F.,  the  pulse  52,  but  varying  much  in  its  regularity,  though  never 
intermittent.  His  respirations  were  regular.  He  lay  in  a  state  of 
stupor,  was  aroused  with  great  difficulty,  his  right  pupil  was  still 
dilated,  the  optic  disks  congested,  and  the  left  side  of  the  face  was 
slightly  flattened,  this  being  the  only  evidence  of  paralysis  that  could 
be  elicited.  The  knee-jerks  were  still  normal  and  equal.  He  was  rest- 
less in  spite  of  his  stupor,  and  occasionally  had  an  expression  of  pain 
jipon  his  face  and  would  put  his  hand  to  the  right  side  of  his  head  as 


SYMPTOMS.  559 

if  in  pain.  In  spite  of  tRe  absence  of  more  definite  symptoms  it  was 
decided  to/ make  an  exploratory  trephining  over  the  right  temporo- 
sphenoidal  lobe,  and  preparations  were  begun  for  this  operation ;  but 
before  the  operation  was  begun  he  suddenly  had  a  spasm  of  the  entire 
left  side  of  the  body,  and  died.  No  autopsy  was  permitted,  but  in 
view  of  the  sudden  death  it  seemed  probable  that  an  abscess  of  the 
brain  which  had  developed  subsequent  to  the  ear  disease  had  suddenly 
ruptured,  probably  into  the  lateral  ventricle. 

In  reviewing  the  case  it  is  evident  that  the  first  period  of  the  disease 
coincided  with  tlie  month  during  which  the  patient  was  being  treated 
for  otitis  media,  and  during  which  he  had  suffered  from  headache  and 
irregular  cerebral  symptoms.  The  second  period  of  the  disease,  or  the 
period  of  acute  onset,  began  on  April  29  th,  and  lasted  for  three  days, 
merging  gradually  into  the  third  period  of  termination,  the  beginning 
of  which  was  shown  by  the  onset  of  stupor. 

A  reference  to  the  published  histories  of  abscesses  by  Bacon  ^  and 
others  will  show  that  three  periods  of  similar  limit  can  be  distinguished, 
and  therefore  it  seems  evident  that  in  the  diagnosis  of  abscess  of  the 
brain  the  combination  of  the  symptoms  in  a  regular  succession  of  stages 
rather  than  the  existence  of  any  one  symptom  is  of  the  greatest  aid  in 
diagnosis. 

The  distinguishing  characteristics  of  these  periods  may  be  briefly 
enumerated  as  follows  :  The  preliminary  period,  during  which  slight, 
irregular,  and  variable  cerebral  symptoms,  chiefly  paroxysmal  pain  and 
a  dull  ache  in  the  head,  occasional  vomiting,  and  a  lack  of  power  of 
concentration,  with  mental  fatigue  developing  easily  and  unusually,  are 
the  chief  symptoms.  During  this  period  occasional  rises  of  tempera- 
ture with  chilly  sensations  or  even  an  occasional  chill  may  be  noticed, 
and  the  patient  has  the  malaise  of  a  slight  septic  infection.  During 
this  period  the  ear  may  discharge  freely  or  the  discharge  may  gradually 
cease.  A  sudden  cessation  of  the  discharge,  and  a  sudden  fall  of  tem- 
perature to  a  point  below  normal  tw^o  or  three  days  after  the  discharge 
has  ceased,  is  a  quite  common  method  of  termination  of  this  stage ; 
hence  the  cessation  of  a  discharge  in  a  chronic  otorrhoea,  if  the  discharge 
has  been  attended  by  febrile  symptoms  for  a  few  weeks  or  days,  and  if 
the  cessation  is  attended  by  a  sudden  fall  of  temperature,  the  patient 
continuing  to  feel  ill,  with  indefinite  cerebral  sensations,  is  very  sug- 
gestive of  the  formation  of  cerebral  abscess.  Leucocytosis  will  be 
observed  early  —  but  in  moderate  degree  not  as  high  a  count  as  in 
meningitis  or  thrombosis  of  the  sinus. 

In  the  second  stage  all  the  symptoms  that  have  been  already  enum- 
erated may  occur  in  varying  combination  and  in  varying  severity  : 
the  increasing  mental  stupor,  alternating  with  irritability  and  restless- 
ness ;  the  general  septic  appearance  of  the  patient,  eitlier  with  irregu- 
lar temperature  or  low  temperature  and  with  persistently  slow  pulse  ; 
more  serious  disturbance  of  digestion  than  can  be  accounted  for  by  the 
apparent  gastric  conditions,  and  tlic  development  of  objective  cerebral 

'Handbook  of  Otology,  -OtJi  cd.     Lea  and  Febiyer,  Philadelphia,  1909. 


560  ABSCESS  OF  THE  BRAIN. 

symptoms ;  difference  in  the  size  of  the  pupils ;  optic  neuritis,  facial 
palsy,  increase  of  reflexes  in  the  limbs  of  the  side  opposite  to  the  lesion 
— all  these  symptoms  are  suggestive  of  cerebral  abscess.  It  is  in  this 
stage  that  operation  should  be  performed  if  the  diagnosis  seems  reason- 
ably probable,  for  if  the  patient  goes  on  to  the  terminal  state,  in  which 
deep  stupor  is  the  chief  characteristic,  the  operation  will  probably  be 
too  late. 

The  position  of  the  injury  in  such  cases  will  indicate  the  point  to  be 
trephined.  In  abscesses  of  the  convexity  of  the  hemisphere  following 
trauma  the  local  symptoms  of  lesion  will  be  those  already  studied  as 
characteristic  of  cortical  lesion,  especially  unilateral  spasm  and  paralysis 
or  aphasia,  hemianopsia,  or  hemiauBesthesia. 

There  are  very  few  localizing  symptoms  of  cerebral  disease  in 
abscesses  following  otitis  media,  for  the  functions  of  the  temporo- 
sphenoidal  lobe,  and  especially  of  that  part  of  it  which  lies  next  to 
the  petrous  portion  of  the  temporal  bone,  and  the  functions  of  the 
cerebellum,  so  far  as  its  lateral  lobes  are  concerned,  are  not  fully 
known.  There  are  apparently  few  distinct  cerebral  symptoms  that 
develop  uniformly  in  abscess  of  these  regions;  but  in  abscess  of  the 
temporo-spheuoidal  lobe  of  the  left  hemisphere  a  certain  form  of 
aphasia  has  recently  been  observed  which  deserves  attention.  It  will 
be  remembered  that  in  the  first  and  second  temporal  convolutions  are 
located  the  memories  of  words  heard,  the  relative  degree  of  develop- 
ment of  this  function  of  the  cortex  varying  much  in  individuals  of 
different  degrees  of  education.  It  is  also  to  be  remembered  that  in 
the  occipital  lobe  are  located  the  memories  of  objects  and  of  words 
seen.  In  1889  Freund  ^  described  a  condition  which  he  named  optical 
aphasia.  This  consists  in  an  inability  to  name  objects  which  are  seen 
and  recognized  and  whose  use  can  be  described.  Sometimes  the  name 
can  be  recollected  if  other  senses — touch,  smell,  and  taste — are  called 
in  to  aid  sight.  Freund  says  :  "  Granting  that  the  function  of  naming 
objects  seen — i.  e.,  making  an  association  between  the  visual  pictures 
of  the  object  and  the  auditory  picture  —  is  performed  through  the 
association  tracts  between  the  occipital  lobe  and  the  speech  centres, 
the  explanation  of  a  disturbance  of  this  function  is  found  in  a  lesion 
which  interferes  with  the  action  of  the  association  tract.  Such  a 
lesion  in  right-handed  persons  must  lie  in  the  temporal  lobe  of  the  left 
hemisphere."  This  localization  has  been  substantiated  by  Freund  by 
several  autopsies.  Arnold  Pick  ^  in  a  well-observed  case  of  cerebral 
abscess  has  described  this  symptom  of  optical  aphasia  fully  and  made 
it  the  basis  of  a  diagnosis  of  the  localization  of  a  cerebral  abscess 
which  the  operation  confirmed.  In  a  case  reported  by  Bacon  ^  I  had 
the  opportunity  of  studying  this  defect  of  speech.  It  is  alluded  to  in 
Bacon's  history  as  verbal  amnesia,  or  an  aphasia  of  conduction.  I  was 
unaware  of  the  description  of  Freund  at  the  time,  and  the  case  was 

1  Arch.  f.  Psych.,  XX.,  276. 

^Praguermed.  Woch.,  1896,  xxi.,  5. 

^  New  York  Medical  Journal,  August  15,  1896. 


SYMPTOMS.  561 

seen  before  the  appearance  of  Pick's  article.  I  can  confirm  fully  the 
description  "^f  Freund  and  of  Pick,  and  therefore  call  attention  of 
English-speaking  physicians  to  this  symptom.  When  the  patient  is 
shown  objects  he  knows  them,  but  he  cannot  name  them.  He  can 
understand  and  recognize  their  names,  however.  He  can  talk  well  and 
will  describe  the  object,  avoiding  its  name.  Thus  he  will  say,  when 
shown  a  knife,  "  Yes,  I  know  it ;  it  is  used  to  cut  with,"  etc.  He  can 
repeat  the  name  perfectly.  He  can  often  name  the  object  if  he 
handles  it  or  smells  it  or  tastes  it,  but  the  name  does  not  come  to  his 
mind  when  he  sees  the  object.  I  noticed  in  Bacon's  case  a  further 
difficulty  which  this  patient,  who  was  very  intelligent,  also  recognized. 
When  the  name  of  an  object  was  given  it  was  not  possible  for  him  at 
once  or  easily  to  call  to  his  mind  the  appearance  of  the  object.  In  a 
word,  it  was  as  difficult  for  him  to  make  an  association  between  his 
auditory  and  his  visual  centres  as  it  was  to  make  one  between  his 
visual  and  his  auditory  centres.  This  is  a  feature  of  the  condition  not 
noted  by  Pick.  This  symptom  of  optical  aphasia,  or  intercortical 
sensory  aphasia,  as  I  name  it,  is  due  to  a  suspension  of  function  of  an 
association  tract  lying  in  the  temporo-occipital  lobes  and  occupying 
the  white  matter  deep  under  the  cortex.  It  lies  in  a  position  in  which 
it  is  almost  inevitably  affected  by  abscess  of  the  temporal  lobe  develop- 
ing after  otitis  media.  This  symptom  should  therefore  always  be 
looked  for,  and  may  be  regarded  of  highest  value  in  locating  the 
abscess.  In  left-handed  persons  it  will  occur  in  abscess  of  the  right 
side.     (See  Plate  XI.  and  page  136  on  Aphasia.) 

When  an  abscess  in  the  temporal  lobe  extends  inward  it  may  com- 
press the  internal  capsule  and  thus  give  rise  to  symptoms  of  slowly 
increasing  hemiplegia,  hemiansesthesia,  and  hemianopsia  upon  the 
opposite  side  of  the  body.  In  a  case  of  Oppenheim's,  operated  upon 
by  Jansen,  these  symptoms  were  present.  There  was  also  a  conjugate 
deviation  of  the  eyes  to  the  side  of  the  abscess. 

The  chief  local  symptoms  of  cerebellar  abscess  are  vertigo  and  a 
staggering  gait.  The  vertigo  is  sometimes  so  severe  that  the  patient 
cannot  raise  the  head  from  the  pillow,  and  cannot  even  attempt  to 
stand.  It  is  often  associated  with  vomiting  and  sometimes  with  double 
vision  and  nystagmus.  The  vertigo  may  lead  to  a  staggering  gait,  but 
this  may  develop  without  true  vertigo.  The  patient  walks  like  a 
drunken  man,  grasps  at  objects  for  support,  and  is  unable  to  walk  a 
line.  Sometimes  there  is  a  marked  tendency  to  stagger  toward  one 
side.  This  is  usually  toward  the  side  of  the  abscess.  In  cases  of 
cerebellar  abscess  the  pressure  exerted  upon  the  side  of  the  pons  or 
medulla  may  lead  to  symptoms  of  cranial  nerve  involvement  upon  the 
side  of  the  abscess.  In  cerebellar  lesions  there  is  sometimes  a  loss  of 
knee-jerk  on  the  side  of  the  lesion.  If  there  is  at  the  same  time  a 
pressure  on  the  pons  there  will  be  an  exaggeration  of  knee-jerk  on  the 
other  side.  The  a})sence  of  symptoms  of  temporal  lobe  lesion  in  a 
case  where  abscess  is  probable  may  lead  to  an  exploration  of  the 
cerebellum. 
36 


562  ABSCESS  OF  THE  BRAIN. 

Diagnosis.  —  The  diagnosis  of  cerebral  abscess  does  not  appear  to 
be  very  difficult  in  ordinary  traumatic  cases,  for  there  is  a  history  of 
the  original  injury,  of  its  exact  location,  and  of  the  development  of  a 
series  of  cerebral  symptoms  pointing  to  a  localized  disease  in  the  brain. 
If,  then,  the  location  of  the  wound  in  the  head  and  the  localization  of 
the  cerebral  disease  based  upon  the  nervous  symptoms  coincide,  there 
is  little  doubt  as  to  the  existence  of  the  abscess. 

Where  there  is  no  history  of  a  cause  the  diagnosis  is  much  more  dif- 
ficult, as  it  is  sometimes  impossible  to  diiferentiate  the  condition  from 
acute  encephalitis.  Thus  in  a  patient  seen  with  James  the  develop- 
ment of  acute  cerebral  symptoms  during  ten  days  suggested  abscess. 
The  patient  was  a  pregnant  woman,  in  the  eighth  month.  No  history 
of  any  of  the  causes  of  abscess  could  be  obtained.  She  developed 
exophthalmus  of  the  right  eye  and  a  slowly  increasing  left  hemiplegia, 
with  high  temperature.  The  autopsy  showed  a  large  abscess  on  the 
surface  of  the  right  frontal  region,  but  no  source  of  infection  could  be 
ascertained. 

In  cases  of  chronic  otitis  media  four  separate  conditions  may  supervene 
which  are  often  difficult  of  differentiation.  These  are  cerebral  abscess, 
meningitis,  thrombosis  of  the  lateral  sinus  and  acute  encephalitis.  The 
relative  frequency  of  the  three  first  of  these  conditions  is  about  the 
same.  Thus  in  thirty-six  cases,  collected  by  Poulson,  of  cerebral  com- 
plications of  ear  disease  there  were  thirteen  cases  of  abscess,  twelve 
cases  of  thrombosis,  and  eleven  cases  of  meningitis. 

In  meningitis  there  is  usually  a  more  rapid  onset  and  progress  of  the 
symptoms  than  in  brain  abscess.  In  meningitis  the  headache  is  asso- 
ciated with  hypersesthesia  to  sound  and  to  light,  and  to  touch  all  over 
the  body,  symptoms  which  are  absent  in  abscess.  In  meningitis  the 
temperature  is  high,  varies  between  101°  and  104°  F.,  and  rarely  if 
ever  goes  below  normal.  It  does  not  show  the  rapid  rises  and  falls 
which  occur  in  thrombosis  of  the  sinuses.  In  meningitis  the  pulse  is 
rapid,  irregular,  and  intermittent,  while  in  abscess  it  is  slow.  In  men- 
ingitis there  are  occasional  twitchings  or  spasm  of  the  limbs,  or  con- 
vulsions ;  strabismus  appears  early,  trismus  is  common,  and  pain  and 
rigidity  of  the  neck  are  complained  of,  as  the  disease  advances.  These 
symptoms  are  wanting  in  brain  abscess.  In  case  of  doubt  lumbar 
puncture  will  reveal  the  presence  of  a  large  number  of  leucocytes  and 
of  microorganisms  in  the  cerebro-spinal  fluid  in  meningitis,  but  not  in 
brain  abscess. 

The  blood  examination  is  of  equal  value.  While  any  inflammatory 
state  like  that  present  in  acute  otitis  media  is  likely  to  increase  mod- 
erately the  leucocytes  in  the  blood,  a  very  great  and  sudden  increase  in 
their  number  is  a  very  valuable  indication  of  a  new  inflammatory  com- 
plication. Thus,  in  a  case  of  chronic  otitis  media  we  may  find  8,000 
or  10,000  leucocytes,  but  if  a  meningitis  or  a  brain  abscess  develops, 
the  number  rises  to  20,000  or  even  35,000.  Such  a  rise  alone  will 
indicate  that  we  have  more  than  a  mere  otitis  media  in  our  patient. 

But  this  is  not  all.     Our  data  as  yet  are  not  very  extensive,  but  I 


TREATMENT.  563 

think  we  may  safely  say  that  a  more  rapid  rise  in  the  number  of  leuco- 
cytes and  a  higher  leucocyte  count  is  found  in  meningitis  than  in  brain 
abscess.  Thus  in  two  cases  which  I  was  able  to  contrast  as  they  pro- 
gressed simultaneously,  and  in  which  the  autopsies  showed  the  exact 
condition,  it  was  observed  that  in  the  case  of  meningitis  the  leucocyte 
count  on  the  second  day  of  the  disease  was  9,000,  on  the  third  11,000, 
and  then  rose  on  the  fourth  day  to  22,000,  and  remained  between 
22,000  and  26,000  till  death  on  the  fourteenth  day.  In  the  case  of 
brain  abscess  the  number  rose  slowly  from  9,500  on  the  third  day  of 
the  disease  up  to  14,000  on  the  eighth  day,  but  never  went  up  above 
15,000  up  to  death,  which  occurred  one  month  after  the  onset. 

In  thrombosis  of  the  lateral  sinus  high  fever  with  pysemic  variations 
in  range  and  frequent  chills  are  the  rule.  The  temperature  in  the 
course  of  twenty-four  hours  may  twice  sink  below  normal  and  rise  to 
105°  F.  The  pulse  is  very  rapid  and  irregular,  but  not  intermittent. 
There  is  tenderness,  swelling,  and  oedema  over  the  mastoid  process,  and 
oedema  of  the  neck,  and  the  jugular  vein  may  stand  out  as  a  hard  blue 
cord  on  the  side  of  the  neck.  Exophthalmus  on  one  side,  and  even 
swelling  of  the  conjunctiva,  may  be  seen.  There  is  often  a  marked 
venous  congestion  of  the  scalp.  Choked  disk  appears  early  in  the 
course  of  the  case.  All  of  these  symptoms  serve  to  distinguish  the 
disease  from  abscess. 

The  diagnosis  of  cerebral  abscess  arising  under  other  conditions  is 
never  positive.  But  when  a  cause  is  present  and  cerebral  symptoms 
such  as  have  been  described  occur  in  a  definite  series  of  stages  the 
probability  of  an  abscess  is  very  great. 

Prognosis. — The  prognosis  will  depend  wholly  upon  the  possibility 
of  evacuating  the  abscess  and  draining  it  successfully. 

Treatment.  —  The  only  treatment  in  a  brain  abscess  is  surgical.  In 
a  collection  of  55  cases  of  abscess  of  the  brain  which  have  been  operated 
upon  I  found  that  28  occurred  after  injuries,  24  after  ear  disease,  and 
3  after  typhoid  fever.  Of  these  34  recovered  and  21  died.  Oppen- 
heim  has  recently  published  more  extensive  statistics.  In  60  cases  of 
traumatic  abscess  operated  upon  38  cases  recovered.  In  196  cases  of 
otitic  abscess  96  were  cured.  In  cases  of  injury  and  fracture  of  the 
skull  trephining  should  be  undertaken  early,  the  local  trauma  com- 
bining with  the  local  symptoms  to  guide  the  surgeon. 

Fig.  241  shows  the  relation  of  the  skull  to  the  convolutions  of  the 
temporal  lobe  and  cerebellum,  and  also  the  position  of  the  lateral  sinus. 
The  rules  given  by  Ballance  for  trephining  in  the  cases  developing 
after  otitis  media  are  given  in  the  explanation  of  the  figure. 

While  the  differentiation  of  abscess  of  the  brain  from  thrombosis  of 
the  lateral  sinus  and  from  meningitis  is  of  great  interest  to  the  physi- 
cian, yet  it  must  be  clearly  stated  that  it  is  not  absolutely  necessary  to 
differentiate  these  conditions  before  deciding  upon  the  question  of  sur- 
gical treatment.  It  must  be  agreed  tliat  all  three  conditions  are  neces- 
sarily fatal  to  the  patient,  and  therefore,  that  any  means  which  may 
offer  a  reasonable  amount  of  relief  to  any  or  all  should  be  employed  at 


564 


ABSCESS  OF  THE  BRAIN. 


the  earliest  possible  moment  without  waiting  for  absolute  diagnosis.  If 
the  condition  is  one  of  meningitis  the  patient  will  die  whether  operated 
upon  or  not,  and  the  operation  will  not  do  any  harm.      If  the  condition 


Fig.  241. 


Lateral  aspect  of  a  small  adult  skull.  The  illustration  shows  the  relations  of  the  lateral  sinus  to  the 
outer  wall  of  the  cranial  cavity  and  the  position  of  the  trephine  opening  (a),  which  should  be  made 
when  it  is  deemed  necessary  to  expose  it.  The  base  line  (Reid's)  passes  through  the  middle  of  the 
external  auditory  meatus,  and  touches  the  lower  margin  of  the  orbit ;  it  is  marked  out  in  eighths  of  an 
inch,  as  are  also  the  perpendicular  lines  drawn  from  it.  The  measurements  are  made  along  the  base 
line  from  the  middle  of  the  bony  meatus.  The  drawing  also  shows  the  convolutions  of  the  temporo- 
sphenoidal  lobe,  the  Sylvian  fissure,  and  the  position  of  the  lower  end  of  the  fissure  of  Rolando  {Rol.) 
X  X  indicates  the  site  of  the  tentorium  as  far  as  it  is  in  relation  to  the  external  boundary  of  the  skull. 
The  anterior  x  shows  the  point  where  the  tentorium  leaves  the  side  of  the  skull  and  is  attached  to  the 
superior  border  of  the  petrous  bone,  a,  trephine  opening  to  expose  sinus,  five-eighths  of  an  inch 
in  diameter,  its  centre  being  one  inch  behind  and  a  quarter  of  an  inch  above  the  middle  of  the 
bony  meatus.  This  opening  can  easily  be  enlarged  upward  and  backward  and  downward  and  forward 
(see  the  dotted  lines)  by  suitable  angular  cutting  bone  forceps.  It  is  always  well  to  extend  it  forward, 
so  as  to  open  up  the  mastoid  antrum  (c)  and  the  gutter  of  the  carious  bone  (if  there  be  one),  which 
leads  from  the  antrum,  tympanum,  or  meatus  down  to  the  bony  groove.  The  position  of  the  trephine 
openings,  which  must  be  made  for  the  relief  of  inflammatory  intracranial  afiections,  secondary  to  dis- 
ease of  the  ear,  other  than  for  sinus  pyaemia,  have  been  added  to  the  drawing  for  the  sake  of  contrast 
and  completeness.  They  are  as  follows :  b,  trephine  opening  to  explore  the  anterior  surface  of  the 
petrous  bone,  the  roof  of  the  tympanum,  and  the  petro-squamous  fissure,  half  an  inch  in  diameter,  its 
centre  being  situated  a  short  inch  (seven-eighths  of  an  inch)  vertically  above  the  middle  of  the  meatus. 
At  the  lower  margin  of  this  trephine  hole  a  probe  can  be  insinuated  between  the  dura  and  bone,  and 
made  to  search  the  whole  of  the  anterior  surface  of  the  petrous,  c,  trephine  opening  for  exposing  the 
mastoid  antrum,  a  quarter  of  an  inch  in  diameter,  and  half  an  inch  behind  and  a  quarter  of  an  inch 
above  the  centre  of  the  meatus  ;  or  a  quarter  of  an  inch  above  the  centre  of  the  meatus  and  a  quarter 
of  an  inch  behind  its  posterior  border.  The  trephine  should  be  directed  inward  and  slightly  downward 
and  forward.  When  a  superficial  disk  of  bone  has  been  removed,  it  is  well  to  complete  the  operation 
with  the  gouge.  A  larger  trephine  may  with  advantage  be  employed,  especially  in  adults,  d,  trephine 
opening  for  temporo-sphenoidal  abscess,  half  an  inch  in  diameter.  Situation  recommended  by  Barker 
one  inch  and  a  quarter  behind  and  one  inch  and  a  quarter  above  centre  of  meatus.  The  needle  of  the 
aspirator  is  to  be  directed  at  first  inward  and  a  little  downward  and  forward.  Birmingham  prefers  one 
and  three-fourths  of  an  inch  above,  in  order  to  avoid  the  lateral  sinus,  e,  trephine  opening  for  cere- 
bellar abscess,  half  an  inch  in  diameter  and  one  inch  and  a  half  behind,  and  a  quarter  of  an  inch  below 
the  centre  of  the  meatus.  Birmingham  prefers  two  inches  behind  and  one  inch  below  to  avoid  the 
occipital  artery.  The  anterior  border  of  the  trephine  should  just  be  under  cover  of  the  posterior  border 
of  the  mastoid  process.  The  drawing  shows  that  a  trephine  hole  made  in  this  situation  is  faraway  from 
the  lateral  sinus,  and  that  the  trocar  and  canula  of  the  aspirator,  if  directed  forward,  inward,  and 
upward,  would  hit  an  abscess  occupying  the  anterior  part  of  the  lateral  lobe  of  the  cerebellum,  which 
is  the  usual  site  of  collections  of  pus  in  this  part  of  the  braiij,  —  Fronj  Brain  Surgery,  by  Starr,  p.  193, 
(Ballance,) 


tbjsatment.  566 

is  oue  of  abscess  or  of  sinus  thrombosis,  the  operation  may  give  relief, 
and  if  one  condition  is  not  found  the  other  may  be  sought  for.  When 
it  is  considered  that  a  few  years  ago  both  these  conditions  were  con- 
sidered necessarily  fatal,  and  when  it  is  known  that  within  the  last  two 
years  the  number  of  successful  operations  reported  for  brain  abscess  has 
been  large,  it  is  evident  that  surgeons  are  appreciating  the  necessity  of 
this  procedure.  And  when  it  is  further  learned  that  of  419  abscesess 
collected  by  me  in  1906^  197  recovered,  and  of  79  cases  of  sinus 
thrombosis  41  recovered,  it  is  evident  that  surgery  has  done  a  great 
work  in  the  treatment  of  these  conditions.  This  is  not  the  place  to  give 
detailed  histories  or  analyses  of  these  operations.  It  is  evident,  how- 
ever, from  their  study,  that  the  cases  in  which  success  did  not  attend 
surgical  procedure  were  those  in  which  the  operation  had  been  de- 
layed too  long  or  had  not  been  thoroughly  exploratory.  When  it 
is  considered  that  the  part  of  the  brain  which  is  usually  involved  in 
the  abscess,  viz.,  the  temporal  lobe  or  the  cerebellar  hemisphere,  may 
be  destroyed  without  producing  any  local  symptoms,  and  when  it  is 
found  that  the  patients,  after  the  evacuation  of  abscesses  as  large  as  a 
hen's  egg,  may  recover  without  the  apparent  loss  of  any  cerebral 
function,  it  seems  justifiable  at  the  time  of  the  operation  to  explore  the 
brain  thoroughly  in  these  regions  without  fear.  Large  incisions  may 
be  made  into  the  base  of  the  temporo-sphenoidal  lobe  or  into  the  lateral 
lobe  of  the  cerebellum  without  doing  any  harm  except  from  the  attend- 
ant hemorrhage.  If  the  abscess  is  not  found  on  exploring  the  temporal 
lobe  it  is  advisable  to  explore  the  cerebellum. 

Inasmuch  as  almost  all  brain  abscesses  have  a  thick  capsule  and 
contain  very  thick  pus,  it  is  not  sufficient  in  the  exploration  to  merely 
puncture  with  a  needle  or  with  a  small  trocar,  as  the  wall  may  be 
pushed  before  these  instruments  and  not  punctured.  I  would  strongly 
urge  the  use  of  an  instrument  devised,  I  believe,  by  Horsley  for  the 
exploration  of  the  deeper  parts  of  the  brain.  This  instrument  re- 
sembles closely  a  large  ear  speculum,  but  is  sharp  at  the  end  and  is 
placed  upon  a  scissors-like  handle  and  is  split  in  two  parts,  so  that 
after  its  introduction  its  sides  may  be  separated,  allowing  a  view  of  the 
bottom  of  the  cavity  into  which  it  is  introduced.  By  pushing  such  a 
speculum  into  the  brain  tissues  gently  an  incision  of  the  vessels  can  be 
avoided,  the  resistance  of  the  capsule  of  the  abscess  more  easily  de- 
tected, and  the  wall  can  be  seen  before  and  during  the  time  of  its 
incision. 

The  question  of  drainage  of  these  abscesses  is  also  one  of  great 
interest.  It  is  to  be  remembered  that  the  cavity  of  the  abscess  in  the 
majority  of  cases  is  large  and  that  it  will  require  some  time  for  it  to 
contract  or  for  its  walls  to  approximate,  and  for  the  cavity  to  be 
obliterated.  Unless  thorough  drainage  is  maintained  during  that  time 
the  abscess  will  reappear.  The  tendency  of  the  brain  tissue  to  collapse 
is  so  great  that  unless  the  drainage  is  maintained  by  a  fairly  stiff  tube 
it  will  not  be  successful ;  hence  it  has  seemed  to  me  that  a  small  roll 
*  New  York  Medical  Kecord,  March,  1906. 


566  ABSCESS  OF  THE  BBAIN. 

of  rubber  tissue  or  a  decalcified  bone  tube  is  preferable  to  horse-hair 
or  to  gauze. 

The  practical  conclusion,  therefore,  which  experience  has  taught  is 
this  :  that  when  a  cerebral  abscess  seems  probable  from  the  history  of 
the  case  and  from  the  symptoms  which  have  developed,  and  the  general 
progress  of  the  case  demonstrates  the  existence  of  an  increasing  and 
serious  focal  disease  of  the  brain,  it  is  advisable  to  operate,  even 
though  the  symptoms  may  not  be  absolutely  typical  and  may  present 
many  variations  from  their  usual  form. 

The  rapidly  increasing  percentage  of  recoveries  after  operation 
warrants  a  degree  of  boldness,  provided  the  surgeon  is  careful  in  the 
observance  of  every  rule  of  asepsis,  without  which  precaution  such  a 
venturesome  operation  would  be  criminal. 


CHAPTER   XXXII. 

THROMBOSIS   OF   THE   VENOUS   SINUSES. 

The  various  venous  sinuses  of  the  cranium  and  their  communica- 
tions are  shown  in  Fig.  242.  A  thrombus  may  form  in  any  of  these 
sinuses,  but  it  is  in  the  lateral  or  transverse  sinus  that  it  is  most  com- 
monly found. 

Etiology.  —  Thrombosis  is  the  result  of  a  phlebitis  of  the  wall  of 
the  sinus  due  to  an  extension  to  it  of  an  inflammatory  process,  usually 
of  a  septic  nature,  in  the  immediate  vicinity,  viz.,  from.the  dura  mater 
in  pachymeningitis,  from  the  bone  and  veins  after  otitis  media,  or 
chronic  nasal  disease  or  orbital  disease.  Occasionally  thrombosis  may 
be  secondary  to  septic  processes  elsewhere,  especially  abscesses  and 
carbuncles.  In  all  these  cases  the  thrombus  is  infected,  contains 
bacteria,  and  if  it  breaks  up  and  its  particles  are  carried  onward  it 
causes  infective  emboli  in  other  organs,  and  pyaemia.  In  children 
dying  of  marasmus,  in  chronic  invalids  who  have  suifered  from  ex- 
hausting diseases  and  have  died  of  heart  failure,  in  a  few  cases  of 
fatal  infectious  fevers,  and  in  cases  of  chlorosis,  thrombi  have  been 
found  in  the  sinuses  without  phlebitis,  and  in  these  cases  they  have 
been  ascribed  to  the  state  of  the  blood.  The  pressure  of  a  brain 
tumor  or  of  a  dural  tumor  occasionally  leads  to  thrombosis.  In  a 
collection  of  cases  by  Allport^  118  out  of  128  cases  were  secondary 
to  otitis  media.  The  majority  of  cases  occur  between  the  ages  of 
fifteen  and  twenty-five  years. 

Pathology. — The  thrombus  may  be  confined  to  one  sinus  or  even 
to  a  portion  of  it,  or  it  may  invade  several  of  the  sinuses  by  gradual 
extension.  It  often  projects  into  the  veins  which  lead  out  of  the  sinus. 
It  is  usually  a  soft  clot  adherent  to  the  wall  of  the  sinus.  The  wall  is 
slightly  roughened.  If  time  has  permitted,  the  clot  may  become  hard 
or  even  organized.  If  septic  it  is  foul  in  odor,  broken  down,  yellowish- 
green,  and  purulent,  and  the  adjacent  wall  is  covered  with  pus.  Thus 
in  a  patient,  operated  upon  for  me  by  Abbe,  with  lateral  sinus  throm- 
bosis secondary  to  ear  disease,  a  purulent,  fetid,  green  mass  occupied 
the  sinus  near  the  ear,  a  long  clot  was  extracted  from  the  posterior 
part  of  the  sinus,  another  was  pulled  up  from  out  of  the  jugular  vein, 
in  which  a  hard  thrombus  had  formed  which  could  be  felt  in  the  neck; 
the  wall  near  the  purulent  mass  was  rough,  softened,  evidently  infil- 
trated with  pus.  In  this  case  the  jugular  was  tied  before  the  sinus  was 
opened.     The  patient  recovered. 

^Journal  of  the  American  Medical  Association,  1902,  p.  690. 
567 


568 


THROMBOSIS  OF  THE  VENOUS  SINUSES. 


In  patients  who  die  a  congestion  of  the  brain  is  found  in  the  area 
whose  blood  empties  into  the  sinus  that  is  the  site  of  thrombosis,  and 


Fig.  242. 


an  acute  hemorrhagic  softening  with  infarction  has  been  found.     In 
these  cases  also  multiple  septic  emboli  are  found  in  other  organs. 


TkEATMEnf.  569 

Symptoms.  —  Thrombosis  of  the  longitudinal  sinus  is  extremely 
rare  and  occurs  chiefly  as  the  result  of  septic  pachymeningitis  or  in 
cases  of  marasmus.  In  both  conditions  its  occurrence  is  rarely  diag- 
nosticated, the  only  symptom  indicative  of  it  being  a  marked  venous 
stasis  in  the  scalp.  When  this  is  present  the  veins  of  the  entire  cranium 
are  distended,  including  those  of  the  orbit  and  eye.  In  a  case  of 
Oppenheim's,  due  to  chlorosis,  choked  disk  was  seen  in  both  eyes. 
Nose-bleed  has  been  observed,  and  bulging  of  the  fontanelle  in  infants. 

Thrombosis  of  the  lateral  sinus  is  a  frequent  complication  of  otitis 
media,  and  its  diagnosis  rests  partly  on  the  prior  existence  of  this  dis- 
ease with  its  symptoms  of  earache,  purulent  discharge,  headache,  and 
general  malaise.  It  causes  a  venous  congestion  of  the  mastoid  region 
and  oedema  over  the  mastoid,  with  pain  and  tenderness ;  a  swelling 
and  thrombosis  in  the  jugular  vein  which  can  be  felt  as  a  hard  cord  in 
the  neck  in  one-third  of  the  cases,  or  an  unusual  emptiness  of  this  vein 
detected  in  inspiration  (Gerhardt's  symptom),  a  swelling  of  the  cervical 
glands,  pain  in  the  neck  on  motion  of  the  head  and  sometimes  on  swal- 
lowing, nystagmus  and  occasionally  torticollis,  the  patient  holding  the 
head  toward  the  aifected  side  to  avoid  pain.  There  is  a  choked  disk 
in  over  one-half  of  the  cases  within  the  first  week.  The  general  con- 
stitutional symptoms  of  thrombosis  of  the  lateral  sinus  are  very  similar 
to  those  of  brain  abscess,  and  the  reader  is  referred  to  that  chapter  for 
the  few  points  of  distinction  which  can  be  made.  The  disease  often 
begins  with  a  chill  and  high  fever  (104°-106°  F.),  headache,  vomit- 
ing, vertigo,  stupor,  and  delirium,  and  these  are  soon  followed  by  local 
cerebral  symptoms,  such  as  hemispasm  or  paralysis,  aphasia,  or  some 
affection  of  the  cranial  nerves.  The  fever,  as  a  rule,  continues  high 
with  frequent  intermissions,  and,  if  septic  emboli  lodge,  each  gives  rise 
to  a  chill,  a  fall  of  temperature  below  normal,  and  a  rise  to  105°  or 
106°  F.  Profuse  sweating,  rapid,  irregular  pulse,  diarrhoea,  jaundice, 
and  local  signs  in  lungs,  liver,  spleen,  and  kidneys  establish  the  exist- 
ence of  pyaemia.  Death  follows  in  the  course  of  ten  days  unless  sur- 
gical treatment  is  efficacious. 

Thrombosis  of  the  cavernous  sinus  occurs  in  connection  with  nasal 
and  orbital  disease.  Its  local  symptoms  are  oedema  and  swelling,  with 
venous  congestion  in  the  face  and  about  the  eye,  nasal  hemorrhage, 
exophthalmus,  and  distention  of  the  retinal  veins.  Various  forms  of 
strabismus  invariably  appear  as  the  oculomotor  and  abducens  nerves 
pass  through  the  walls  of  the  sinus. 

Prognosis. — The  prognosis  is  absolutely  fatal  excepting  in  the  cases 
where  an  operation  can  be  performed  in  time  —  viz.,  in  thrombosis  of 
the  lateral  sinus. 

Treatment.  —  The  treatment  of  thrombosis  of  the  lateral  sinus  is  by 
trephining  the  bone  over  the  sinus,  or  by  chiselling  away  the  mastoid 
process  and  laying  bare  the  sinus,  which  is  then  opened  freely  and 
cleaned  out.  It  is  always  well  to  tie  the  jugular  vein  prior  to  the 
opening  of  the  sinus.  If  a  clot  is  found  and  taken  away  free  flow  of 
blood  must  be  allowed  to  wash  out  the  remnants  of  the  thrombus,  and 


570  THROMBOSIS  OF  THE  VENOUS  SINUSES. 

the  inner  wall  of  the  sinus  may  be  scraped  with  a  small  curette.  The 
hemorrhage  is  easily  arrested  by  pressure,  and  the  spontaneous  tendency 
of  the  sinus  wall  to  heal  is  remarkable.  The  only  cases  which  prove 
fatal  are  those  in  which  the  thrombus  is  not  entirely  evacuated,  in 
which  secondary  thrombi  lead  to  pyaemia,  or  in  which  gangrene  of  the 
wall  of  the  sinus  leads  to  a  secondary  thrombosis.  In  a  collection  of 
150  cases  from  recent  literature  90  cases  were  found  to  have  recovered 
from  this  operation.  For  full  details  of  the  operation  the  reader  is 
referred  to  MacEwen's^  book  and  to  the  articles  by  Koerner^  and 
Jansen.^ 

^Pyogenic  Infective  Diseases  of  the  Brain,  Macmillan  Co.,  1893. 

2  Die  otitische  Erkrankungen  des  Gehirns,  Jena,  1894. 

3  Berliner  klin.  Wochenschrift,  1891,  p.  1160,  and  1901,  p.  721. 


CHAPTER  XXXIII. 

TUMOES  OF  THE  BRAIN. 

Tumors  of  the  brain  are  not  uncommon  relatively  to  other  nervous 
diseases,  and  are  seen  both  in  children  and  in  adults.  Collections  of 
cases  of  brain  tumors  have  been  published  during  the  past  twenty  years 
by  Bernhardt/  Steffan,^  Bramwell,^  Mary  Putnam  Jacobi/  Mills  and 
Lloyd/  Knapp/  Bruus/  Oppenheim/  and  myself.''  It  is  possible  by 
the  study  of  these  collections  of  cases  to  make  a  fair  estimate  of  the 
relative  frequency  of  different  varieties  of  tumor  and  of  the  various 
situations  in  the  brain  in  which  tumors  grow,  and  also  to  estimate  the 
probabilities  of  success  in  finding  and  removing  a  tumor.  Without 
attaching  too  great  importance  to  statistics  on  this  subject,  I  have 
selected  as  the  basis  of  this  chapter,  from  the  collections  already  named, 
600  tumors.  These  are  tabulated  in  Table  XIII.,  in  which  tumors  in 
individuals  below  the  age  of  eighteen  years,  chiefly  children,  are  sep- 
arated from  those  in  adults,  for  the  purpose  of  contrast.  In  this  table 
no  tumors  are  included  which  have  been  removed  or  in  which  surgical 
interference  has  been  attempted.  Yet  during  the  past  twenty  years 
over  1200  tumors  have  been  operated  upon. 

The  percentage  of  cases  open  to  operation  has  been  variously  esti- 
mated by  different  authors,  and  their  results  are  demonstrated  in  Table 
XIV.  The  various  authors  cited  have  made  collections  of  cases  with 
post-mortem  records,  and  from  a  study  of  the  condition  found  at  au- 
topsy have  estimated  the  probability  of  success  in  the  removal  of  the 
tumor  found.  In  this  table  I  have  included  100  cases  under  my  own 
care,  some  without  autopsies.  In  20  of  these  the  tumor  was  found 
either  at  the  operation  or  at  the  autopsy  to  be  accessible.  From  this 
table  it  is  evident  that  about  9  per  cent,  of  tumors  can  be  removed. 

Etiology.  —  Little  is  known  regarding  the  cause  of  brain  tumor, 
though  in  a  certain  small  percentage  of  cases  a  history  of  a  blow  or  fall 

^  Beitrage  zur  Symptomatologie  und  Diagnostik  der  Himgeschwiilste,  1881.  Also 
yearly  summary  in  Virchow's  Jahresbericht,  1881  to  1909. 

^Die  Krankheiten  des  Gehirns  im  Kindesalter.  Gerhardt's  Handb.  der  Kinder- 
krankheiten,  1880,  vol.  V. 

'Intracranial  Tumors,  1888.     Edinburgh  Medical  Journal,  December,  1894. 

*  Keference  Handbook  of  Medical  Sciences,  1888. 

*  Pepper's  System  of  Medicine,  vol.  v. 
^Intracranial  Growths,  1801. 

■^  Encyclopadisches  .Jahrbuch,  1896. 

SNothnagel'sallg.  Path.  u.  Therap.,  1896. 

®  Medical  News,  .Jan.  12,  1889;  Intracranial  Tumors,  Keating's  Cyclopaedia  of  Chil- 
dren's Diseases,  1890.  Brain  Surgery,  1893.  New  York  Medical  Record,  February, 
1896.  Montreal  Medical  Journal,  Nov.,  1897.  Journ.  Nerv.  and  Ment.  Dis.,  June, 
1903.     .Jour.  Anier.  Med.  Assoc,  July,  1907.     Anier.  Jour.  Med.  Sci.,  April,  1910. 

571 


57^ 


TUMORS  OF  THE  BRAIN. 


on  the  head  has  been  obtained.  It  is  known  that  males  are  more  sub- 
ject to  brain  tumor  than  females,  and  that  persons  of  all  ages  are  lia- 
ble to  brain  tumor. 

Table  XIII. 

(The  first  column  are  children's  tumors ;  the  second  column  adults'  tumors.) 


Situation. 

1 
i 

m 

s 

1 

1 

CO 

9 
1 
1 
O 

S 
o 

o  ce 

03 

Cystic. 
Carcinomatous. 

Gummatous. 

11 

"3 

0 

13 
6 

14 

16 
19 
2 

"i 

47 
34 

152 

9 
2 

3 

1 
11 

3 

"s 

4 
41 

l'  46 
5      7 

6 

1 

3 

1 
10 

"i 

15 
37 

19 
11 

9 
2 

"i 

2 

"s 

2 

54 

"i 

5 

8 
4 

5 
1 

"i 
"e 

25 

15 
1 

"i 

1 
1 

9 
2 

30 

...      1 
...      1 

1      1 

t 
i 

!!!  "2 

:::!  1 

...1     3 
1    ... 

1 

2'  10 

19 
3 

2 

1 
2 

"2 

"2 

7i 

i 

... 

"i 
2 

13 

1 
"3 

"3 

20 

"5 

3 

11 
3 

12 
4 

5 

7 

"i 
1 
10 

1 

41 

21  127 

35 

27 

21 
38 
6 
8 
5 
96 
43 

300 

51 

III.  Cerebral  axis : 

1.  Basal  ganglia  and  lateral 

5 

3 

5 

"i 

1 

10 
3 

34 

8 

2 

1 

3 

1 

13 

5 

86 

.34 

2.  Corpora    quadrigemina 

14 

3.  Pons 

17 

2 

9 

4 

IV.  Cerebellum   

41 

57 

300 

Table  XIV.  —  Percentage  of  Brain  Tumors  Eemovable. 


Author. 

No.  of  Cases. 

Operable. 

Reference. 

100 
100 
300 
40 
53 
49 
100 
29 
300 
50 
23 
33 
100 

10 
10 
16 
2 
4 
6 
3 
5 
21 
3 
1 
3 
20 

Pepper's  System,  1886,  vol.  v. 

Hale  White           

Guy's  Hospital  Reports,  1888. 

Starr                       

Medical  News,  January  12, 1889  (children). 

Intracranial  Growths,  1891. 

Gray                   

Sajous'  Annual,  1891. 

1892. 

Verhand.  Deut.  Gesell.  f.  Chir.,  1892. 

Trans.  New  York  Acad.  Med.,  Jan.,  1893. 

Starr          ,             

Brain  Surgery,  1893  (adults). 

Edinburgh  Med.  Journ.,  June,  1894. 

Die  Geschwultze  des  Gehirns,  1896. 

Encyclo.  Jahresbericht.,  1896. 

Starr 

Personal  cases  to  1903. 

1,277 

104 

Pathology.  —  The  Varieties  of  Brain  Tumor. — Almost  every  variety 
of  tumor  known  has  been  found  within  the  brain.  There  are  certain 
tumors,  however,  which  are  quite  common,  and  others  which  are  very 
rare.  In  the  first  class  are  tubercular,  sarcomatous,  gliomatous,  gliosar- 
comatous,  cystic,  carcinomatous,  and  gummy  tumors.  In  the  second 
class  are  fibromatous,  angiomatous,  myxomatous,  osteomatous,  and 
lipomatous  tumors,  also  psammoma,  cholesteatoma,  and  echinococcus 
cysts. 

Tubercular  tumors  of  the  brain  are  the  most  common  of  all  the  forms 
of  tumor.  They  occur  with  greatest  frequency  in  childhood,  being 
sometimes  primary,  but  usually  secondary  to  tuberculosis  of  other 
organs.  In  20  per  cent,  of  the  cases  recorded,  tubercular  tumors  are 
multiple.     This  fact  should  not  be  forgotten  in  studying  the  symptoms 


PATHOLOGY.  573 

of  brain  tumor  and  in  diagnosticating  the  location  of  tlie  tumor,  for  if 
the  tumor  is  probably  tubercular,  and  if  the  symptoms  are  not  clearly 
explicable  upon  the  theory  of  its  location  in  one  part  of  the  brain,  it  is 
to  be  remembered  that  two  or  more  different  tumors  in  different  loca- 
tions may  give  rise  to  a  great  variety  of  symptoms.  Tubercular 
tumors  vary  in  size  from  a  small  collection  of  miliary  tubercles  lying 
in  a  mass  of  thickened  pia  mater  up  to  a  large  solid  circumscribed 
mass,  with  hard,  cheesy,  or  broken-down  granular  centre  and  a  dis- 
tinct capsule.  Not  infrequently  the  tumor  is  surrounded  by  a  tuber- 
cular infiltration  both  in  the  brain  and  its  membranes.  Sometimes 
irregularly-shaped  deposits  of  tubercular  tissues  are  found  upon  the 
base  of  the  brain,  in  the  meshes  of  the  pia  mater,  compressing  the 
cranial  nerves.     (Fig.  243.) 

Tubercular  tumors  may  be  found  within  the  cerebral  tissue  at  some 
distance  from  the  surface,  and  no  part  of  the  brain  can  be  said  to  be 
free  from  a  liability  to  tubercular  deposits.      But  the  large  majority  of 


Tubercular  tumor  in  right  optic  thalamus.     Tubercular  meningitis  of  the  median  surface 
of  the  hemisphere. 

this  variety  of  tumors  are  found  in  connection  with  the  meninges  and 
about  the  large  vessels  of  the  brain,  so  that  they  are  more  commonly 
discovered  upon  the  surface  and  about  the  base  than  lying  deep  within 
the  organ.  When  the  tumor  arises  in  the  meninges  meningeal  thicken- 
ing by  tubercular  deposits  is  quite  common.  In  a  few  cases  the  cranial 
bones  have  been  eroded  in  the  growth  of  the  tumor.  Tubercular  tumors 
are  sometimes  found  in  the  brain  after  death  when  no  cerebral  symp- 
toms have  been  present  during  life.^ 

The  important  facts  to  be  considered  in  making  the  diagnosis  of 

^Edinburgh  Hospital  Reports,  vol.  i.,  p.  420, 


574  TUMORS  OF  THE  BRAIN. 

tubercular  tumors  are  (a)  hereditary  tendeDcies  of  the  individual  to 
tuberculosis  ;  {b)  exposure  to  such  influences  as  are  known  to  favor  the 
development  of  the  disease  ;  (c)  the  history  of  symptoms  of  primary 
pulmonary,  or  intestinal,  or  joint,  or  glandular  affections,  and  (cZ)  the 
presence  of  local  signs  or  general  evidences  of  tubercular  infection. 

Fig.  244. 


Section  through  cerebrum,  showing  a  large  tubercular  tumor  with  thick  capsule  and  granular  contents 
lying  beneath  the  cortex  in  the  centrum  ovale  of  the  frontal  lobe. 

When  cerebral  symptoms  develop  in  an  individual  with  tubercular 
disease  the  possibility  both  of  tubercular  tumor  and  of  tubercular 
meningitis  should  not  be  overlooked. 

Sarcomatous  tumors  are  next  in  frequency  to  tubercular.  They  are 
rarely  secondary  to  sarcoma  in  other  parts  of  the  body,  and  they  are 
only  multiple  when  they  appear  in  the  form  of  melanotic  nodules,  which 
are  rare  in  the  brain. 

Sarcoma  is  usually  encapsulated,  round,  or  oval  in  shape,  hard, 
approaching  fibroma  in  consistency,  and  is  easily  separable  from  the 
brain  tissue,  which  it  compresses  and  indents  more  frequently  than  it 
infiltrates.  Fig.  244  shows  a  sarcoma  lying  on  one  hemisphere  of 
the  cerebellum  and  Fig.  245  shows  a  sarcoma  lying  upon  the  base 
between  the  cerebellum  and  the  pons.  The  relation  of  this  tumor  to 
the  brain  and  the  skull  is  shown  in  Fig.  247,  which  demonstrates  the 
difficulty  of  reaching  tumors  of  the  cerebellum  at  an  operation.  ^ 
^  American  Journal  of  the  Medical  Sciences,  April,  1893. 


PATHOLOGY. 


575 


Round-cell  and  spindle-cell  sarcomata  are  more  frequently  met  with 
than  myx-^sarcomata  or  gliosarcomata.  These  tumors  grow  rapidly 
and  produce  well-marked  symptoms  which  do  not  vary  very  greatly  in 


Sarcoma,  encapsulated,  with  pedicle  attached  to  the  dura,  lying  in  the  posterior  cranial  fossa, 
and  coiupressing  the  left  hemisphere  of  the  cerebellum. 

their  intensity.  They  are  not  vascular,  and  hence  the  occurrence  of 
apoplectic  attacks  during  their  growth  is  infrequent.  It  is  not  to  be 
forgotten  that  a  sarcomatous  deposit  in  the  meninges  may  occur,  pro- 

FiG.  246. 


Sarcoma,  encapsulated,  lying  on  the  base  of  the  brain  and  compressing  the  right  side  of  the 
pons  and  right  hemisphere  of  the  cerebellum.     Ihe  situation  of  acoustic  nerve  tumors. 

ducing  a  thick  layer  of  new  tissue  over  an  extensive  surface  of  the 
cortex  or  base,  and  in  such  a  case  operative  treatment  is  necessarily 
unsuccessful.'     Occasionally  a    sarcoma    has  no  defined    limits,  and 

'  Eskridge,  Journal  of  the  American  Medical  Association,  September  30,  1893, 


576 


TJJMOBS  OF  THE  BBAIN. 


infiltrates  the  tissue  of  the  brain.     Hence,  sarcoma  is  not  uniformly 
fa  voidable  for  operation. 

From  the  study  of  Table  XIII.  it  is  evident  that  sarcoma  may  be 
found  in  any  part  of  the  brain,  but  it  is  more  frequent  in  the  cortex  and  in 


Fig.  247. 


The  situation  of  a  tumor  at  the  base  of  the  cerebellum,  shown  on  a  Fraser  photograph. 
This  is  the  situation  in  which  tumors  of  the  acoustic  nerve  occur. 

the  cerebellum  than  in  any  other  localities.  It  is  the  form  of  tumor 
most  easily  extracted  from  the  skull,  and  the  majority  of  the  successful 
cases  of  removal  of  brain  tumor  have  been  sarcomata. 


PATHOLOGY.  577 

Gliomatqus  tumors  come  third  in  the  list.  This  tumor  is  a  product 
of  the  neuroglia  and  presents  the  appearance  of  a  connective  tissue 
fibrillary  network  containing  a  greater  or  less  number  of  large  branch- 
ing cells  and  many  small  nucleated  cells. 

Glioma  of  the  brain  is  usually  primary,  but  occasionally  may  develop 
secondarily  to  glioma  of  the  retina. 

Gliomata  vary  in  density,  but  are  usually  softer  than  sarcomata,  and 
are  rarely  well  defined  or  separable  from  the  brain  tissue,  even  those 
which  appear  to  have  a  definite  limit  being  found,  on  microscopic 
study,  to  be  surrounded  by  a  zone  of  gliomatous  infiltration  in  the 
brain.  The  usual  consistency  of  a  glioma  is  about  that  of  the  brain 
substance.     There  is  a  tendency  in  all  gliomatous  tissues  to  undergo 

Fig.  248. 


Glioma  of  the  upper  parietal  lobule  with  infiltration  of  brain  tissue  and  hemorrhage.    (Larkin. ) 

fatty  degeneration,  to  break  down  and  liquefy,  so  that  cavities  filled 
with  clear  fluid  of  a  straw  color  or  more  sharply-defined  cysts  are  very 
frequently  found  in  and  about  a  gliomatous  tumor,  and,  in  fact,  some 
authors  have  maintained  that  all  cysts  found  in  the  brain,  not  the 
relics  of  previous  hemorrhages  or  of  embolic  softening,  are  due  to 
gliomatous  formations.  A  similar  tendency  to  the  formation  of  cavities 
within  gliomatous  tissue  is  manifested  in  the  spinal  cord  in  the  disease 
syringomyelia. 

Glioma  may  appear  in  any  part  of  the  brain,  but  is  somewhat  more 
frequent  in  the  white  substance  of  the  brain  than  in  the  gray  matter. 
As  a  glif)ma  grows  it  destroys  the  brain  tissue,  its  branching  cells  sur- 
rounding and  annihilating  both  nerve  cells  and  nerve  fibres.  It  doed 
not  compress  the  brain  as  a  sarcoma  does. 

Glioma  is  very  vascular,  and  hence  the  symptoms  which  it  produces 
37 


578 


TUMORS  OF  THE  BRAIN. 


are  somewhat  variable  in  intensity,  and,  in  case  the  delicate  vessels 
within  it  rupture,  apoplectiform  attacks  occur. 

Gliosarcoma  is  a  variety  of  tumor  not  very  frequent,  partaking  of 
the  nature  both  of  glioma  and  of  sarcoma,  commonly  accompanied  by 


Fig.  249. 


A  glioma  of  the  pons  and  medulla  infiltrating  the  tissues,  but  not  destroying  the  tracts. 
The  symptoms  were  chiefly  cerebellar. 

cystic  cavities.  Like  glioma  the  tumor  cells  are  infiltrated  through 
the  brain  tissues ;  but  like  sarcoma,  its  constituents  are  spindle  and 
round  cells,  and  its  density  is  considerable.  It  is  rarely  encapsulated, 
and,  like  glioma,  is  much  more  difficult  of  removal  than  sarcoma. 


Fig.  250. 


Gliosarcoma  of  frontal  lobes  and  corpus  callosum.     (Francis.) 

It  is  questionable  whether  the  origin  of  a  gliosarcoma  is  the  devel- 
opment of  sarcomatous  cells  infiltrating  the   brain,  and  causing  an 


PATHOLOGY.  579 

irritation  ^nd  proliferation  of  the  neuroglia,  or  whether  there  is  a  devel- 
opment of  hard,  round  connective  tissue  elements,  sarcomatous  in 
character,  in  the  midst  of  the  original  glioma. 

The  symptoms  are  more  closely  like  those  "of  glioma  than  of  sarcoma, 
the  same  variability  of  intensity  being  observed. 

Carcinoma  of  the  brain  is  usually  secondary  to  carcinoma  in  other 
parts  of  the  body,  either  of  the  orbit  or  retina,  or  of  the  internal  organs 
or  of  the  breast. 

Carcinoma  is  a  variety  seldom  found  in  children,  and  usually  de- 
velops in  adults  above  the  age  of  fifty  years.  In  cases  in  which  it  has 
occurred  in  children  it  has  been  from  direct  extension  of  the  disease 
from  the  orbit.  It  may  occur  as  the  primary  development  of  carci- 
noma in  the  body,  giving  rise  to  secondary  infection  elsewhere,  but 
this  also  is  rare. 

Carcinoma  grows  rapidly  in  the  brain,  is  not  sharply  defined,  rarely 
has  a  capsule,  is  decidedly  vascular,  and,  of  all  brain  tumors,  the  one 
least  open  to  operative  treatment. 

Cystic  tumors  of  the  brain  may  arise  either  in  connection  with  glioma 
and  gliosarcoma,  as  already  described,  or  independently,  as  the  result 
of  parasitic  infection.  Hydatid  cysts,  echinococcus,  and  cysticercus  are 
much  more  frequently  recorded  in  German  and  Australian  medical 
journals  than  in  this  country. 

Kuchenmeister  has  made  a  collection  of  88  cases  of  this  variety,  in 
all  but  13  of  which  multiple  cysts  were  found.  Of  these  49  involved 
the  membranes,  41  were  in  the  cortex,  19  in  the  white  substance,  19 
in  the  cerebellum,  18  in  the  ventricles,  17  in  the  basal  ganglia,  and  a 
few  in  the  corpora  quadrigemina,  pons,  and  medulla.  These  are  not 
included  in  the  tables,  as  it  would  give  an  appearance  of  undue  fre- 
quency of  this  form  of  tumor.  These  parasitic  cysts  grow  slowly  and 
produce  compression  of  the  brain  tissue,  but  do  not  destroy  it.  The 
symptoms  which  they  produce  are  very  rarely  so  well  marked  as  to 
enable  a  diagnosis  to  be  reached,  but  in  cases  in  which  a  diagnosis  is 
possible  these  tumors  may  be  easily  removed,  as  the  cyst  wall  is  separ- 
able from  the  brain  tissue.  Several  such  cysts  have  been  removed  in 
Germany. 

Gummata  of  the  brain,  though  very  rare  in  childhood,  and  never  the 
result  of  inherited  syphilis,  are  very  frequent  in  adult  life,  and  may 
develop  within  a  year  of  the  original  infection.  They  may  also  appear 
as  the  only  apparent  evidence  of  tertiary  syphilis,  even  twenty  years 
after  the  disease  was  acquired. 

Gumma  may  occur  as  a  soft  gelatinous  exudation  upon  the  base  of 
the  brain,  its  favorite  position,  or  anywhere  in  the  membranes,  or  as  a 
harder  and  more  circumscribed  tumor  in  the  meninges,  especially  of  the 
cortex  and  cerebellum.  It  grows  rapidly,  but  also  subsides  rapidly 
under  specific  treatment.  In  one  case  under  my  care,  where  many 
symptoms,  including  (If)uble  optic  neuritis,  were  present,  the  patient 
was  perfectly  well  in  nine  months.  The  occurrence  of  nocturnal  head- 
ache and  insomnia  of  an  obstinate  character  in  any  case  presenting  the 


580 


TUMORS  OF  THE  BRAIN 


symptoms  of  brain  tumor  will  suggest  the  probability  of  a  gumma,  and, 
in  fact,  in  every  case  in  which  the  diagnosis  of  brain  tumor  is  made  it 
is  well  to  subject  the  patient  to  a  course  of  treatment  by  mercury  and 
iodide  for  a  period  of  at  least  two  months,  in  order  to  eliminate  the  pos- 
sibility of  gumma.  Yet  it  must  not  be  hastily  argued  that  the  subsi- 
dence of  symptoms  under  this  treatment  is  positive  evidence  that  the 
tumor  is  syphilitic.  In  a  case  under  my  observation,  which  autopsy 
proved  to  be  a  small  sarcoma  with  a  large  cyst,  the  wall  of  which  was 
sarcomatous  tissue,  all  symptoms,  including  optic  neuritis,  subsided 
under  this  treatment,  and  the  patient  remained  well  for  a  period  of  five 
months,  and  then  sudden  death  in  coma  occurred  unexpectedly. 

Fig.  251. 


Malignant  chordoma  on  the  base  of  the  brain.    Kindness  of  Dr.  Smith  Ely  JellifiFe.    Zeit.  f.  Neurologie, 

V.  596,  1911. 


Chordoma  occurs  on  the  base  involving  the  cranial  nerves  and  the 
bones  of  the  skull. ^ 

Psammoma  is  usually  the  form  of  tumor  occurring  in  the  situation 
of  the  pineal  gland,  and  gives  rise  to  symptoms  similar  to  those  pro- 
duced by  tumors  of  the  corpora  quadrigemina.  Other  forms  of  tumor 
however  are  occasionally  found  in  this  location.^  They  produce 
adiposis  and  sexual  irritation. 

8  cases. 


'  Jelliffe  and  Larkin. 
'  Bailej  and  Jelliffe. 


Jour.  Nerv.  and  Ment.  Dis.,  Jan.,  1912. 
Arch,  of  Int.  Med.,  Dec,  1911,     60  cases. 


SYMPTOMS.  581 

Tumors  of  the  pituitary  body  are  not  uncommon,  and  are  usually  of 
the  natur^  of  fibroma  or  myxoma.  In  some  cases  tliey  cause  acro- 
megalic, a  growth  of  the  bones  of  the  extremities  and  of  the  jaw.  In 
other  cases  they  cause  a  production  of  fat  throughout  the  body,  sexual 
impotence  and  infantilism.  In  both  cases  bilateral  temporal  hemi- 
anopsia and  rapidly  advancing  optic  atrophy  may  be  found. ^ 

Chloroma  is  a  form  of  tumor  recently  described.  It  is  a  flat  greenish 
colored  tumor  affecting  chiefly  the  bones  of  the  skull,  especially 
those  of  the  orbit  and  of  the  base  of  the  brain,  but  occasionally  appear- 
ing in  various  lymphatic  glands.  It  was  at  first  supposed  to  be  a 
round  cell  sarcoma  of  periosteal  origin  but  when  its  tendency  to  appear  in 
lymphatic  structures  anywhere  in  the  body  was  recognized  this  view  was 
abandoned.  Chloroma  is  a  primary  disease  of  the  hematopoetic  organs 
with  special  tendency  to  the  formation  of  malignant  growths  of  a  green 
color  in  various  localities  but  chiefly  on  the  base  of  the  brain.  The 
diagnosis  from  other  tumors  affecting  the  brain  is  made  by  the  exami- 
nation of  the  blood.  This  has  the  characteristics  of  anseniia  or  of 
leukaemia  in  all  cases,  being  lacking  in  hemoglobin  and  red  cells.  The 
chief  peculiarity  is  the  existence  in  the  blood  of  large  lymphocytes, 
with  irregular  nuclei  which  stain  with  hematoxylin.  These  cells  are 
four  times  the  size  of  erythrocytes  and  are  not  identical  with  normal 
large  lymphocytes,  being  true  pathological  cells.  Myeloblasts  and 
myelocytes  and  granula  are  also  found  in  some  cases. 

The  symptoms  of  chloroma  begin  with  the  usual  symptoms  of 
profound  anaemia;  pallor,  malaise,  headaches,  general  pains  and  cachexia. 
Hemorrhages  from  the  mucous  membranes  are  frequent.  The  pain  may 
first  be  felt  in  the  eye  or  ear.  Soon  exophthalmos  appears  or  a  swell- 
ing in  or  behind  the  ear  which  soon  leads  to  deafness.  The  skull  is 
enlarged  about  the  orbit  or  the  ear  and  is  very  tender.  As  the  tumor 
increases  the  eyes  bulge  extremely  or  some  of  the  cranial  nerves  become 
involved  by  pressure  on  their  trunks  as  they  pass  through  the  enlarged 
bone.  There  are  rarely  symptoms  of  pressure  directly  on  the  brain. 
In  one  case  seen  by  me  with  Dr.  Janeway,  both  optic  nerves,  one  sixth 
nerve,  both  glossopharyngeal  nerves  and  one  hypoglossal  nerve  were 
paralyzed.  Tumors  developing  in  the  pharynx  or  bronchial  glands  or 
other  glands  produce  a  variety  of  symptoms.  Sometimes  the  tumor 
affects  the  bones  of  the  spine,  causing  compression  of  the  spinal  nerves 
or  even  a  compression  myelitis.  In  my  case  extensive  atrophy  and 
paralysis  of  the  muscles  of  one  arm  were  present.  Occasionally  green 
warts  on  the  skin  appear. 

The  course  is  a  slow  one  to  exhaustion  and  death  in  two  years  from 
some  complication  of  the  ansemia. 

Other  varieties  of  brain  tumor,  such  as  fibroma,  angioma,  myxoma, 
psammoma,  osteoma,  cholesteatoma,  lipoma,  teratoma, which  occur  in  the 
brain,  are  of  great  rarity.  They  occur  both  in  children  and  in  adults, 
and  their  diagnosis  cannot  be  made  from  other  forms  of  tumor  during  life. 

The  appearance  of  a  brain  in  which  a  tumor  has  developed  is  usually 

'Harvey  Cusliing.     Ilarvey  LectureH,  1911,  pp.  30-45. 


582  TUMOBS    OF    THE    BFAIN. 

characteristic.  The  membranes  are  tense,  the  convolutions  are  flat- 
tened by  pressure,  the  ventricles  are  distended  by  serous  fluid,  the  brain 
tissue  is  wet,  the  weight  of  the  brain  is  increased,  and  frequently  about 
the  tumor  there  is  a  zone  of  softening  of  greater  or  less  extent,  accord- 
ing to  the  variety  of  the  tumor.  Encapsulated  tumors  are  less  likely 
to  be  surrounded  by  a  softened  tissue. 

Intracranial  aneurisms  present  the  same  clinical  features  as  tumor 
of  the  brain.  They  are  rarely  large  in  size,  they  appear  upon  the  larger 
arteries  at  the  base  of  the  brain  and  upon  the  Sylvian  arteries.  They 
are  usually  fusiform,  occasionally  round.  They  increase  in  size  more 
rapidly  than  ordinary  brain  tumors,  or  than  aneurisms  elsewhere,  and 
usually  rupture  before  attaining  any  great  size.  They  produce  symp- 
toms by  their  pressure  and  are  occasionally  to  be  diagnosticated  by 
pulsating  headache  or  the  sensation  which  they  produce,  or  by  a  dis- 
tinct murmur  audible  to  the  stethoscope  on  the  head.  Such  a  murmur 
is  not,  however,  pathognomonic,  as  I  have  heard  such  a  loud  double 
murmur  over  the  Sylvian  region  in  a  case  which  proved  at  the  opera- 
tion to  be  one  of  extensive  softening  —  no  aneurism  being  found.^  In 
two  cases,  one  of  which  is  shown  in  Fig.  252,  the  rupture  of  the 
aneurism  caused  sudden  death,  but  the  aneurism  had  not  produced  any 
symptoms  during  life.  In  a  case  recently  seen  at  St.  Luke's  Hospital 
the  aneurism  developed  rapidly  after  a  blow  on  the  head,  producing, 
first,  third  nerve  paralysis,  and  one  month  later  right  hemiplegia.  The 
patient  died  three  months  after  the  injury,  having  had  all  the  general 
symptoms  of  brain  tumor.  An  aneurism  of  the  left  internal  carotid 
artery  at  the  point  of  union  with  the  posterior  communicating  artery 
was  found,  which  had  ruptured.  As  the  arteries  were  not  diseased, 
it  seemed  probable  that  the  bruise  of  the  artery  against  the  bone  at 
the  time  of  the  injury  had  weakened  the  wall  and  predisposed  to 
aneurism.^ 

Symptoms.  —  While  the  symptoms  of  brain  tumor  are  in  many 
cases  very  clear  and  characteristic,  so  that  there  need  be  little  doubt 
in  regard  to  the  diagnosis,  both  of  the  existence  of  a  tumor,  of  its 
nature,  and  of  its  location,  it  must  be  stated  that  there  are  other  cases 
in  which  no  characteristic  symptoms  appear  at  all. 

Numerous  cases  have  been  recorded  in  which  large  tumors  have 

been  found  unexpectedly  after  death,  even  in  locations  in  the  brain  in 

which  the  existence  of  a  tumor  would  presumably  have  produced  very 

marked  symptoms.     Thus,  in  one  case  under  my  observation,  a  large 

glioma  occupied  the  entire  white  substance  of  the  frontal  lobe  upon  the 

left  side,  yet  the  patient  had  sufl^ered  only   from  occasional   attacks  of 

epileptic  convulsions,  not   preceded  by   an  aura,  and  never  unilateral, 

was  as  intelligent  as  ever  up  to  the  time  of  his  death,  though  his  family 

had  noticed  some  irritability  of  temper  at  times,  and  on   account  of 

certain    peculiarities  had  occasionally   questioned   his  responsibility. 

This  man  was   under  observation   by  most  competent  physicians  for 

^  Starr,  Brain   Surgery,  Case  xix. 

2  Beadles,  Brain,  1907,  p.  285,  has  analyzed  555  cases  of  intraci-anial  aneurism. 


SYMPTOMS. 


583 


several  years,  and  was  supposed  to  have  epilepsy.  His  eyes  were  ex- 
amined arfd  found  to  be  normal  within  a  few  weeks  of  his  sudden 
death,  which  took  place  in  a  convulsion.  He  had  never  had  the  ordi- 
nary symptoms  of  a  brain  tumor.  Schoenthal  ^  has  recorded  a  case  of 
supposed  hysteria  in  which  the  variability  of  the  symptoms  was  surpris- 
ing, and  in  which  careful  examination  failed  to  reveal  any  permanent 
or  tangible  physical  signs.  After  death  a  large  tumor  of  the  right 
frontal  lobe  was  found.     Mayer  ^  has    collected    several   such   cases. 


Fig.  2e52. 


'/'If    i:     ' 


Aneuiisiii  of  the  basilar  artery.     (Larkin.) 

Buzzard  has  recorded  similar  cases.  Bramwell  ^  has  described  an 
enormous  tumor  lying  in  the  central  region  upon  the  cortex  of  the 
riglit  side  in  which  there  were  no  symptoms  of  paralysis  whatever. 
And  many  tumors  have  been  found  in  and  about  the  cerebellum 
which  have  failed  to  produce  the  ordinary  symptoms  of  brain  tumor. 

'  Berliner  klin.  Woch.,  1891,  No.  10.     See  also  Williams,  Lancet,  October  14,  1893. 
*E.  Mayer,  Inaug.  Dissert.,  Berlin,  1891. 
^  Intracranial  Tumors,  p.  12. 


584  TUMOBS    OF    THE    BRAIN. 

Tumors  upon  the  base  of  the  brain  may  also  be  found  involving 
structures  which,  during  life,  appear  to  have  been  capable  of  carrying 
on  their  ordinary  functions.  Thus  a  child  under  my  observation 
suffered  for  five  months  from  occasional  convulsions,  from  headache, 
from  spastic  jjaraplegia,  but  had  no  difficulty  whatever  of  vision,  and 
no  paralysis  of  the  cranial  nerves.  The  autopsy  showed  a  large 
tumor  upon  the  base  in  the  median  line,  through  which  both  the  optic 
nerves  passed  and  in  which  the  optic  chiasm  lay.  It  must  have  rested 
upon  all  the  motor  nerves  of  the  eyes  and  compressed  the  fifth  nerve 
on  both  sides.  This  tumor  had  grown  upward,  filling  both  the  lateral 
ventricles  and  obliterating  the  third  ventricle  entirely.^     (^ig-  253.) 

I  have  seen  a  tumor  of  the  medulla  oblongata,  an  infiltrating  glioma, 
which  produced  an  apparent  uniform  increase  in  size  of  the  entire 
medulla  to  double  its  ordinary  dimensions,  but  in  which  there  were 
absolutely  no  signs  of  any  disease  of  either  cranial  nerves  or  tracts 
passing  through  this  important  part  of  the  nervous  system.  The 
patient  had  headaches,  slight  optic  neuritis,  occasional  convulsions,  and 
a  slightly  staggering  gait,  which  led  to  the  diagnosis  of  tumor  of  the 
cerebellum.     (See  Fig.  249.) 

It  is  thus  evident  that  tumors  in  the  various  parts  of  the  brain  sub- 
stance may  develop  and  assume  a  considerable  size  without  producing 
characteristic  symptoms  either  of  a  local  or  of  a  general  type.  Such 
cases,  however,  are  rare,  and  the  careful  observer  will  usually  be  able 
to  diagnosticate  a  brain  tumor. 

The  symptoms  of  brain  tumor  are  very  numerous,  but  for  purposes 
of  convenience  may  be  separated  into  two  categories. 

I.  General  symptoms  due  to  the  existence  of  a  new-growth  irre- 
spective of  its  position.  These  are  headache,  general  convulsions, 
double  optic  neuritis,  and  optic  atrophy,  changes  of  disposition  and  of 
mental  power,  vomiting,  vertigo,  insomnia,  changes  in  the  pulse  rate, 
attacks  of  syncope,  polyuria,  and  progressive  malnutrition. 

II.  Focal  symptoms  of  the  disease  in  the  cortex  of  the  brain  or 
beneath  the  cortex  in  the  projection  tracts  which  join  the  cortex  to  the 
various  subcortical  centres.  These  symptoms  are  unilateral  spasms, 
monoplegia,  or  hemiplegia,  paraesthesia  or  anaesthesia  in  one  or  more 
limbs,  hemianopsia,  and  the  various  forms  of  aphasia.  Affections  of 
the  cranial  nerves  and  basal  parts  of  the  brain,  which  occur  with  tumors 
in  the  basal  ganglia  and  cerebral  axis,  or  external  to  the  brain  upon 
the  base.  These  latter  symptoms  are  frequently  very  complex,  as  may 
be  supposed,  when  it  is  remembered  that  the  twelve  cranial  nerves 
have  extensive  nuclei  of  origin  and  a  long  course,  and  that  all  con- 
nection between  the  external  world  and  the  brain  passes  through  the 
cerebral  axis. 

In  the  vast  majority  of  cases  of  brain  tumors  we  find  some  of  these 

general  and    focal  symptoms  present.     And  from  their  combination 

and  the  order  of  their  development  it  is  usually  possible  to  arrive  at  a 

definite  diagnosis.     These  symptoms  must,  therefore,  be  studied  with 

care. 

'  Transactions  Amer.  Neurol.  Assoc,  1891,  Starr,  Brain  Tumor. 


SYMPTOMS. 


585 


I.  General  symptoms  occur  irrespective  of  the  location  of  the  tumor 
and  depend  upon  its  rapidity  of  growth,  its  vascularity,  and  its  patho- 
logical character.  They  vary  in  severity  from  time  to  time  probably 
in  accordance  with  the  activity  of  the  pathological  process  going  on  in 
and  about  the  tumor  and  with  the  condition  of  blood  supply  in  the 
brain.  When  a  tumor  is  growing  rapidly  they  are  very  severe ; 
if  it  remain  stationary  they  may  almost  disappear.  They  are  often 
affected  by  those  agents  which  produce  a  temporary  cerebral  hyper- 
semia  or  anaemia,  such  as  hot  foot  baths  or  general  hot  baths. 

Headache  is  the  most  prominent  and  constant  symptom  of  brain 
tumor.  It  varies  in  severity,  but  is  usually  very  intense.  It  may  be 
intermittent,  it  may  recur  with  periodicity,  is  usually  worse  at  night 


Fig.  253. 


Sarcoma  of  base  separating  the  crura  and  surrounding  the  optic  chiasm  and  greatly  stretching  the 
optic  nerves.    The  exit  of  the  optic  nerves  from  the  tumor  is  indicated  by  the  white  dots. 

in  syphilitic  cases,  worse  on  waking  in  other  cases,  is  increased  by 
physical  effort  or  mental  strain,  or  emotional  excitement.  It  may  be 
a  dull,  continuous  pain,  with  paroxysms.  It  varies  in  its  location,  is 
usually  frontal  or  occipital,  and  the  situation  of  the  pain  rarely  indi- 
cates the  situation  of  the  tumor.  If,  however,  the  pain  is  constantly 
located  in  the  occipital  region  the  tumor  is  probably  in  the  posterior 
fossa.  The  pain  is  often  associated  with  indefinite  cerebral  sensations 
described  as  fulness,  pressure,  confusion,  tightness,  as  if  a  band  were 
drawn  al:)out  the  forehead,  and  these  give  rise  to  great  discomfort.  In 
infants  the  existence  of  headache  may  be  inferred  from  constant  motion 
of  the  liead,  from  movements  of  the  hands  grasping  the  head,  or  pull- 
ing the  hair,  and  from  sudden  outbursts  of  screaming  without  other 
ascertainable  cause.     Headache  is  probably  less  severely  felt  in  cases 


586  TUMOBS    OF    THE    BRAIN . 

of  tumor  in  infants,  as  the  opening  of  the  sutures  may  prevent  the 
extreme  degree  of  intracranial  pressure  to  wliich  the  headache  is  chiefly 
ascribed.  In  adults  the  headache  prevents  the  patient  from  making 
any  physical  or  mental  exertion,  and  leads  to  a  "desire  for  seclusion  and 
quiet. 

The  causes  which  are  supposed  to  produce  the  headache  are  the 
increase  of  the  intracranial  pressure  and  the  consequent  stretching  of 
the  membranes  ;  the  existence  of  effusion  into  the  ventricles,  which  is 
a  frequent  accompaniment  of  a  tumor ;  the  variations  in  the  cerebral 
circulation  produced  by  the  pressure  of  the  tumor,  or  the  direct  involve- 
ment of  the  sensitive  dura  mater  and  branches  of  the  fifth  nerve.  Such 
effects  of  the  presence  of  a  tumor  are  more  likely  to  follow  when  the 
new-growth  is  in  the  posterior  cranial  fossa  under  the  tentorium  cere- 
belli,  and  in  such  cases  headache  is  most  constant  and  most  severe. 

The  headache  is  often  associated  with  a  marked  tenderness  of  the 
head  to  percussion,  and  if  this  is  not  due  to  sensitiveness  of  a  single 
nerve  trunk  it  is  a  very  valuable  sign  of  the  location  of  a  tumor.  Such 
a  tenderness  to  percussion  is  more  commonly  found  in  tumors  lying 
just  beneath  the  bone.  Flatness  to  ausculatory  percussion  can  be  de- 
tected over  a  tumor  in  some  cases. 

General  Convulsions  are  the  next  most  frequent  symptom  of  brain 
tumor.  They  are  particularly  liable  to  occur  as  an  early  symptom  in 
children,  but  may  also  be  the  first  symptom  of  tumor  in  adults.  Thus, 
in  a  patient  of  mine  from  whom  McBurney  removed  a  large  sarcoma 
of  the  frontal  lobe,  the  first  symptom  was  a  general  convulsion  which 
occurred  two  months  before  the  headache  began,  and  was  never 
repeated  during  the  three  years'  course  of  the  disease.^  In  another 
patient  under  my  care,  who  subsequently  developed  all  the  general 
symptoms  of  brain  tumor,  general  convulsions  occurred  at  intervals 
during  three  months  before  any  other  symptom  appeared.  A  case 
diagnosticated  as  epilepsy  has  already  been  mentioned. 

It  is  not  uncommon  to  find  convulsions  occurring  at  irregular  inter- 
vals from  the  onset  of  the  disease  to  its  termination.  They  may  be 
slight  in  degree,  almost  of  the  nature  of  petit  mal,  with  a  sudden  loss 
of  consciousness,  or  this  loss  of  consciousness  may  be  attended  by  a 
little  twitching  of  the  face  and  eyes,  with  stiffening  of  the  back  and 
extremities,  balancing  movements  of  an  automatic  kind,  which  prevent 
falling,  and  then  recovery,  or  they  may  have  all  the  general  character- 
istics of  a  general  epileptic  seizure  followed  by  coma.  Sometimes  a 
peculiar  general  tremor  follows  the  attack  and  may  last  for  an  hour  or 
more. 

General  convulsions  may  be  a  culmination  of  a  local  spasm ;  hence, 
wherever  a  convulsion  occurs  particular  inquiry  is  to  be  made  as  to  the 
manner  of  its  beginning,  and  the  patient  is  to  be  carefully  observed  and 
instructed  to  watch  for  a  conscious  aura  on  the  next  occasion.  The 
significance  of  local  spasms  will  be  discussed  in  the  next  section. 

Convulsions  are  usually  indicative  of  rapid  progress  in  the  new- 
^  See  American  Journal  of  the  Medical  Sciences,  January,  1893. 


SYMPTOMS.  587 

growth,  of  effusion  into  the  ventricles,  of  hemorrhage  within  the  tumor 
or  of  a  secondary  affection  of  the  meninges.  They  may  occur  from 
tumors  situated  anywhere,  and  have  no  significance  as  a  diagnostic 
symptom  of  the  location.  Death  not  infrequently  occurs  in  convul- 
sions in  brain  tumor,  and  heiice  a  development  or  rapid  increase  of  this 
symptom  is  a  sign  of  danger. 

Changes  of  Disposition  and  of  Mental  Power  occur  in  the  course  of 
brain  tumor  with  considerable  frequency.^  In  children  this  symp- 
tom is  quite  noticeable,  the  child  becomes  fretful  and  irritable,  refuses 
to  notice  its  toys  or  to  play,  or,  if  it  does  so,  soon  becomes  wearied,  and 
requires  constant  attention.  It  may  become  indifferent  to  things  in 
which  it  was  formerly  interested,  prefer  to  lie  down  and  to  keep  quiet, 
in  a  manner  unnatural  to  a  healthy  child,  and  may  even  become  som- 
nolent and  lethargic,  or  it  may  have  apparent  attacks  of  causeless  ter- 
ror. In  adults  the  mental  dulness  is  very  noticeable.  The  patient 
takes  little  interest  in  his  ordinary  surroundings  or  business,  and  is 
content  to  sit  or  lie  for  hours  doing  nothing  and  apparently  with  a 
vacant  mind.  Such  a  patient  is  easily  aroused  and  replies  intelligently 
to  questions,  but  cannot  be  considered  in  a  normal  state  of  mental 
activity.  On  the  other  hand,  such  a  patient  is  not  demented,  although 
late  in  the  course  of  the  disease  a  condition  approaching  dementia  is 
quite  often,  observed,  especially  in  tumors  of  the  frontal  lobes.  Attacks 
of  maniacal  excitement  have  been  recorded,  but  are  very  rare.  The 
usual  mental  state  present  in  brain  tumor,  after  it  has  existed  for  a 
period  of  six  months  may  be  described  as  one  of  apathy.  These  symp- 
toms are  probably  referable  to  an  interference  with  the  processes  of 
association  upon  which  all  thought  depends.  Somnolence  may  be 
associated  with  this  apathy.  In  the  case  of  a  tumor  of  the  frontal  lobes 
and  corpus  callosum  ^  mental  apathy  was  the  chief  symptom  from 
beginning  to  end.     (See  Fig.  250.) 

Double  Optic  Neuritis  and  Optic  Nerve  Atrophy  are  very  important 
diognostic  symptoms  of  intracranial  tumor.  Neuritis  is  usually  asso- 
ciated with  other  signs  of  increased  intracranial  pressure,  but  may  occur 
without  such  pressure.  It  is  present  in  80  per  cent,  of  the  cases.  A 
marked  degree  of  optic  neuritis  may  exist  without  any  impairment 
of  vision.  When  the  patient  shows  impairment  of  visual  power  or 
limitation  of  the  visual  field  for  colors  or  for  light,  or  is  becoming 
blind,  it  will  be  found  that  optic  neuritis  or  optic  atrophy  is  fully 
developed.  Sudden  loss  of  vision  appears  to  be  more  commonly  noted 
in  histories  of  children's  cases  than  in  those  of  adults,  possible  because 
a  gradual  loss  of  sight  is  not  detected.  It  is  true  that  hydrocephalus 
may  cause  choked  discs,  and  hence  from  this  symptom  alone  a  tumor 
cannot  be  diagnosticated.  But  in  cases  where  the  diagnosis  is  difficult 
no  more  important  objective  evidence  of  brain  tumor  can  be  found. 
A  limitation  of  the  visual  field  for  colors  and  an  interlacing;  of  the  blue 
and  red  color  fields  may  occur  before  the  neuritis,  and  always  accom- 
panies it. 

'  Schuster,  P.syclii.sohe  Storunpen  hei  Ilirntnmoren,  Stuttgart,  1902. 

^  11.  G.  Francis,  American  Journal  of  the  Medical  Sciences,  June,  1895. 


588  TUMORS    OF    THE    BRAIN. 

Tumors  of  the  cerebellum  and  corpora  quadrigemina  and  tumors 
upon  the  base  of  the  brain  and  in  the  basal  ganglia  produce  optic 
neuritis  more  constantly  and  earlier  in  their  course  than  tumors 
situated  in  the  cortex  or  centrum  ovale.  Tumors  within  the  pons 
itself  are  less  liable  to  cause  optic  neuritis  than  those  outside  of  the 
pons  on  the  base.  Optic  neuritis  is  usually  double,  though  it  always 
appears  first  in  one  eye,  and  is  rarely  equally  intense  in  both  eyes ;  but 
in  a  few  cases  it  has  been  found  in  one  eye  only,  and  then  is  thought 
to  indicate  disease  of  the  nerve  in  the  orbit  or  in  front  of  the  optic 
chiasm,  rather  than  a  distant  tumor.  For  the  exact  changes  in  the 
retina  and  for  the  pathological  causes  of  optic  neuritis  the  reader  is 
referred  to  Chapter  XXXV.^ 

Vomiting  is  a  symptom  of  brain  tumor  more  frequently  observed  in 
children  than  in  adults.  It  may  or  may  not'  be  accompanied  by 
nausea.  It  may  occur  accidentally,  without  special  relation  to  the  time 
of  meals,  or  it  may  be  so  continuous  as  to  threaten  inanition.  It 
occurs  not  infrequently  on  any  movement  of  the  head  after  the  patient 
has  been  confined  to  bed  for  some  time,  and  then  it  is  usually  associated 
with  vertigo.     It  also  frequently  accompanies  severe  headache. 

Vertigo  is  a  common  symptom.  The  patient  feels  dizzy,  feels  him- 
self turning  or  falling,  and  things  about  him  appear  to  be  in  motion. 
He  grasps  at  near  objects  for  support,  covers  his  eyes  with  his  hands 
or  lies  down  on  the  floor  and  cries  out  with  bewilderment  and  distress. 
Like  vomiting,  vertigo  may  be  excited  by  changes  of  position.  It 
occurs  at  intervals  in  attacks  of  short  or  long  duration.  It  occurs 
more  frequently  with  tumors  in  the  posterior  fossa,  in  the  cerebellum, 
or  pons,  or  on  the  base  involving  the  auditory  nerve,  than  with  tumors 
elsewhere.  Such  attacks  of  vertigo  are  not  to  be  confounded  with  the 
slight  constant  vertigo  due  to  double  vision,  and  secondary  to  paresis 
of  the  third  and  sixth  nerves. 

Insomnia  may  be  due  to  disturbances  of  the  cerebral  circulation  or  to 
the  intensity  of  the  other  general  symptoms,  and  is  much  moi'e  rarely 
complained  of  in  cases  of  tumor  in  children  and  youth  than  in  adults 
suffering  from  syphilitic  tumors. 

Fever  and  Changes  in  the  Eapidity  and  Rhythm  of  the  Pulse  have  been 
observed  in  the  course  of  brain  tumors.  The  former  is  ascribed  to 
inflammatory  changes  in  the  brain  or  meninges  as  a  complication. 
The  latter  is  regarded  as  evidence  of  increased  intracranial  pressure. 
Slow  and  irregular  pulse  is  the  form  usually  noted,  but  toward  the 
close  of  life  very  rapid  pulse  has  been  observed.  Irregular  or  Cheyne- 
Stokes  respiration  has  also  been  noticed  as  a  terminal  symptom. 
■  Occasionally  attacks  of  syncope  occur  in  patients  with  tumor  of  the 
posterior  fossa,  and  a  general  feeling  of  weakness  is  not  infrequently 
complained  of.  Polyuria  and  glycosuria  may  develop  in  the  course  of 
brain  tumor  ^  as  a  symptom  of  increased  pressure.     It  has  been  found 

1  See  especially  Knies,  The  Eye  in  General  Diseases,  William  "Wood  &  Co.,  1895. 
'Rothmann,  Zeitschrift  f.  klin.  Med.,  1893,  xxiii.,  p.  339. 


SYMPTOMS.  589 

also  in  small  tumors  of  the  medulla^  as  an  evidence  of  irritation  of 
the  vagus  ffucleus. 

A  rise  of  temperature  in  the  scalp  over  the  tumor  has  been  observed 
in  a  few  cases,  but  is  not  a  uniform  or  reliable  symptom  of  brain 
tumor. 

A  unilateral  thickening  of  the  cranial  bones  including  the  face  has 
been  observed  in  a  few  cases  where  the  tumor  grew  from  the  dura. 

The  combination  of  several  of  these  general  symptoms  in  any  case 
should  lead  to  a  strong  suspicion  of  intracranial  tumor.  When,  in 
addition,  local  symptoms  are  added,  the  diagnosis  should  not  be  diffi- 
cult. In  all  cases  the  onset  of  the  general  symptoms  is  gradual,  their 
intensity  increasing  as  time  goes  on,  and  a  careful  study  of  their  mode 
of  development  and  progress  is  a  great  aid  in  the  diagnosis  of  the  kind 
of  tumor  present,  of  its  size,  and  of  the  rapidity  of  its  growth. 

The  distress  produced  by  the  general  symptoms  is  usually  much 
greater  than  that  caused  by  the  local  symptoms,  and  consequently  treat- 
ment is  often  required  for  their  alleviation. 

The  Focal  Symptoms  of  brain  tumor  depend  entirely  upon  the  situa- 
tion of  the  tumor. 

A  distinction  is  made  between  direct  and  indirect  focal  symptoms, 
the  first  being  due  to  irritation  or  destruction  of  a  limited  area  by  the 
tumor,  the  second  being  due  to  interference  with  the  function  of  an 
area  by  disease  at  a  distance  from  it  which  impairs  its  circulation  or 
causes  pressure  upon  it.  Thus,  a  tumor  of  the  cerebellum  may  cause 
incoordination  and  vertigo  as  direct  symptoms,  and  also  cause  paralysis 
of  the  sixth  and  seventh  cranial  nerves  as  an  indirect  symptom  of  dis- 
placement of  the  pons  Varolii  to  one  side,  which  stretches  these  nerves 
unduly.  Tumors  usually  cause  both  forms  of  focal  symptoms,  and, 
therefore,  much  care  must  be  given  to  the  question  of  their  significance 
in  any  case. 

Focal  symptoms  may  further  be  divided  broadly  into  two  classes, 
those  produced  by  tumors  in  the  cortex  and  cerebral  hemispheres  and 
those  produced  by  tumors  upon  the  base  of  the  brain  affecting  the 
cranial  nerves.  Reference  may  be  made  to  Chapter  V.  upon  the 
diagnosis  of  brain  diseases  for  a  special  consideration  of  these  vari- 
ous symptoms.  With  the  anatomy  of  the  brain  in  mind  it  is  not  diffi- 
cult to  understand  the  various  local  symptoms  that  may  arise  from  the 
existence  of  brain  tumor  in  different  localities.  It  should  be  remem- 
bered, however,  that  a  tumor  of  very  slow  growth,  which  compresses 
and  does  not  infiltrate  the  brain  may  exist  in  any  location  without 
necessarily  producing  focal  symptoms,  until  it  has  attained  a  consider- 
able size.  It  is  evident  that  a  single  tumor  rarely,  if  ever,  produces 
a  large  number  of  focal  symptoms  but  that  all  tumors  lying  in  any 
given  location  will  produce  the  same  symptoms.  It  is  also  evident 
that  tumors  will  almost  always  produce  unilateral  symptoms,  inasmuch 
as  they  rarely  lie  exactly  in  the  median  line.  And  even  the  tumors  of 
the  corpus  callosum,  of  which  about  twenty  have  been  recorded,  have 
^De  Jonge,  Arch.  f.  Psych.,  xiii.,  p.  658. 


590  TUMOBS    OF    THE    BMAIN. 

rarely  been  so  exactly  median  in  their  situation  as  to  give  rise  to  sym- 
metrical symptoms  upon  both  sides. 

The  focal  symptoms  usually  commence  gradually  and  are  limited  in 
extent,  but  increase  steadily  as  the  tumor  grows. 

The  Focal  Symptoms  of  Tumors  of  the  Frontal  Cortex  may  be 
enumerated  as  follows  :  mental  inactivity,  forgetfulness,  lack  of  judg- 
ment, decided  change  in  character,  irritability  of  temper  and  unusual 
stupidity,  an  inability  to  concentrate  the  attention,  to  think  connectedly 
and  continuously,  to  learn  easily,  to  exercise  self-control,  lastly  a  state 
approaching  mild  dementia  without  delusions,  in  which  the  patient 
may  become  dirty  and  disregard  all  restraints  of  decency.  These  symp- 
toms are  especially  marked  in  tumors  of  the  frontal  cortex  and  subjacent 
white  matter.^  They  are  not  present  in  tumors  lying  upon  the  base  of 
the  frontal  region  on  the  orbital  bone.  They  rarely  appear  in  tumors 
in  other  regions  of  the  cortex  until  the  last  stage  of  the  disease,  when 
the  intracranial  pressure  is  very  great.  A  decided  change  in  character 
and  disposition,  a  mental  apathy,  and  a  tendency  to  somnolence  must 
be  regarded  as  a  local  sign  of  frontal  lobe  disease.  There  is  no  ap- 
parent way  to  detect  from  the  mental  symptoms  in  which  hemisphere 
of  the  brain  the  tumor  lies.  It  is  true  that  much  of  our  thought 
is  dependent  upon  the  integrity  of  our  speech  centres,  and  hence 
the  lesions  of  the  left  hemisphere,  if  they  interfere  with  the  use  of 
language,  or  even  of  the  recollection  of  words,  appear  to  give  rise 
to  mental  confusion,  which  is  not  so  noticeable  in  lesions  of  the  right 
hemisphere.  But  it  is  not  possible  to  locate  all  mental  action,  as  has 
been  done  by  Phelps,^  in  the  left  frontal  lobe.  A  consideration  of  the 
facts  known  regarding  the  wide  dissemination  of  memory  pictures  over 
the  entire  hemisphere,  and  their  fundamental  importance  in  all  think- 
ing is  sufficient  to  prove  that  mental  activity  is  not  a  function  of  any 
one  region  of  the  brain.  (See  page  134.)  The  diagnosis  of  tumor  of 
the  frontal  lobes  is,  therefore,  rarely  made  from  a  study  of  direct  focal 
symptoms.  It  is  to  be  remembered,  however,  that  a  tumor  when 
situated  in  other  regions  of  the  cerebral  hemispheres,  excepting  only 
the  temporo-sphenoidal  lobes,  will  produce  other  direct  focal  symp- 
toms, the  absence  of  which  may  lead  to  the  suspicion  that  a  tumor  is 
situated  in  the  frontal  lobe. 

Tumors  in  the  frontal  lobe  frequently  produce  irritation  of  the  cor- 
tex. While  such  irritation  is  limited  to  the  frontal  cortex  we  have  no 
evidence  of  its  occurrence,  but  when  irritation  starts  from  the  frontal 
region  it  frequently  appears  to  radiate  backward,  and  thus  involve  the 
central  region  of  the  brain,  and  under  these  circumstances  localized 
spasms,  parsesthesia,  and  paralysis,^  on  the  opposite  side  may  occur. 
Stewart  has  noticed  a  loss  of  the  superficial  abdominal  and  epigastric 
reflexes  on  the  side  opposite  to  the  tumor,  and  a  marked  tremor  of  the 
limbs  on  the  same  side  as  the  tumor. 

^  Ferrier,  Lancet,  June  4,  1892. 

2  American  Journal  of  the  Medical  Sciences,  April,  1902, 

^  See  a  case  of  mj  own,  shown  in  Fig.  239. 


SYMPTOMS.  591 

Bruns  ^  has  carefully  recorded  three  cases  of  frontal  tumor  in  which 
there  had  been  some  disturbance  in  the  balancing  power  and  a  stagger- 
ing gait  similar  to  that  observed  in  cerebellar  disease ;  but  this  symp- 
tom has  not  been  uniformly  noticed.  It  is  known  that  the  frontal 
lobes  and  cerebellar  hemispheres  have  a  crossed  connection,'  and  it  is 
possible  that  this  symptom  may  prove  of  some  diagnostic  value  if  con- 
firmed. It  may  have  been  due  to  an  irritation  of  the  cerebellum  con- 
veyed along  these  connecting  tracts. 

Tumors  situated  in  the  third  frontal  convolution  of  the  left  hemi- 
sphere in  right-handed  persons  and  of  the  right  hemisphere  in  left- 
handed  persons  produce  motor  aphasia  with  agraphia,  the  patient  being 
able  to  understand  language  both  written  and  spoken,  but  being  unable 
to  give  expression  to  his  ideas.  When  this  symptom  develops  as  the 
result  of  brain  tumor  the  disturbance  of  speech  is  gradual  in  its  onset, 
the  patient  noticing  first  a  hesitancy  in  speech,  a  loss  of  words,  and 
possibly  a  misuse  of  words  before  he  loses  the  power  of  expression. 
The  aphasia  is  less  complete  in  brain  tumor  than  in  those  apoplectic 
conditions  which  are  the  common  cause  of  aphasia. 

Tumors  Involving  the  Motor  Area  of  the  brain  give  rise  to  spasms 
or  paralysis.  The  special  functions  of  the  motor  area  are  displayed  in 
Figs.  34  and  35,  and  the  symptoms  that  are  produced  by  a  tumor  begin- 
ning in  any  portion  of  this  area  are  easily  deduced  from  reference  to 
these  figures.  If  the  tumor  is  cortical  it  almost  inevitably  gives  rise 
to  tonic  or  clonic  convulsions,  occurring  at  intervals,  either  limited  to 
one  part  of  the  body,  such  as  the  face,  or  hand,  or  foot,  or  extending 
from  the  part  first  invaded  to  other  parts  in  a  definite  order  of  succes- 
sion, the  extension  being  commonly  from  face  to  arm,  to  body,  and  then 
to  leg,  or  in  the  reverse  order.  Such  attacks,  first  described  by  Hugh- 
lings  Jackson,  are  known  as  attacks  of  Jacksonian  epilepsy.  If  it  be 
remembered  that  the  irritation  starting  from  one  part  of  the  cortex  and 
radiating  outward  to  other  parts  produces  a  spasm  beginning  in  the 
muscles  represented  in  the  centre  first  irritated,  and  extending  to  other 
muscles  represented  in  adjacent  centres,  it  will  be  evident  that  the  exact 
order  of  the  spasm  aids  one  to  determine  the  situation  of  the  tumor. 
A  convulsion  beginning  with  the  turning  of  the  head  and  eyes  to  one 
side  indicates  a  tumor  situated  in  the  anterior  middle  portion  of  the 
motor  zone,  and  if  that  irritation  extends  from  this  point  backward  or 
downward  the  spasm  will  extend  from  head  and  eyes  to  arm  or  face, 
respectively.  The  situation  of  several  tumors  which  produced  such 
local  spasms  is  shown  in  Figs.  36  to  44,  pages  93  to  95. 

Therefore,  the  point  of  beginning  of  a  spasm  and  its  order  of  ex- 
tension are  the  most  important  localizing  symptoms  of  brain  tumor  in 
the  motor  area.  Such  a  spasm  is  usually  followed  by  temporary 
paralysis,  and  as  the  tumor  increases  in  size  and  the  spasm  increases 
in  frequency  and  extent,  the  paralysis  may  remain  permanent  between 
the  spasms.  Spasms  due  to  cortical  irritation  are  almost  invariably 
attended    by  numbness  and    tingling  in  the    part  first  affected  by 

^  Deutache  medicinische  Wochenschrift,  1892^  p.  138. 


592  TUMOES    OF    THE    BEAIN. 

spasms,  so  that  such  a  tingling  is  often  a  valuable  indication  of  the 
location  of  irritation  in  the  cortex.  This  tingling  may  even  precede 
the  spasm,  and  has  been  termed  by  Seguin  the  "  signal  symptom  "  of 
a  cortical  irritation.  When  such  a  paraesthesia  is  a  permanent  symp- 
tom the  tumor  is  more  likely  to  be  situated  behind  the  fissure  of 
Rolando  than  in  front  of  it.  When  the  irritation  produced  by  a 
tumor  in  the  motor  zone  is  very  intense,  the  local  spasm  after  extend- 
ing from  one  limb  to  the  entire  side  of  the  body  may  even  become 
general,  be  associated  with  loss  of  consciousness,  and  terminate  as  a 
general  convulsion.  While  such  an  order  of  extent  preceding  the 
general  convulsion  is  of  great  diagnostic  importance,  a  general  convul- 
sion beginning  suddenly,  with  loss  of  consciousness  and  without  any 
local  spasm,  is  not  to  be  regarded  as  indicative  of  disease  in  the  motor 
area.  There  are  cases  of  brain  tumor  in  which  the  motor  area  has  been 
generally  compressed  and  destroyed  without  the  production  of  these 
local  spasms,  but  with  a  progressive  and  increasing  paralysis  alone. 
It  is  thought  that  such  a  paralysis  not  associated  with  spasms  indicates 
that  the  situation  of  the  tumor  is  in  the  white  matter  beneath  the 
cortex  and  not  in  the  cortex  itself,  and  that  the  symptoms  are  due  to 
an  invasion  of  the  motor  tract  in  its  passage  toward  the  internal 
capsule.  Some  ataxia  is  commonly  associated  with  the  paralysis  pro- 
duced by  tumors,  and  a  marked  inco5rdination  or  disturbance  of  the 
muscular  sense,  like  anaesthesia,  points  to  a  situation  of  the  tumor 
behind  the  fissure  of  Rolando.  The  condition  of  the  deep  reflexes  is 
altered  in  all  cases  of  tumor  in  the  motor  area ;  a  marked  increase  in 
the  tendon  reflexes  at  the  elbow,  wrist,  knee,  and  ankle  is  one  of  the 
early  signs  of  paralysis.  There  is  no  atrophy  in  the  paralyzed  muscles, 
but  merely  a  slight  wasting  from  disuse,  and  there  is  no  change  in  the 
electric  reaction  of  the  muscles.  There  should  be  no  difficulty  in 
differentiating  a  cerebral  paralysis,  even  of  the  monoplegic  type,  from 
spinal  or  nerve  trunk  paralysis,  even  in  infancy,  because  of  these 
points  of  contrast.  The  careful  study  of  the  localizing  symptoms  of 
tumors  of  the  motor  region  has  resulted  in  successful  localization  of 
such  tumors  and  in  their  successful  removal  from  the  brain  ;  in  fact, 
there  is  no  region  in  the  brain  in  which  a  tumor  is  more  easily  access- 
ible or  more  possible  of  early  diagnosis  than  in  the  motor  area. 

Tumors  of  the  Parietal  Region,  including  the  superior  and  inferior 
parietal  lobules,  are  not  uniformly  attended  by  distinctive  and  local 
symptoms,  but  in  a  considerable  number  of  cases  disturbances  of  sen- 
sation and  of  muscular  sense  in  the  limbs  of  the  opposite  side  have 
been  observed.  Tumors  in  the  posterior  central  convolution  are  more 
liable  to  produce  sensory  symptoms  than  those  in  the  anterior  central 
convolution.  Tumors  lying  in  the  parietal  region  cause  sensory 
symptoms  by  pressure  upon  the  sensory  tracts  on  their  way  from  the 
posterior  portion  of  the  internal  capsule  to  the  central  region  of  the 
brain.  If  this  is  so,  then  ataxia  and  anaesthesia  appearing  in  con- 
nection with  a  tumor  of  the  parietal  region  would  be  an  indirect  local 
symptom  rather  than  a  result  of  a  lesion  in  the  sensory  centres  them- 


SYMPTOMS.  593 

selves.  The  question  of  the  existence  of  a  muscular  sense  as  distinct 
from  the  sslisorj  centres  and  the  motor  centres  has  been  discussed  on 
page  109. 

The  symptom  of  word-blindness  is  almost  uniformly  due  to  a  lesion 
of  the  inferior  parietal  lobule  in  the  left  hemisphere  in  right-handed 
people  and  in  the  right  hemisphere  in  left-handed  people.  A  reference 
to  Plate  XI.,  and  to  the  discussion  of  aphasia  on  page  136  will  show 
the  importance  of  this  symptom. 

Tumors  lying  in  the  lower  half  of  the  parietal  region  are  necessarily 
near  to  the  visual  tract  which  passes  outward  from  the  internal  capsule 
to  the  occipital  lobe,  and  hence,  defects  of  vision  of  the  form  of 
bilateral  homonymous  hemianopsia  have  been  recorded  in  connection 
with  tumors  in  this  locality. 

Tumors  of  the  Occipital  Region  of  the  brain  give  rise  to  the  important 
local  symptom  of  lateral  homonymous  hemianopsia.     (See  page  120.) 

Henschen's  researches  show  that  a  lesion  at  almost  any  portion  of 
the  occipital  lobe,  if  sufficiently  deep  to  compress  the  white  matter  of 
this  region,  will  produce  hemianopsia,  and  it  is  to  be  expected  that  in 
any  lesion  of  the  nature  of  a  tumor  in  which  small  areas  of  tissue  are 
not  destroyed,  but  considerable  areas  are  primarily  aifected  and  ad- 
jacent regions  are  strongly  compressed,  symptoms  of  hemianopsia  will 
be  frequent. 

The  occipital  lobe  is  easily  accessible  to  the  surgeon,  and  therefore 
it  is  important  that  tumors  here  should  be  discovered  as  early  as 
possible.  It  is  not  generally  appreciated  that  hemianopsia  is  a 
symptom  often  entirely  overlooked  by  a  patient,  a  case  having  been 
recently  reported  by  Bleuler  ^  in  which,  though  well-marked  hemian- 
opsia existed,  the  patient  was  entirely  unaware  of  any  visual  defect. 
It  is  therefore  exceedingly  important  that  the  extent  of  the  visual  field 
in  both  eyes  should  be  carefully  tested  in  every  case  of  suspected  brain 
disease,  each  eye  being  tested  separately. 

Lesions  of  the  occipital  lobe  are  capable  of  producing  a  disturbance 
known  as  blindness  of  mind,  in  which  the  patient,  though  seeing  an 
object,  no  longer  recognizes  it  as  having  been  previously  known.  This 
is  a  condition  allied  to  word-blindness,  in  which  the  patient  is  sud- 
denly deprived  of  the  power  of  reading.  This  condition  appears  to  be 
more  frequent  in  lesions  upon  the  left  side  of  the  brain  in  right-handed 
persons,  and  upon  the  right  side  of  the  brain  in  left-handed  persons. 
It  may  also  occur  when  a  lesion  is  entirely  subcortical,  as  well  as 
when  it  is  in  the  cortex  of  the  occipital  lobe.  This  symptom  has  been 
discussed  in  connection  with  aphasia  (pages  122  and  137). 

Tumors  of  the  Temporal  Lobes  or  tumors  lying  upon  the  base  of  the 
cerebral  hemispheres  above  the  tentorium  or  upon  the  petrous  portion 
of  the  temporal  bone  give  rise  to  few  recognizable  symptoms.  In  a 
few  such  tumors  disturbance  of  taste  and  smell  seem  to  have  been 
present  when  the  aj)ex  of  the  temporal  lobe  had  been  invaded  by  the 
givnvth,  and  when  tlie  uncinate  gyrus  had  been  destroyed.  There  are 
no  known  symptoms  produced  by  disturbance  of  the  lingual  lobule. 

38  ^  Archiv.  f.  Psychiatric,  vol.  xxv.,  p.  39. 


594  TUMOLS  OF  THE  BEAIN. 

Tumors  invading  the  first  and  second  temporal  convolution  upon  the 
left  side  in  right-handed  persons  and  upon  the  right  side  in  left-handed 
persons  produce  the  form  of  aphasia  known  as  word-deafness,  in  which 
the  person  is  unable  to  recall  the  names  of  places  or  persons,  and  can- 
not understand  what  is  said  to  him.  They  may  also  cause  inter- 
cortical-sensory-aphasia.     (See  page  138.) 

It  is  believed  that  the  first  and  second  temporal  convolutions  are  the 
termination  of  the  auditory  tract,  and  that  each  hemisphere  is  related 
to  both  ears.  Deafness  of  cerebral  origin  is,  however,  rare,  and  is  not 
often  observed  in  tumors  of  these  convolutions. 

Tumors  Lying  Within  the  Sylvian  Fissure  and  upon  the  island  of  Reil 
produce  numerous  symptoms  on  account  of  their  pressure  upon  adja- 
cent parts ;  thus,  by  pressing  upon  the  third  frontal  convolution  they 
cause  motor  aphasia ;  by  invading  the  operculum  they  produce  facial 
paralysis  ;  by  pressing  upon  the  inferior  parietal  lobule  or  the  superior 
temporal  convolution  they  may  produce  various  symptoms  of  sensory 
aphasia,  and  by  direct  pressure  upon  the  island  of  Reil  and  upon  the 
underlying  external  capsule,  they  may  cause  paraphasia.  If  this  pres- 
sure is  transmitted  more  deeply  to  the  internal  capsule  symptoms  of 
tumor  in  this  region  may  also  be  caused. 

Tumors  Lying  in  the  Median  Surface  of  the  brain,  upon  the  gyrus 
fornicatus,  gyrus  marginalis,  or  upon  the  hippocampal  convolution  are 
not  easily  diagnosticated.  Ferrier  believes  that  in  the  latter  convolu- 
tion are  situated  the  tactile  centres.  The  majority  of  physiologists 
(Munk,  Horsley,  Schaeifer)  and  of  pathologists  deny  this,  and  recently 
a  case  of  glioma  of  this  region  without  sensory  symptoms  has  been 
reported  by  Humphrey.^  Tumors  in  this  region  are  of  rare  occur- 
rence, and  no  focal  symptoms  can  be  assigned  to  them  as  yet. 

Tumors  of  the  Corpus  Callosum  are  not  at  all  common,  only  twenty 
cases  being  so  far  recorded  in  literature.^ 

The  symptoms  in  a  majority  of  these  cases  have  been  chiefly  general 
symptoms,  viz. :  headache,  vomiting,  optic  neuritis,  epileptiform  attacks, 
physical  inertia,  hemiparesis,  and  disturbance  of  intelligence,  principally 
in  the  form  of  dementia.  These  were  the  symptoms  in  one  case  under 
my  observation.  The  last-named  symptom  is  the  only  one  that  appears 
to  be  of  constant  occurrence  in  these  cases,  and  in  many  of  them  it  has 
been  preceded  by  symptoms  quite  comparable  to  those  of  hysteria. 
The  majority  of  cases  of  tumor  of  the  corpus  callosum  have  therefore 
resembled  tumor  of  the  frontal  lobes.  When  tumors  occur  in  the  cor- 
pus callosum  they  are  more  likely  to  be  situated  far  forward  about  its 
knee,  rather  than  in  its  thinner  posterior  part.      (See  Fig.  250.) 

Our  knowledge  of  the  function  of  the  corpus  callosum  is  very  im- 
perfect, though  it  is  evident  from   its  structure  that  it  associates  the 

1  British  Medical  Journal,  August  27,  1893. 

2  Bruns,  Berliner  klin.  Woch.,  1886,  Nos.  21  and  22.  Bristow,  Dis.  of  the  Nervous 
System,  p.  271.  Lutzenberger,  Neurol.  Centralb.,  1890,  p.  251.  Berkley,  American 
Journal  of  the  Medical  Sciences,  June,  1890.  Oliver,  University  Medical  Magazine, 
April,  1891.  Francis  and  Starr,  American  Journal  of  the  Medical  Sciences,  June, 
1895. 

*  Bruce,  Brain,  xii.,  p.  171. 


SYMPTOMS. 


595 


action  of  the  two  hemispheres.  The  corpus  callosnm  has  been  wholly 
wanting  in  the  brain  in  a  few  cases,  and  although  this  is  occasionally 
associated  with  imbecility  it  is  a  condition  which  has  been  found  in 
persons  who  presented  no  symptoms  during  life.^  It  is  evident,  there- 
fore, that  tumors  in  this  locality  cannot  be  positively  diagnosticated,  and 
the  situation  of  such  tumors  deep  between  the  hemispheres  forbids  the 
possibility  of  their  removal. 

Tumors  of  the  Basal  Ganglia,  including  the  lenticular  and  caudate 
divisions  of  the  corpus  striatum  and  the  optic  thalamus,  are  not  very 
rare.  Our  knowledge  of  the  functions  of  these  ganglia  is  very  imper- 
fect, and  the  necessary  loss  of  function  which  must  occur  when  they 

Fig.  254. 


Sarcoma  of  the  left  optic  thalamus  and  internal  capsule.    Cyst  in  the  centre  of  the  tumor. 

are  destroyed  by  softening  cannot  be  detected  during  life.  Tumors 
in  this  region  almost  always  produce  pressure  upon  the  internal 
capsule  through  which  pass  the  most  important  motor  and  sensory 
tracts,  and  which  lies  between  these  ganglia.  Hence  symptoms  of 
the  nature  of  hemiplegia,  hemiataxia,  hemiansesthesia,  and  hemianopsia 
are  observed  when  the  tumor  invades  one  or  more  of  these  tracts. 
(See  Fig.  53.)  It  may  then  be  possible  to  detect  the  progressive 
growth  of  a  tumor  here  by  the  succession  of  symptoms  produced,  one 
'  Bruce,  Brain,  xii.,  p.  171. 


596 


TVMOBS  OF   THE  BRAIN. 


tract  after  another  being  invaded  by  the  disease.  Motor  symptoms 
are  more  common  in  cases  of  tumors  of  the  corpora  striata.  Sensory 
symptoms  occur  in  cases  of  tumors  of  the  optic  thalami.  When  a 
tumor  invades  the  posterior  part  of  one  thalamus,  homonymous  hemi- 
anopsia is  an  invariable  symptom.  This  will  be  easily  understood  by 
reference  to  Fig.  58. 

It  has  been  supposed  that  lesions  in  the  optic  thalamus  give  rise  to 
disturbances  in  the  recognition  of  the  position  of  the  limbs  of  the  op- 
posite side,  and  that  in  consequence  awkward  positions  are  assumed 
and  awkward  movements,  athetoid  in  character,  are  made  (Meynert). 
This  I  can  confirm.  In  the  patient  whose  tumor  of  the  optic  thala- 
mus is  shown  in  Fig.  254   there  was  noticed  a  peculiar  forced  posture 

Fig.  255. 


Teratoma  of  pineal  gland,  showing  secondary  hydrocephalus.     Kindness  of  Dr.  Pearee  Bailey 

of  the  limbs  when  he  was  at  rest.  The  leg  was  thrown  back  and  out 
with  the  toe  pointed  down,  and  the  hand  was  held  up  above  and  in 
front  of  the  head.  This  symptom  persisted  for  many  weeks  until  total 
hemiplegia  gradually  developed.  It  has  also  been  affirmed  that  a 
tremor  quite  similar  to  that  of  multiple  sclerosis  may  be  caused  by  a 
tumor  of  the  thalmus.^  It  has  been  noticed  that  disturbances  in  facial 
expression  and  in  the  natural  play  of  facial  feature  in  speech  and 
thought  have  been  noticed  in  patients  suffering  from  disease  of  the 
optic  thalamus.^  The  face  is  affected  on  the  side  opposite  to  the  lesion 
and  does  not  act  in  smiling,  but  can  be  moved  voluntarily.  It  has 
been  thought  that  certain  inhibitory  functions  are  exercised  by  the 
thalamus,  and  that  undue  emotional  reactions  occur  when  it  is  diseased. 

1  D.  H.  Cooke,  Lancet,  :^Iay  28,  1892. 

^Kirilzew,   Neurol.  Centralbl.,  1891,  p.  310;  Nothnagel,  Zeitschrift  f.  klin.  Med., 
1889 ;  Bechterew,  Neurol.  Centralbl.,  1884,  p.  102. 


SYMPTOMS.  597 

Disturbances  in  the  body  temperature  have  been  observed  in  a  few 
cases. ^  Cases  are  on  record  to  support  each  of  these  statements,  yet  it 
must  be  admitted  that  numerous  negative  cases,  that  is,  cases  of  tumor 
of  the  thalamus  in  which  these  symptoms  are  wanting,  throw  much 
doubt  on  the  diagnostic  significance  of  the  effects  mentioned.^  My  own 
view  of  the  functions  of  these  ganglia  is  that  they  preside  over  many 
acts  which  are  commonly  performed  in  a  purely  automatic  manner,  for 
example,  the  posture  of  the  body,  the  ordinary  gait,  the  facial  expres- 
sion, and  probably  emotional  control ;  that  these  acts  can,  however,  be 
performed  voluntarily  as  well  as  automatically,  and  hence,  when  the 
automatic  mechanism  fails,  a  volitional  act  takes  its  place,  which  prac- 
tice soon  perfects,  leaving  the  individual  quite  unconscious  of  his  defect, 
and  hence  causing  few  or  no  symptoms.  It  is,  therefore  —  on  this 
theory  —  clear  that  tumors  of  the  basal  ganglia  cause  few  recognizable 
symptoms. 

Tumors  of  the  Corpora  Quadrigemina  present  symptoms  of  a  focal 
kind  which  are  characteristic.^  These  consist  of  a  staggering  gait 
similar  to  that  seen  in  cerebellar  disease  and  symptoms  of  paralysis  or 
paresis  in  the  muscles  controlled  by  the  ocular  nerves.  The  co5rdi- 
nation  in  movements  of  the  eyes,  the  reflex  motions  of  the  eyes  in  fol- 
lowing a  light  or  turning  toward  a  sound,  and  the  power  of  steady 
gazing  at  an  object  are  controlled  by  automatic  centres  in  the  corpora 
quadrigemina.  Disease  here  frequently  produces  nystagmus  and  in- 
terferes with  these  automatic  movements.  The  symptoms  are  not 
referable  to  a  disease  of  one  third  nerve  alone,  for  the  pupils  are  often 
unaffected.  They  are  often  bilateral  and  more  nearly  resemble  the 
symptoms  of  ophthalmoplegia  externa.  Hence,  it  is  possible  to  distin- 
guish lesions  of  the  corpora  quadrigemina  from  lesions  in  the  crus 
cerebri,  which  cause  true  third  nerve  paralysis.  The  eyeballs  are  not 
often  equally  affected,  one  being  more  paralyzed  than  the  other ;  but 
neither  eyeball,  as  a  rule,  is  totally  paralyzed.  The  superior  and  in- 
ferior recti  are  more  commonly  affected  than  the  lateral  muscles.  It 
is  the  combination  of  these  ocular  symptoms  with  the  reeling  gait  which 
will  lead  to  the  diagnosis  of  tumor  of  the  corpora  quadrigemina  when 
the  other  (general)  symptoms  of  brain  tumor  are  present. 

The  focal  symptoms  of  tumors  at  the  base  of  the  brain  are  necessarily 
very  complex.  Suffice  it  to  say  that  tumors  lying  upon  the  base  of  the 
brain  will  necessarily  compress  one  or  more  of  the  cranial  nerves,  either 
upon  one  side  alone  or  upon  both  sides,  in  case  the  tumor  is  near  the 
median  line.  The  situation  of  the  tumor  can  usually  be  determined  by 
noting  the  order  in  which  the  various  cranial  nerves  are  invaded  from 
first  to  twelfth.  (See  Figs.  52  to  56,  and  77.)  The  reader  is  also 
referred  to  Cluipter  XXXIV.,  for  the  consideration  of  lesions  at  the 
base,  and  to  Cliapter  XXXV.,  for  lesions  of  the  cranial  nerves. 

Tumor  of  the  Crus  Cerebri  is  characterized  by  the   combination  of 

'  Cowan,  Lancet,  December,  1892  ;  Lloyd,  Med.  News,  January,  1892. 
2  Wharton  Sinklcr,  T'nivcrsity  Med.  Mafr.,  October,  1893. 
"Nothnagel,  Brain,  xii.,  21  ;  Hall,  Heidelberg  Dissert.,  1892. 


598  TUMOBS  OF  TEE  BBAIN. 

hemiplegia  of  one  side  with  third  nerve  paralysis  of  the  opposite  side. 
The  tumor  lies  on  the  side  on  which  the  third  nerve  is  affected.  To 
this  combination  bilateral  hemianopsia  may  be  added  if  the  tumor  com- 
presses the  optic  tract.  ^ 

Tumors  in  the  Upper  Part  of  the  Pons  or  in  the  crus  cerebri  affect 
the  third  and  fifth  nerves,  producing  external  strabismus,  with  dila- 
tation of  the  pupil  and  ptosis,  and  also  tingling,  pain  and  ansesthesia 
of  the  face  with  ulceration  of  the  cornea,  and  sometimes  grating  of 
the  teeth  during  sleep.  Tumors  in  or  near  the  lower  half  of  the  pons 
involve  the  sixth  and  seventh  and  eighth  nerves,  causing  internal 
strabismus  with  contracted  pupil,  paralysis  of  the  face  including 
inability  to  close  the  eyes,  and  deafness,  with  vertigo.  Alternating 
hemiplegia  is  also  produced  by  a  lesion  in  this  region. 

Tumors  in  the  Medulla  may  disturb  the  action  of  the  glossopharyn- 
geal, pneumogastric,  spinal  accessory,  and  hypoglossal  nerves,  pro- 
ducing difficulty  in  deglutition,  irregular  respiration,  irregular  pulse, 
flushing  of  the  skin,  with  sweating,  sometimes  unilateral,  projectile 
vomiting,  polyuria,  or  glycosuria,  retraction  of  the  head  or  rolling  of 
the  head  upon  the  pillow,  and  lastly,  paralysis  of  the  tongue,  with  in- 
ability to  articulate  distinctly  or  to  swallow. 

When  the  tumor  lies  upon  the  base  of  the  brain,  it  not  only  invades 
the  cranial  nerves,  but  it  presses  upon  the  various  tracts  which  traverse 
the  crura,  pons,  and  medulla.  Thus  it  may  give  rise  to  hemiplegia  or 
hemiataxia  or  hemiansesthesia,  according  to  the  tract  invaded.  These 
symptoms  are  easily  understood  by  reference  to  Figs.  52  to  56. 

Tumors  of  the  pons,  producing  pressure  upon  the  middle  peduncle 
of  the  cerebellum,  produce  a  tendency  to  stagger  in  walking.  The 
patient  usually  staggers  toward  the  side  opposite  the  tumor,  but  this 
is  not  an  invariable  rule.  Tumors  of  the  pons  quite  uniformly  pro- 
duce a  loss  of  the  tendon  reflexes  at  the  knee.  The  control  of  the 
bladder  and  rectum  is  also  frequently  impaired. 

Tumors  of  the  Cerehellum  are  exceedingly  frequent  both  in  children 
and  in  adults,  and  present  almost  all  of  the  characteristic  general 
symptoms  of  brain  tumor.  The  situation  of  the  cerebellum  held  down 
in  a  small  cavity  by  the  tentorium  cerebelli  is  such  that  a  small 
growth  in  the  posterior  fossa  is  capable  of  producing  an  increase  of 
intracranial  pressure  quite  early  in  the  course  of  the  case.  As  a  result 
of  such  compression  an  accumulation  of  fluid  in  the  ventricles  is  usual. 
Hence,  symptoms  appear  earlier  in  the  course  of  the  disease  in  tumors 
about  the  cerebellum  than  in  tumors  elsewhere  in  the  brain.  Head- 
ache, occipital  or  frontal,  is  usually  the  first  symptom,  and  optic  neur- 
itis appears  very  early  in  the  course  of  the  case.  The  percentage  of 
cases  having  optic  neuritis  is  much  greater  in  tumors  of  the  cerebellum 
than  in  tumors  in  the  cerebral  hemispheres.  Nystagmus  is  also  an 
early  and  constant  symptom. 

The  focal  symptoms  of  tumors  of  the   cerebellum  are  quite  char- 
acteristic.    They  are  staggering  in  walking,  sometimes  attended  by  a 
1  Venal,  Bullet,  de  la  Soc.  Anat.,  Paris,  Jan.,  1891. 


SYMPTOMS.  69S 

strong  tendency  to  stagger  or  to  fall  in  a  particular  direction  ;  vertigo  ; 
cerebellar  Seizures  ;  abnormal  positions  of  the  head  and  body ;  and 
symptoms  referable  to  a  compression  or  displacement  or  stretching  of 
the  cranial  nerves  or  basal  arteries  lying  in  the  posterior  fossa.^ 

Tumors  in  the  middle  lobe  of  the  cerebellum  uniformly  produce  cere- 
bellar ataxia  or  a  staggering  gait.  Tumors  in  the  hemispheres  of  the 
cerebellum  near  to  and  compressing  the  middle  lobe  are  attended  by  the 
same  symptoms.  So  also  are  tumors  which  compress  the  peduncles  of 
the  cerebellum,  especially  the  middle  peduncles  in  their  course  to  the 
pons,  or  the  superior  peduncles  on  their  way  toward  the  corpora 
quadrigemina.  Tumors  lying  within  the  hemispheres  which  do  not 
invade  these  parts  do  not  always  produce  a  staggering  gait.  Hence,  a 
diagnosis  of  a  tumor  of  the  cerebellum  is  easy  when  the  tumor  lies 
near  to  the  median  line,  but  is  difficult  when  it  lies  near  the  surface 
in  the  lateral  portion  of  the  hemispheres.  Thus  in  the  tumor  shown 
in  Fig.  245  there  was  no  staggering ;  while  in  the  tumor  shown  in 
Fig.  246  the  patient  staggered  toward  the  left  side.  The  early  ap- 
pearance of  staggering  as  related  to  the  general  symptoms  is,  there- 
fore, an  important  sign  in  favor  of  a  cerebellar  tumor,  while  the 
appearance  of  staggering  many  months  after  the  development  of  optic 
neuritis  will  merely  indicate  that  a  tumor  in  the  hemisphere  has  finally 
reached  or  encroached  upon  the  middle  lobe.  The  characteristics  of 
cerebellar  ataxia  are  a  staggering  gait  with  steps  of  irregular  length 
and  position,  the  body  swaying  like  that  of  a  person  intoxicated.  The 
ataxia  of  the  legs  and  of  the  body  is  attended  by  a  decided  sense  of 
vertigo,  and  does  not  usually  persist  when  the  patient  lies  down.  The 
ataxia  of  the  hand  is  often  attended  by  a  tremor.  Stewart  and  Holmes  ^ 
in  a  study  of  the  vertigo  in  forty  cases  of  cerebellar  tumor  reach  the 
conclusion  that  in  cerebellar  tumors  the  sense  of  displacement  of 
external  objects  in  front  of  the  patient  is  from  the  side  of  the  lesion  to 
the  opposite  side ;  thus  if  the  tumor  be  on  the  left  side  objects  seem 
to  move  to  the  right.  They  found  that  in  intracerebellar  tumors  the 
sense  of  self-rotation  is  from  the  side  of  the  lesion  to  the  healthy  side. 
But  in  extracerebellar  tumors  the  sense  of  self-rotation  is  from  the 
healthy  side  toward  the  side  of  the  lesion.  The  sense  of  self-rotation 
leads  to  an  attempt  to  correct  the  supposed  position,  hence  the  patient 
may  roll  over  in  bed  or  may  stagger  in  one  direction.  Such  a  uni- 
lateral staggering  is  not  a  common  symptom.  These  characteristics, 
together  with  the  fact  that  the  knee-jerks  are  often  exaggerated  in  cere- 
bellar tumor,  will  enable  anyone  to  distinguish  this  disturbance  of  gait 
from  that  appearing  in  locomotor  ataxia.  Occasionally,  however,  the 
knee-jerk  is  lost  on  the  side  of  the  tumor.  Cerebellar  ataxia  is  due  in 
part  to  a  disturbance  in  the  mechanism  of  equilibrium  in  so  far  as  this 
depends  upon  impressions  coming  to  the  brain  from  muscles  of  the 
trunk  and  legs.  The  direction  of  staggering  is  not  an  indication  of 
the  side  on  which  the  tumor  lies. 

^  See  Mills  and  Frazier,  New  York  Medical  Journal,  February  11  and  18,  1905, 
2  Brain,  1904,  p.  526. 


600  TUMOBS  OF  TEE  BEAIN. 

Cerebellar  staggering  is  also  due  to  a  cessation  of  outward  impulses 
which  hold  many  of  the  body  and  neck  muscles  in  proper  tonic  con- 
traction. An  abnormal  position  of  the  head  and  body  occurs  in  cere- 
bellar disease.  The  head  is  turned  back  and  down  to  the  side  of  the 
tumor  and  is  held  very  rigid  on  any  change  of  posture.  When 
standing  the  foot  on  the  side  of  the  tumor  is  thrown  outward  and  the 
shoulder  elevated  to  help  balance  the  body.  Batten^  observed  that  a 
patient  with  a  tumor  of  the  right  lobe  of  the  cerebellum  carried  his 
head  inclined  toward  the  left  shoulder  and  Mills  ^  has  seen  the  same 
symptom.  I  have  often  seen  abnormal  postures  of  the  head  and  body 
with  cerebellar  tumors  but  do  not  think  that  the  direction  of  the  head 
is  a  definite  sign  of  the  side  on  which  the  tumor  lies.  Sudden  relax- 
ation of  the  muscles  of  the  legs  may  occur,  leading  to  falls  which  have 
been  termed  cerebellar  attacks.  They  are  often  attended  by  a  loud 
tinnitus,  vertigo,  sudden  blindness  or  a  loss  of  consciousness,  and  tonic 
spasms  lasting  several  minutes.  Sometimes  the  patients  fall  to  one  side, 
or  forward.^  Bablnski  has  named  this  symptom  hemiasynergia.  When 
the  middle  peduncle  of  the  cerebellum  is  invaded  there  are  usually 
cranial  nerve  symptoms  produced,  and  these  are  always  upon  the  side 
on  which  the  tumor  lies.  Hence  the  combination  of  the  staggering 
toward  one  side  with  cranial  nerve  symptoms  of  the  opposite  side  will 
indicate  inevitably  which  peduncle  of  the  cerebellum  is  involved.  Thus 
in  the  case  shown  in  Fig.  236  the  diagnosis  was  made  before  death  from 
this  combination  of  symptoms.  A  deviation  of  the  eyes  called  "  skew 
deviation  "  has  been  seen  in  some  cases,  one  eye  directed  down  and 
inwards,  the  other  upwards  and  outwards. 

Another  symptom  of  cerebellar  origin  is  an  imperfect  muscular 
tone  in  the  muscles  of  the  arm  and  leg  on  the  side  of  the  tumor.  It 
is  uniformly  admitted  that  the  cerebellum  has  a  direct  tonic  influence 
upon  the  muscles,  and  that  this  influence  is  homolateral.  This  lack 
of  tone  is  attended  by  an  awkwardness  of  movement  not  due  to  any 
actual  weakness,  but  to  an  imperfect  co5rdination  of  the  motions 
performed.  Thus,  rapid  movements  made  by  both  hands  or  fingers 
together  often  prove  that  the  hand  on  the  side  of  the  lesion  is  moved 
less  freely,  rapidly,  and  skilfully ;  and  other  tests  may  show  a  true 
ataxia.  There  may  be  also  an  ataxia  of  the  leg,  so  that  to  stand  on 
one  foot  is  impossible.  Such  an  ataxia  in  the  leg  may  lead  to  stagger- 
ing toward  the  side  of  the  tumor.  This  symptom  has  been  named 
diadokokinesis.  The  maintenance  of  a  proper  tone  in  the  muscles 
appears  to  have  some  relation  to  the  response  of  those  muscles  to  irri- 
tation, and  it  is  probably  on  this  account  that  lesions  of  the  cerebellar 
peduncles  or  of  the  cerebellum  are  usually  attended  by  a  loss  of  knee- 
jerk  and  of  the  deep  reflexes  on  the  side  of  the  lesion  ;  yet  this  symptom 
of  a  loss  of  reflex  on  the  side  of  the  tumor  is  not  always  found  in  the 
cases  of  pontine  angular  growth.  It  has  been  observed,  however,  in 
too  many  cases  to  allow  us  to  overlook  it. 

1  Brain,  Part  101,  1903.     2  New  York  Medical  Journal,  February  11,  1905. 
'  New  York  Medical  Journal,  February  11,  1905. 


SYMPTOMS.  601 

Tumors  of  the  Cerebello-pontine  Angle.  —  Tumors  of  this  locality 
are  not  un(5ommon. 

The  symptoms  which  indicate  the  location  of  a  tumor  in  the  cere- 
bellar pontine  angle  or  upon  the  acoustic  nerve  may  be  divided  into 
three  classes  :  (1)  Those  referable  to  the  cranial  nerves  ;  (2)  those  refer- 
able to  the  involvement  of  the  cerebellar  peduncles  ;  and  (3)  those  refer- 
able to  compression  of  the  tracts  passing  through  the  pons. 

1.  Symptoms  Referable  to  the  Cranial  Nerves  : 

(a)  Slight  anaesthesia  of  the  face  on  the  side  of  the  tumor,  first 
shown  by  corneal  anaesthesia  and  an  absence  of  the  corneal  reflex. 

(6)  Weakness  of  the  external  rectus  muscle  of  the  eye  on  the  side 
of  the  tumor,  due  to  a  pressure  on  the  sixth  nerve.  This  may  be  first 
noticed  by  the  occurrence  of  nystagmus  on  turning  the  eyes  toward 
the  side  of  the  tumor,  and  later  a  decided  internal  strabismus  may 
occur.     The  eye  looks  away  from  the  tumor. 

(c)  A  weakness  of  the  muscles  of  the  face  on  the  side  of  the  tumor, 
first  noticeable  in  a  slight  disturbance  of  the  act  of  winking  and  a  slight 
lack  of  expression,  which  is  later  followed  by  an  actual  weakness  of  all 
the  muscles  supplied  by  the  seventh  nerve.  Pressure  on  this  nerve  is 
rarely,  if  ever,  sufficient,  however,  to  cause  a  reaction  of  degeneration 
in  these  muscles. 

(d)  Tinnitus  in  the  ear  on  the  side  of  the  tumor,  soon  followed  by 
some  deafness  in  the  ear,  which  may  be  slight  and  only  detected  by 
tests  with  the  Galton  whistle,  high  notes  or  low  notes,  or  both,  being 
lost.  This  deafness  may  be  as  marked  for  bone  conduction  as  for  ear 
conduction. 

(e)  Vertigo,  felt  by  the  patient  as  a  sensation  of  falling  toward  one 
side,  or  as  a  sensation  of  rotary  movement  of  the  body,  both  of  which 
symptoms  must  be  ascribed  to  irritation  of  the  labyrinthine  division  of 
the  auditory  nerve. 

(/)  Difficulty  of  swallowing  and  hoarseness  of  the  voice,  owing  to 
pressure  on  the  ninth  nerve. 

( g)  Sudden  attacks,  which  may  be  called  vagal  attacks,  referable  to 
irritation  of  the  tenth  nerve,  consisting  of  rapid  pulse,  sensations  of 
flushing  of  the  body  or  of  the  head,  sudden  faintness  without  an  actual 
loss  of  consciousness,  attended  by  more  or  less  vertigo. 

(Ji)  Paralysis  of  the  tongue,  shown  by  thickness  of  speech  or  dif- 
ficulty of  articulation,  and  by  protrusion  of  the  tongue  toward  the  side 
of  the  tumor. 

2.  Symptoms  Referable  to  the  Involvement  of  the  Cerebellar  Peduncles. 
—  A  staggering  gait  and  feeling  of  uncertainty  of  the  position  of  the 
body  in  space  when  supported  upon  the  legs,  which  causes  swaying 
when  the  eyes  are  closed  and  staggering  when  the  eyes  are  open,  at- 
tended by  a  disagreeable  sense  of  vertigo,  which  leads  the  patient  to 
seek  support  whenever  in  a.  standing  posture.  The  direction  of  the 
staggering  is,  in  the  majority  of  instances,  away  from  the  tumor,  al- 
though cases  have  been  put  on  record  in  which  the  patient  staggered 
toward  the  side  of  the  tumor.     The  direction  of  the  staggering  is  not 


B02  TVMOBS  OF  THE  BMAIN. 

to  be  taken  in  itself  as  a  very  important  symptom,  but  the  fact  that  the 
patient  staggers  uniformly  toward  one  side  is  an  important  symptom  of 
an  aflfection  of  the  peduncles  of  the  cerebellum  as  they  enter  the  organ, 
and  therefore  the  existence  of  staggering  in  a  definite  direction,  com- 
bined with  evidences  of  the  implication  of  the  cranial  nerves  upon  one 
side,  is  a  definite  local  sign  of  a  tumor  in  the  cerebellar  pontine  angle. 

A  loss  of  muscular  tone  and  abnormal  positions  of  the  head  are 
present  in  these  cases  as  in  cerebellar  tumors. 

3.  Symptoms  Referable  to  Compression  of  the  Tracts  Passing 
Through  the  Pons.  —  A  third  set  of  symptoms  is  to  be  traced  to  a  com- 
pression of  the  motor  tracts  and  sensory  tracts  passing  through  the  pons 
and  medulla  on  their  way  to  and  from  the  limbs  of  the  opposite  side. 
Compression  of  the  pons  is  very  much  less  likely  to  cause  disturbance 
in  the  transmission  of  sensation  than  in  the  transmission  of  motor  im- 
pulses, and  therefore  weakness  of  a  hemiplegic  type  in  the  arm  and  the 
leg  in  the  side  opposite  to  the  tumor  is  more  commonly  observed  than 
is  anaesthesia  in  the  corresponding  parts.  This  weakness  is  usually 
attended  by  an  exaggeration  of  the  reflex,  by  an  increase  in  the  knee- 
jerk,  ankle  clonus,  and  Babinski  reflex,  and  such  exaggeration  is  doubly 
evident  because  of  the  loss  of  the  knee-jerk  upon  the  side  correspond- 
ing to  the  tumor.  This  weakness  of  one  leg  may  intensify  the  diffi- 
culty of  walking  and  may  influence  very  markedly  the  gait.  A  patient 
with  a  weak  leg  has  something  of  a  tendency  to  stagger  toward  the  leg 
that  is  weak,  to  use  it  less  freely,  and  to  bend  downward  toward  that 
side.  It  has  already  been  remarked  that  many  of  these  patients  stag- 
ger away  from  the  tumor,  and  it  is  not  at  all  impossible  that  this  direc- 
tion of  the  staggering  is  due  to  the  imperfect  use  of  the  leg  on  the  side 
opposite  to  the  tumor.  The  actual  weakness  present  in  the  arm  or  leg 
may  be  easily  detected  by  dynamometer  tests,  and  in  many  cases  has 
amounted  to  such  a  marked  hemiplegia  that  any  use  of  the  arm  or  of 
the  leg  was  impossible.  While  anaesthesia  and  analgesia  in  the  limbs 
of  the  side  opposite  to  the  tumor  is  much  less  commonly  observ^ed  than 
is  paralysis,  many  patients  complain  of  feelings  of  numbness,  tingling, 
gooseflesh,  or  burning  in  these  limbs ;  and  such  parsesthesias  may  be 
followed  by  an  actual  disturbance  of  sensation.  In  the  advanced  stages 
of  these  cases,  in  which  the  patients  have  died  of  the  tumor,  a  hemi- 
plegia has  developed  toward  the  close  of  life,  not,  however,  involving 
the  face  and  it  is  in  these  extreme  cases  that  anaesthesia  has  been  ob- 
served in  the  paralyzed  limbs. ^ 

Diagnosis.  —  The  review  of  the  symptoms  just  described  will  con- 
vince the  reader  that  in  the  majority  of  cases  of  tumor  of  the  brain 
there  are  sufficient  evidences  of  the  existence  of  disease  within  the 
cranial  cavity,  and  that  the  gradual  development  and  progress  of  the 
disease  will  enable  the  physician  to  come  to  the  conclusion  that  it  is  of 
the  nature  of  a  tumor  If  in  any  case  the  symptoms  which  are  pres- 
ent are  carefully  classified,  the  general  symptoms  being  separated  from 
the  local  symptoms,  and  the  order  of  appearance  of  the  local  symptoms 

'Starr.     Amer.  Jour.  Med.  Sci.,  April,  1910.     Six  cases. 


DIAGNOSIS.  603 

fully  determined,  it  will  usually  be  possible  to  reacu  a  conclusion  as  to 
the  situation  of  the  tumor.  The  combination  of  local  symptoms  is 
sometimes  as  characteristic  as  their  order  of  appearance  and  method  of 
extension,  as,  for  example,  in  cases  of  tumors  of  the  crus  or  of  the 
corpora  quadrigemina. 

It  is  not  to  be  forgotten,  however,  that  tumors  of  the  frontal  lobes 
and  tumors  of  the  temporal  lobes,  especially  those  situated  in  the  right 
hemisphere,  often  fail  to  cause  any  local  symptoms.  The  absence  of 
distinct  local  symptoms,  when  general  symptoms  are  present,  will  point 
to  these  localities  as  the  probable  position  of  the  suspected  tumor. 

It  is  not  to  be  forgotten  that  many  local  symptoms  are  produced 
indirectly  by  pressure  upon  parts  not  far  from  the  tumor,  but  yet  not 
directly  invaded  in  its  growth. 

It  is  also  to  be  remembered  that  tumors  pressing  upon  large  vessels 
of  the  brain  may  so  disturb  the  circulation  as  to  produce  symptoms 
quite  similar  to  those  of  thrombosis,  and  these  symptoms  may  be  due  to 
suspension  of  function  of  a  part  lying  at  some  distance  from  the  tumor. 

Gushing  ^  has  called  attention  to  the  fact  that  a  tumor  anywhere  in 
the  brain  may  so  displace  the  pons  as  to  put  the  basal  arteries  on  the 
stretch ;  and  that  these  arteries  may  then  compress  the  cranial  nerves, 
especially  the  sixth  to  the  tenth.  This  may  give  rise  to  a  mistaken 
diagnosis  of  a  tumor  on  the  base. 

The  diagnosis  of  the  variety  of  the  tumor  present  can  be  reached 
only  by  careful  study  of  the  general  history  of  the  patient  and  by  a 
consideration  of  those  facts  that  have  been  already  mentioned  in  the 
discussion  of  the  varieties  of  brain  tumor.  It  is  possible  to  determine 
the  question  of  the  tubercular  nature  of  the  tumor  by  tuberculin  injec- 
tions or  by  Calmette's  test.  It  is  possible  to  establish  the  existence 
of  gumma  by  lumbar  puncture  and  the  discovery  of  lymphocytes  in 
the  cerebro-spinal  fluid,  or  by  Wassermann's  blood  tests. 

It  is  therefore  evident  that  in  the  diagnosis  of  the  disease  there  are 
always  three  questions  to  be  settled  :  first,  the  existence  of  a  tumor ; 
secondly,  its  situation  ;  thirdly,  its  variety.  A  study  of  the  symptoms 
will  usually  enable  the  physician  to  reach  an  answer  to  the  first  two 
questions,  but  the  answer  to  the  last  question  will  always  remain 
uncertain. 

The  symptoms  of  brain  abscess  may  be  the  same  in  kind  as  those  of 
brain  tumor,  since  both  produce  an  increase  of  intracranial  pressure  and 
a  progressive  destruction  of  brain  tissue.  In  their  origin,  mode  of 
development,  progress,  and  termination,  however,  there  are  not  infre- 
quently marked  differences.  Brain  abscess  develops  most  commonly 
after  severe  injuries  or  in  conjunction  with  suppurative  affections  of  the 
inner  ear  and  of  the  nasal  and  orbital  cavities,  and  with  caries  of  any 
of  the  cranial  V^ones  which  lie  in  contact  with  the  membranes.  These 
rarely  occur  prior  to  the  development  of  brain  tumor.  The  symptoms 
common  to  tumor  and  absr;ess  may  develop  after  a  blow  on  the  head, 
but  when  the  condition  is  one  of  abscess  the  symptoms  appear  in  more 

» Brain,  1911. 


604  TUM0B8  OF  THE  BEAIN. 

rapid,  succession,  with  greater  severity,  and  more  frequently  with  fever 
than  wlien  the  condition  is  that  of  tumor.  Furthermore,  the  symp- 
toms of  abscess,  after  appearing  suddenly,  often  subside,  the  abscess 
becoming  latent,  and  all  symptoms  disappearing  for  months  or  years, 
and  then  breaking  out  again  with  suddenly  fatal  termination.  This 
course  contrasts  markedly  with  that  in  a  case  of  a  tumor  where  a  grad- 
ual progress  with  slowly  increasing  intensity  of  all  the  symptoms  is 
found.  The  constant  addition  of  new  symptoms  is  usual  in  tumors, 
and  a  temporary  remission  rather  than  intermission  of  the  symptoms 
is  the  rule  when  the  progress  is  not  continuous.  There  may  also  be 
some  points  of  distinction  found  in  the  individual  symptoms.  Thus 
headache  is  more  severe  and  paroxysmal  with  tumor;  optic  neuritis  is 
much  more  commonly  found  with  tumor ;  mental  changes  are  more 
gradual  and  constant  with  tumor,  and  local  symptoms  are  slower  in 
onset  and  more  apt  to  develop  with  tumor.  A  complication  of  tumor 
not  infrequent  is  cerebral  hemorrhage.  Meningitis  is  the  usual  com- 
plication of  abscess.  Lastly,  a  duration  of  from  one  to  two  years,  with 
symptoms  constantly  present,  points  directly  to  tumor. 

Tubercular  meningitis  is  under  certain  circumstances  easily  mistaken 
for  cerebral  tumor.  This  is  not  true  of  the  ordinary  cases  of  tubercu- 
lar meningitis  with  acute  hydrocephalus,  which  develop  marked  symp- 
toms rapidly  and  terminate  fatally  within  four  or  six  weeks.  But  there 
are  a  number  of  cases  of  tubercular  meningitis  which  present  a  chronic 
course  with  gradual  progress,  and  in  which  the  diagnosis  from  tuber- 
cular tumor  is  almost  impossible.  It  is  true  that  the  symptoms  often 
develop  rapidly  in  these  cases,  and  yet  this  is  sometimes  apparently  the 
case  in  tumor,  for,  unless  a  patient  is  carefully  watched,  the  early 
symptoms  of  tumor  may  escape  notice  for  some  time.  The  symptoms 
of  chronic  tubercular  meningitis  may  be  the  same  as  those  described  as 
general  symptoms  of  brain  tumor,  but  the  headache  is  more  severe  in 
meningitis  and  more  continuous  ;  there  is  more  likely  to  be  hypersen- 
sitiveness  to  light,  sound,  or  touch  in  meningitis,  and  optic  neuritis 
develops  less  frequently,  less  rapidly,  and  with  less  intensity  than  in 
tumor.  Tubercles  upon  the  choroid  are  found  more  frequently  in  men- 
ingitis than  in  tubercular  tumor.  It  is,  of  course,  understood  that  a 
localized  meningitis  may  give  rise  to  the  same  symptoms  as  a  small 
tumor,  and  then  the  differentiation  is  impossible.  This  is  more  common 
about  the  base  of  the  brain,  in  the  region  of  the  cranial  nerves,  than 
elsewhere.  It  is  also  to  be  remembered  that  a  chronic  progressive 
meningitis  may  develop  in  the  vicinity  of  a  tumor.  Here,  again,  the 
diagnosis  will  be  impossible.  A  gradual  subsidence  of  the  symptoms, 
with  recovery,  will  point  to  meningitis  rather  than  to  tumor. 

Chronic  hydrocephalus,  while  not  infrequently  the  result  of  tumor 
or  meningitis,  may  be  due  to  a  ch^'onic  inflammation  of  the  ependyma 
of  the  ventricles.  It  then  advances  slowly.  The  fluid  within  the 
ventricles  produces  pressure  upon  the  brain,  causing  atrophy.  The 
course  is  chronic,  and  the  general  symptoms  are  those  of  cerebral 
tumor.     The  local  symptoms,  however,  differ  in  some  respects  from 


DIAGNOSIS.  605 

those  of  tumor.  Spastic  paralysis  develops  with  chronic  hydrocephalus 
without  lo(!klized  spasms,  aud  is  always  bilateral ;  the  lower  limbs  are 
affected  more  intensely  than  the  upper.  The  child  presents  the  extended, 
adducted,  stiif  legs,  with  overlapping  knees,  rigid  muscles,  increased 
tendon  reflexes,  and  the  spastic  gait  so  familiar  in  infantile  diplegia 
(see  Chapter  XXVIII.),  and,  in  addition,  the  hands  move  without 
proper  coordination.  As  the  disease  progresses  the  crura  cerebri  and 
pons  may  be  displaced  by  the  pressure  of  the  fluid,  and  irregular  symp- 
toms due  to  stretching  of  the  cranial  nerves  may  appear.  These,  with 
the  paraplegia,  may  lead  to  a  suspicion  of  a  tumor  of  the  pons  or  base 
of  the  brain,  and  only  by  the  order  of  development  of  the  symptoms 
can  the  differentiation  be  made. 

The  diagnosis  from  syphilitic  meningitis  is  considered  in  Chapter 
XXXVII. 

The  diagnosis  between  cerebral  tumor  and  cerebral  hemorrhage  will 
be  necessary  only  in  a  few  cases  in  which  the  onset  of  the  symptoms 
has  been  very  sudden.  There  are  a  few  cases  of  tumor,  chiefly  glioma, 
in  which  the  growth  has  been  latent  for  some  time,  and  has  then  given 
rise  suddenly,  after  a  blow  on  the  head,  or  exposure  to  the  sun,  or  some 
other  accidental  influence,  to  well-marked  symptoms.  These  are  usually 
both  general  and  local  symptoms,  chiefly  the  latter.  The  suspicion  of 
a  tumor  will  be  aroused  if,  after  such  an  apoplectic  stroke,  the  symp- 
toms persist  and  increase  instead  of  subsiding,  and  if  headache,  con- 
vulsions, and  optic  neuritis  appear.  Hemorrhage  alone  never  gives 
rise  to  the  last-named  symptom. 

A  novel  means  of  establishing  the  diagnosis  of  brain  tumor  and  of 
ascertaining  at  the  same  time  its  nature  has  been  proposed  by  Neisser 
and  by  Pollack  ^  and  fully  described  by  Pfeifer.^  It  has  been  adopted 
by  F.  Krause.^  A  very  small  hole  is  bored  in  the  skull,  the  scalp  over 
it  being  anaesthetized  by  cocaine,  and  a  hollow  needle  is  thrust  into  the 
brain  under  strict  aseptic  precautions  and  some  aspiration  is  employed. 
The  needle  is  then  withdrawn  and  its  contents  examined.  In  this  way 
tumors  have  been  detected  and  their  nature  determined.  Such  punc- 
tures are  not  often  injurious,  but  two  deaths  due  to  hemorrhage  are  on 
record.  By  their  means  abscess,  cysts,  hemorrhages  and  hydrocephalus 
have  been  diagnosticated  as  well  as  tumors. 

The  examination  of  the  head  by  means  of  the  a:;-rays  does  not  often 
help  in  the  diagnosis  of  brain  tumor.  Occasionally  cases  have  been  re- 
ported where  the  presence  of  the  tumor  appeared  to  be  visible.  In  the 
majority  of  cases  nothing  can  be  seen,  as  the  consistency  of  the  tumor 
is  about  the  same  as  that  of  the  brain. 

There  are  a  few  general  diseases  which  present  certain  symptoms 
somewhat  similar  to  those  occurring  in  brain  tumor,  and  these  should 
always  be  kept  in  mind.  They  are  extreme  anaemia,  with  defective 
vision,  from  myopia  or  hypermetropia ;  chronic  lead   poisoning  and 

'  Neisser  and  Pollack.  Mittheilungcn  aas  der  Grenzgeb.  der  Med.  u.  Cliir. ,  Bd. 
xiii.,  1906. 

'Pfeifer.     Arch.  f.  PHych.,  Bd.  xlii.,  pp.  451-fi03,  1907. 
^F.  Krause.     Chirurgie  des  Gehirns,  Berlin,  1908. 


606  TUMOBS  OF  THE  BJRAIN. 

chronic  diffuse  nephritis  or  contracted  kidneys.  The  knowledge  that 
these  diseases  may  simulate  brain  tumor  will  lead  the  physician  to  be 
upon  his  guard.  It  is  not  necessary  to  mention  the  numerous  points 
of  the  differential  diagnosis  which  will  enable  a  definite  conclusion  to 
be  reached  in  any  case. 

The  Course  of  the  Disease.  —  The  general  history  of  the  progress 
of  a  patient  suffering  from  brain  tumor  has  to  some  extent  been  indi- 
cated in  the  discussion  of  the  symptoms.  Suffice  it  to  say  that  a 
gradual  increase  in  the  number  and  intensity  of  the  various  symptoms 
is  usual.  In  some  cases  the  general  symptoms  precede  the  local  symp- 
toms by  several  months,  and  optic  neuritis  does  not  often  appear  within 
three  months  of  the  beginning  of  the  symptoms,  unless  the  tumor  is  in 
the  cerebellum  or  on  the  base  of  the  brain.  In  some  cases  the  local 
symptoms  appear  before  the  general  symptoms,  especially  if  the  tumor 
is  located  in  the  motor  cortex,  when  the  case  may  be  regarded  for  some 
time  as  one  of  cortical  epilepsy  until  the  general  symptoms  of  brain 
tumor  intervene.'^ 

As  the  case  goes  on  and  both  local  and  general  symptoms  become 
more  numerous  the  suffering  of  the  patient  becomes  more  intense. 

If  the  case  is  one  of  gumma  it  may  be  possible  to  relieve  the  symptoms 
and,  by  a  progressive  course  of  treatment  to  cure  the  patient  entirely. 
Under  these  circumstances  the  symptoms  gradually  subside  and  become 
less  in  intensity  up  to  recovery.  In  other  cases  it  is  possible  to  locate 
the  tumor  absolutely  in  a  position  accessible  to  the  surgeon  and  to 
remove  it  by  operation,  and  under  these  circumstances  the  recovery  of 
the  patient  is  gradual  but  progressive  after  the  operation  is  over.  The 
brain  resumes  its  functions  after  the  pressure  of  the  tumor  is  removed. 
When  a  portion  of  the  brain  has  been  injured  in  the  removal  of  the 
tumor  recovery  may  be  imperfect,  with  some  defects  of  sight,  or  motion, 
or  sensation  remaining.  In  those  cases  that  are  not  subject  to  specific 
treatment  and  in  which  the  tum3r  cannot  be  removed  the  course  is 
progressively  downward,  the  patient  suffering  more  and  more  intensely 
as  the  tumor  grows,  and  finally  passing  into  a  state  of  coma  or  dying 
in  convulsions. 

The  average  duration  of  the  symptoms  is  said  to  be  three  years,  but 
individual  cases  vary  greatly. 

There  are  a  few  cases,  however,  in  which,  either  spontaneously  or 
under  specific  treatment,  a  tumor  has  ceased  to  grow  and  the  patient 
has  apparently  recovered  and  remained  quite  well  for  some  months  or 
even  years,  the  brain  apparently  resuming  its  function.  Two  such 
cases  have  come  under  my  observation.  In  one  the  recovery  lasted 
four  months,  sudden  death  following,  due  to  the  rupture  of  a  cyst 
which  lay  at  the  side  of  a  sarcoma.  In  the  other  an  interval  of  eight 
years  occurred,  after  which  the  symptoms  returned  and  caused  death. 
In  the  last  case  optic  neuritis,  which  was  present  at  the  first  attack, 
subsided  entirely,  but  recurred  at  the  second  attack.  The  tumor  was 
a  sarcoma  in  the  cerebellum.  In  both  cases  there  was  no  evidence  of 
1  Kocher,  Zeitsch.  f.  Chirurgie,  June,  1893, 


PBOGNOSIS.  607 

syphilis,  yet  the  treatment,  which  was  apparently  successful  was  by 
inunctions ■^of  mercury  and  large  doses  (300  to  400  grains  daily)  of 
iodide  of  potassium. 

In  some  cases  of  tumor  of  the  brain  sudden  death  has  occurred 
unexpectedly.  I  have  witnessed  four  such  deaths  in  patients  about  to 
be  subjected  to  operation.  In  two  of  these  cases  tumor  of  the  cere- 
bellum near  to  and  pressing  upon  the  medulla  was  found.  In  one  of 
these  cases  the  heart  continued  to  beat  six  hours  after  voluntary  res- 
piratory movements  had  ceased. 

Prognosis.  —  It  is  evident  from  this  statement  of  the  course  of  the 
disease  that  the  general  prognosis  in  brain  tumor  is  unfavorable.  We 
have  seen  from  a  table  on  page  572  that  but  9  per  cent,  of  tumors  of 
the  brain  are  open  to  operation.  It  is  therefore  evident  that  in  the 
vast  majority  of  the  cases  we  cannot  give  the  patient  any  hope. 

If  a  tumor  is  syphilitic  salvarsan  may  be  given  in  repeated  doses  at 
intervals  of  two  weeks.  In  some  cases  its  effect  is  immediately  bene- 
ficial. If  it  does  not  relieve  the  symptoms  mercury  and  iodide  are  to 
be  tried.  I  prefer  inunctions  of  mercury,  one  drachm  of  blue  ointment 
being  rubbed  in  at  night  after  a  hot  bath,  a  different  part  of  the  body 
being  selected  for  each  application,  and  after  the  application  being  cov- 
ered with  bandages  so  that  the  process  of  absorption  of  the  ointment 
remaining  upon  the  skin  may  occur  during  the  night.  At  least  two 
ounces  of  blue  ointment  should  thus  be  used,  and  if  improvement  is  evi- 
dent the  inunctions  may  be  repeated  after  an  interval  of  two  weeks. 
The  occurrence  of  salivation  will  necessitate  the  cessation  of  the  use  of 
mercury  temporarily.  At  the  same  time  large  doses  of  iodide  of 
potash  should  be  given,  beginning  with  twenty  grains  three  times  a 
day,  and  increasing  the  dose  one  or  more  grains  daily  until  100  grains 
three  times  a  day  have  been  reached.  The  iodide  may  be  given  in 
Vichy  water  or  in  milk,  before  or  after  meals,  according  to  the  con- 
dition of  digestion.     My  preference  is  to  give  it  before  meals. 

Care  in  the  regulation  of  the  diet,  the  use  of  simple  and  nutritious 
food,  frequent  massage  to  aid  digestion,  and  a  daily  hot  bath  at  a 
temperature  of  100°  F.  continued  for  fifteen  to  twenty  minutes  are 
essential  during  the  taking  of  these  large  doses  of  iodide. 

When  the  tumor  is  of  a  gummatous  nature  a  decided  improvement 
in  all  the  symptoms,  and  particularly  in  insomnia  and  headache, 
should  be  observed  within  a  month.  Such  improvement  may,  how- 
ever, occur  in  cases  of  either  sarcoma,  or  glioma,  or  cystic  tumors.  It 
is  therefore  necessary  to  keep  up  this  treatment  for  two  months 
longer.  If  the  improvement  continues  and  the  patient  gradually 
recovers,  it  is  probable  that  a  gumma  has  been  absorbed.  In  a  case 
under  my  observation  in  which  the  symptoms  in  January,  1903,  were 
intense  headache,  insomnia,  mental  apathy,  staggering  gait,  great 
general  weakness,  optic  neuritis  in  both  eyes,  with  partial  blindness 
in  the  right  eye  and  total  paralysis  of  the  right  third  nerve,  par- 
tial anaesthesia  of  tlie  right  side  of  the  face,  and  paresis  of  the  right 
sixth  and  seventh  nerves,  there  was  by  November,  1893,  a  complete 


608  TUMOES  OF  THE  BEAIN. 

recovery,  which  still  persists,  an  occasional  nocturnal  headache  being 
the  only  symptom  remaining.  Five  courses  of  inunction  have  been 
employed  and  iodide  has  been  given  to  the  extent  of  250  grains  a  day, 
the  dose  being  varied  from  time  to  time  when  symptoms  of  intolerance 
appeared.  Thus  in  ten  months  a  basal  gumma  of  considerable  size 
has  been  absorbed  by  persistent  treatment. 

If  the  tumor  present  is  a  sarcoma  or  glioma,  and  an  improvement 
has  occurred  during  the  first  months  of  treatment,  such  improvement 
will  not  always  persist,  and  therefore  a  return  of  the  symptoms  during 
the  course  of  specific  treatment  is  a  pretty  sure  proof  that  the  tumor  is 
not  of  the  nature  of  a  gumma.  It  is  useless  to  continue  specific  treat- 
ment after  three  months  of  thorough  trial ;  it  is  better  to  refuse  medical 
treatment  or  to  depend  entirely  upon  palliatives  under  these  circum- 
stances. It  must  be  stated,  however,  that  some  authorities  believe 
that  tumors  of  the  brain,  not  syphilitic  in  nature,  have  been  cured  by 
the  persistent  use  of  large  doses  of  iodide.  In  every  case,  therefore, 
where  surgical  treatment  is  impossible  it  is  well  to  employ  this  remedy. 

Treatment.  —  1.  Medical  Treatment.  —  Headache  can  be  relieved 
by  a  free  use  of  phenacetin,  antipyrine,  or  acetanilid.  The  doses  of 
these  druo-s  which  must  be  used  are  larger  than  those  commonly  em- 
ployed, and  it  is  my  practice  to  begin  with  the  ordinary  dose  and  rapidly 
increase  the  number  of  doses  given,  combining  with  the  drug  any  heart 
stimulant,  cafieine  being  the  one  preferred.  If  the  patient  be  carefully 
watched  while  this  is  being  done  it  will  soon  be  found  possible  to  give 
safely  twenty  grains  of  antipyrine,  fifteen  to  twenty  grains  of  phena- 
cetin, or  ten  grains  of  acetanilid  at  a  dose,  and  this  dose  may  be  repeated 
after  three  hours,  provided  the  headache  returns.  In  a  few  cases  the 
headache  may  be  benefited  by  ice-bags  to  the  head,  by  hot  baths,  or 
by  ergot.  If  these  remedies  are  useless  resort  must  be  had  to  mor- 
phine, but  this  drug  is  especially  unsatisfactory  in  the  treatment  of 
headache  from  brain  tumors,  unless  very  large  doses  are  given.  Vomit- 
ing and  vertigo  in  brain  tumor  are  best  relieved  by  the  use  of  bromide 
of  sodium  or  by  hydrobromate  of  hyoscine  in  ^rw  E^-  ^^^^  repeated 
every  four  hours. 

The  course  of  optic  neuritis  cannot  be  arrested,  though  it  may  be 
somewhat  delayed  by  cupping  the  temples.  While  strychnine  will  at 
any  time,  when  given  hypodermically,  improve  temporarily  the  power 
of  vision,  yet  it  does  no  permanent  good,  and  is  thought  by  some  to 
hasten  the  progress  of  the  disease. 

Epileptiform  convulsions  may  be  reduced  in  frequency  by  the  free 
use  of  bromides,  but  cannot  be  arrested  as  long  as  the  disease  goes  on. 
It  is  thus  evident  that  the  treatment  of  the  general  symptoms  of  brain 
tumor  is  exceedingly  unsatisfactory.  There  is  no  treatment  known 
that  will  in  any  way  affect  the  local  symptoms,  massage  and  electricity 
to  paralyzed  limbs  being  mere  palliatives,  capable  only  of  maintaining 
the  nutrition  of  the  muscles. 

2.  The  Surgical  Treatment  of  brain  tumors  is  a  subject  which  has 
awakened  much  interest  of  late,  inasmuch  as  over  1,000  tumors  have 
been  operated  upon  up  to  the  present  time. 


TBEATMENT.  609 

A  careful  review  of  a  large  number  of  cases  of  brain  tumor,  exam- 
ined post  mortem,  shows  that  about  10  per  cent,  of  tumors  are  open 
to  surgical  treatment.  The  most  recent  statistics  of  results  in  such 
cases  have  been  published  by  Dr.  Philips  Coombes  Knapp,^  whose 
table  I  reproduce  below.  Of  828  tumors  operated  on  up  to  July, 
1905,  it  was  found  that  in  471  cases  the  tumor  was  found  and 
removed;  in  189  cases  the  tumor  was  not  found,  in  104  cases  the 
operation  was  a  palliative  one  only,  and  in  64  cases  the  tumor,  though 
found,  could  not  be  safely  removed.  Further  analysis  of  the  cases, 
471  in  number,  in  which  the  tumor  was  removed,  shows  that  the 
diagnosis  was  correct,  the  removal  easy,  and  the  recovery  or  improve- 
ment most  satisfactory  in  those  cases  in  which  the  tumor  lay  near  the 
Rolandic  or  Sylvian  fissures,  while  the  mortality  was  greatest  in  the 
cerebellar  cases. 

These  statistics  are  quite  in  accord  with  my  own  experience.  It  is 
evident  then  that  out  of  100  brain  tumors  we  may  expect  success  in 
surgical  treatment  in  but  five  only.  Yet  there  is  no  doubt  that  even 
if  the  number  of  lives  saved  is  small  it  is  our  duty  to  study  every  case 
with  our  minds  fixed  on  the  question  of  possible  surgical  relief. 

Table  XV.  —  Kesults  in  Operations  for  tumors  of  the  Brain. 

Not  im-  Result  not 

Recovered.  Improved.       proved.  Died.  stated.  Total. 

Removed 112  122  78  123  36  471 

Not  found 4  24  61  87  13  189 

Impossible 0  11  11  40  2  64 

Palliative ^  4^  36  J5  5  104 

Total 116  205  186  265  56  828 

Frontal 15  6  3  8  2  34 

Central 52  82  43  54  5  236 

Parietal 7  5  5  12  0  29 

Temporal 2  4  6  5  1  18 

Occipital 3  3  2  2  1  11 

Cerebellum 7  16  8  23  1  55 

Not  stated 26  6  11  19  26  88 

It  is  imperative  to  consider  the  possibility  of  operation  in  every 
case  of  brain  tumor.  Success  in  these  operations  depends  (1)  on  the 
situation  of  the  tumor ;  (2)  on  its  removal  early  in  the  course  of  the 
disease ;  (3)  on  the  variety  of  tumor  found ;  (4)  on  the  amount  of 
brain  tissue  destroyed  either  by  the  tumor  or  in  the  course  of  the 
operation. 

An  analysis  of  the  successful  cases  shows  that  they  Were  chiefly 
tumors  situated  about  the  fissures  of  Rolando  or  Sylvius,  giving  rise  to 
motor  or  aphasic  symptoms  easily  localized.  I  have  had  a  number  of 
these  cases  ;  in  four  perfect  recovery  has  been  the  result,  and  the  patients 
are  now  alive  and  well.  A  number  of  tumors  have  been  successfully 
removed  from  the  frontal  convolutions,  from  the  parietal  region,  and 
from  the  occipital  region,  but  the  percentage  of  failures  in  these  locali- 
ties, due  probably  to  uncertainty  in  diagnosis,  was  greater  than  in  the 
motor  region.     I  have  had  successful  cases  in  each  of  these  localities. 

39  1  Boston  Med,  and  Surg.  Jour.,  Feb.  1,  1906. 


610  TUMOES  OF  THE  BEAIN. 

The  following  is  an  example  :  In  January,  1 905,  a  healthy  business 
man  began  to  suffer  from  severe  frontal  headaches,  sometimes  attended 
by  vomiting,  and  it  was  soon  noticed  that  he  misplaced  words  and  had 
difficulty  in  expressing  his  ideas.  These  symptoms  increased  steadily 
during  February,  and  early  in  March  he  had  a  temporary  attack  of 
right  hemiplegia  and  aphasia,  which,  however,  passed  oif  entirely  in 
two  days,  but  left  his  speech  more  defective.  This  was  followed  by 
recurring  attacks  of  twitching  in  the  right  hand  and  in  the  right  side 
of  the  face,  attended  and  followed  by  numbness  in  these  parts.  By  the 
middle  of  March  his  speech  defect  had  become  constant  and  he  could 
no  longer  write,  though  he  could  read  and  understand  everything  said 
to  him  ;  and  he  had  become  very  dull  and  drowsy. 

On  March  25  he  went  into  a  semi-stuporous  state  in  which  I  first 
saw  him.  I  found  an  extreme  choked  disc  in  both  eyes,  worse  in  the 
left ;  no  paralysis,  but  a  flatness  of  the  right  side  of  the  face  and  in- 
creased reflexes  in  the  right  limbs.  There  was  motor  aphasia.  I 
concurred  in  the  diagnosis  of  brain  tumor  made  by  his  physician.  Dr. 
Rutz,  and  located  the  tumor  in  the  left  third  frontal  convolution,  and 
recommended  operation. 

On  March  27  Dr.  Hartley  operated,  making  a  large  bony  flap  over 
the  left  temporal  region  four  inches  in  diameter.  When  the  dura  was 
opened  the  tumor  was  visible,  lying  in  the  middle  of  the  third  frontal 
convolution.  It  was  easily  shelled  out  by  a  spoon,  and  some  small 
discolored  parts  of  the  white  matter  of  the  brain  about  it  were  also 
taken  out,  leaving  a  clean  cavity. 

His  recovery  was  rapid.  The  day  after  the  operation  he  was  still 
speechless,  but  within  a  week  he  had  regained  his  speech,  could  read 
aloud,  and  could  write,  and  had  no  paralysis.  On  April  16  he  was 
up  and  the  wound  had  healed.  On  May  16  he  went  back  to  business 
in  apparently  good  health.  In  May,  1906,  he  had  no  return  of  brain 
symptoms. 

In  any  case,  where  the  tumor  is  cortical,  can  be  localized  accurately, 
and  is  accessible,  the  chance  of  success  is  good.  In  some  cases  tumors 
beneath  the  cortex  can  be  removed.  It  is  quite  safe  to  incise  the  brain 
to  a  depth  of  several  inches  if  the  ventricle  is  not  opened.  If  a 
tumor  is  not  seen  at  an  operation  it  may  be  felt  for,  the  finger  being 
thrust  into  the  brain  at  the  bottom  of  a  sulcus.  Thus  in  a  case  of  deep 
lying  tumor  of  the  parieto-occipital  region,  removed  for  me  by  McCosh, 
it  was  only  at  the  second  operation  and  by  a  deep  incision  that  this  was 
found.  It  proved  to  be  an  encapsulated  sarcoma  with  a  pedicle  attached 
to  the  falx,  and  its  removal  was  followed  by  apparent  recovery,  hemi- 
anopsia remaining.  The  tumor,  however,  recurred  some  months  later, 
and  the  patient  died.  It  is  to  be  remembered  that  deep  incisions  or 
lacerations  of  the  brain  in  the  removal  lead  to  permanent  symptoms, 
and  the  scar  is  likely  to  prove  the  focus  of  irritation  for  subsequent 
epileptic  attacks. 

The  cerebellum  is  very  difficult  to  reach,  only  a  small  part  of  each 
hemisphex'e  being  accessible  through  one  half  of  the  occipital  bone. 


TREATMENT.  611 

Gushing  has  however  succeeded  by  removing  the  entire  occipital  bone  ; 
and  since  1904  73  successful  cases  with  recovery  have  been  recorded/ 
A  few  tumors  from  the  cerebello-pontine  angle  involving  the  eighth 
nerve  have  been  successfully  removed,  but  this  is  a  very  difficult  locality 
to  reach. 

Gushing  has  succeeded  in  reaching  and  removing  tumors  of  the 
hypophysis,  entering  the  skull  through  the  mouth  above  the  upper  jaw. 

The  second  factor  in  success  is  the  promptness  with  which  the  need 
of  an  operation  is  recognized  and  the  procedure  undertaken.  Many 
patients  could  have  been  saved,  who  have  died,  had  the  tumor  been 
removed  at  an  earlier  time  in  the  course  of  the  case.  The  moment  an 
accessible  tumor  is  diagnosticated  an  operation  should  be  undertaken. 
It  is  a  mistake  to  try  medical  treatment,  even  antisyphilitic  treatment 
in  syphilitic  cases,  as  the  operation  even  in  these  affords  rapid  relief, 
and  in  cases  where  it  fails  valuable  time  is  lost. 

The  third  factor  in  success  is  the  variety  of  tumor  found.  The 
table  here  given  illustrates  this  fact. 

Table  XVI.  —  Table  of  Variety  of  Tumor  Eemoved,  and  Eesxjlt. 

Variety.                                                                         Recovered.  Died. 

Sarcoma      .......  52  20 

Gliosarcoma        ......  10  0 

Cyst 29  11 

Angioma    .....••           7  1 

Gumma      .......           8  3 

Tubercular          ......  19  8 

Fibroma     .......           7  2 

Endothelioma 5  4 

Glioma       .......  15  13 

Sarcoma,  gliosarcoma,  and  fibroma  are  usually  hard,  encapsulated, 
easily  separable  from  the  brain  tissue,  and  can  be  removed  without 
laceration. 

Gysts  of  the  brain  are  easily  emptied  when  found,  but  the  mere 
abstraction  of  the  fluid  gives  no  permanent  relief,  as  it  reaccumulates. 
It  is  necessary,  therefore,  either  to  drain  the  cyst  and  thus  secure  its 
closure  from  the  bottom  by  the  approximation  of  its  walls,  or  else  to 
remove  the  whole  of  the  cyst  by  dissecting  it  out  from  the  brain.  The 
latter  is  the  best  method  when  the  cyst  is  on  the  surface,  but  is  a 
dangerous  process,  and  is  sure  to  be  followed  by  the  formation  of  scar 
tissue,  which  in  itself  is  a  constant  irritant  to  the  brain.  Drainage  is 
necessary  in  deeper  lying  cysts.  If,  however,  the  cyst  is  a  part  of  a 
glioma  the  removal  of  its  wall  and  adjacent  tissue  affords  less  hope. 

Angioma,  if  lying  on  the  cortex  and  if  made  up,  as  is  usual,  of 
vessels  from  the  pia,  can  be  easily  removed  if  care  be  taken  to  ligate 
all  the  vessels  entering  the  tumor  before  any  are  cut.  But  such  liga- 
tion may  be  followed,  as  in  one  patient  of  my  own,  by  extensive 
softening  of  the  area  the  veins  of  which  were  tied. 

Gumma,  if  hard,  is  easily  taken  out,  but  if  soft  and  infiltrating  the 
pia  is  more  difficult  of  removal.  The  hai^d  type  requires  surgical 
'Starr,  Amer.  Jour.  Med,  Sci.,  April,  1910. 


612  TUMOBS  OF  THE  BRAIN. 

treatment ;  hence  it  should  be  removed.  The  soft  type  will  yield  to 
medical  treatment,  which  should  in  any  case  follow  the  operation  when 
a  gumma  is  found. 

The  question  of  operative  interference  when  a  tubercular  tumor  is 
diagnosticated  has  given  rise  to  some  discussion,  von  Bergman  n  being 
opposed  to  such  operations  and  Horsley  being  in  their  favor.  It  must 
be  admitted  that  a  permanent  cure  by  operation  is  less  likely  to  be 
achieved  in  the  case  of  a  tubercular  tumor  than  in  the  case  of  a  non- 
tubercular  tumor,  there  being  not  only  the  danger  of  recurrence  but 
also  the  possibility  of  the  existence  of  undetected  tumors  elsewhere  in 
the  brain  and  the  danger  of  the  development  of  tubercular  meningitis 
subsequently  to  the  operation.  An  operation  is  capable  of  prolonging 
life,  and  hence  should  not  be  wholly  condemned. 

The  prospect  of  recovery  when  an  endothelioma  or  a  glioma  is  found 
is  not  good,  for  it  is  rarely  possible  to  remove  these  tumors  completely, 
as  they  infiltrate  the  brain,  and  even  if  removed  they  are  liable  to  recur. 
Carcinoma  is  particularly  unfavorable  because  it  is  usually  secondary 
to  carcinoma  elsewhere. 

The  degree  of  ultimate  recovery  after  such  operations  will  depend 
upon  the  amount  of  destruction  of  brain  tissue  produced  by  the  tumor 
or  by  the  necessary  incisions  of  the  operator.  Sometimes  this  is  con- 
siderable, and  the  patients  are  left  incapacitated  for  life.  In  many 
cases,  however,  of  which  I  can  record  several,  a  complete  recovery  has 
occurred,  even  when  extensive  paralysis  had  preceded  the  operation. 
In  the  table  of  results  the  word  "  recovered  "  indicates  that  the  patient 
survived  the  operation  and  was  improved  by  it.  It  is  not  to  be  inter- 
preted as  implying  a  complete  restoration  to  health,  which  rarely  occurs. 
It  is  no  small  result  in  such  cases  to  save  a  patient's  life  and  to  arrest 
the  progress  of  a  fatal  disease. 

The  surgical  details  of  the  operation  do  not  require  description,  being 
beyond  the  scope  of  the  physician.^  They  are  many  and  complex. 
The  operation  is  not  to  be  undertaken  lightly  or  without  experience, 
and  it  is  better  for  the  physician  to  refer  such  cases  to  some  surgeon 
who  is  familiar  with  the  methods  of  procedure. 

When  an  operation  for  a  brain  tumor  is  advised  it  should  be  explained 
that  it  is  an  exploratory  operation,  that  it  may  save  life,  but  that  it  may 
leave  disabilities  which  will  be  permanent ;  that  it  may  fail  because  of 
the  impossibility  of  removal  of  the  tumor,  either  because  of  its  deep 
position  or  of  its  nature.  At  the  same  time  it  should  be  urged  as 
affording  a  possible  cure  for  an  otherwise  fatal  disease. 

In  cases  where  it  is  evident  that  a  brain  tumor  is  present,  is  causing 
urgent  symptoms,  especially  blindness,  and  yet  in  which  the  tumor 
cannot  be  located,  Horsley,  Harvey  Gushing  and  others  advise  a  de- 
compressing operation  ;  that  is,  a  large  opening  in  the  skull,  usually 
over  the  temporo-parietal  region,  the  dura  being  opened  and  cerebro- 

1  See  Brain  Surgery,  by  M.  Allen  Starr,  M.D.,  Wm.  Wood  &  Co.,  1893.  Also,  Brain 
Surgery,  by  Harvey  Gushing,  in  Keen's  Surgery,  vol.  iii.  Also,  Krause,  Chirurgie 
des  Gehtrns,  Berlin,  1908, 


TBEATMENT.  613 

spinal  fluid  being  allowed  to  drain  out  into  the  dressings.  It  is  cer- 
tain that  such  an  operation  relieves  the  general  symptoms  and  restores 
the  sight  if  there  is  no  optic  atrophy.  In  a  case  of  my  own,  operated 
upon  by  Harvey  Gushing/  a  choked  disk  causing  blindness  was  relieved 
in  a  week  and  vision  returned.  I  can  report  two  similar  cases.  The 
course  of  the  disease  is  in  these  cases  prolonged  and  the  patients  may 
be  more  comfortable.  In  a  few  cases  subsequently  to  the  operation  a 
tumor  has  appeared  in  the  opening  and  has  been  removed  at  a  second 
operation.  The  hernia  cerebri  which  tends  to  form  after  the  operation 
may  attain  a  large  size ;  the  discharge  of  cerebro-spinal  fluid  may  be 
enormous  (60  oz.  daily  for  two  weeks  in  one  of  my  cases)  and  there  is 
consequently  great  danger  of  infection.  If  this  is  avoided,  however, 
the  hernia  may  gradually  decrease  until  after  three  months  it  has  dis- 
appeared ;  the  flow  of  fluid  may  cease  ;  and  the  scalp  may  heal,  leaving 
only  the  bone  defect.  This  has  been  the  result  in  two  of  my  cases, 
with  gradual  subsidence  of  the  symptoms.  Whether  the  relief  of 
pressure,  or  the  discharge  of  fluid  arrested  the  growth  of  the  tumor,  or 
led  to  its  absorption,  must  be  undecided.  Others  report  similar  results. 
If  the  operation  does  not  cure  it  at  least  relieves  some  symptoms  and 
rarely  hastens  the  fatal  end.  Hence  in  every  case  of  brain  tumor  it 
seems  a  duty  to  resort  to  a  decompressive  operation.  Gushing  prefers 
to  open  the  skull  under  the  temporal  muscle  for  cases  of  cerebral  tumor 
and  under  the  occipital  muscles  for  cerebellar  tumor,  the  muscles  being 
split  and  then  united  so  as  to  prevent  too  great  protrusion. 

In  inaccessible  tumors  with  hydrocephalus  puncture  of  the  lateral 
ventricles  through  the  corpus  callosum  has  been  done,  an  opening  a 
quarter  of  an  inch  in  diameter  being  made,  and  left  open  permanently  ; 
so  that  drainage  of  the  ventricle  into  the  membranes  is  obtained.  In 
some  cases  this  has  relieved  the  symptoms  for  a  time.  In  other  cases 
the  relief  has  been  very  temporary. 

^Amer.  Jour.  Med.  Sci.,  April,  1910. 


CHAPTER  XXXIV. 

BULBAR   PARALYSIS. 
THE   CEREBRAL  AXIS. 

The  cerebral  axis  consists  of  the  crura  cerebri,  pons  Varolii,  and 
medulla  oblongata,  which  lie  upon  the  base  of  the  skull.  (See  Fig. 
78,  page  149.)  It  contains  the  nuclei  of  origin  of  all  the  cranial  nerves 
except  the  first  and  second.  These  are  arranged  in  groups,  lying  upon 
the  floor  of  the  fourth  ventricle  or  near  to  it.  It  also  contains  the  great 
motor  and  sensory  tracts  connecting  the  cerebrum  and  cerebellum  with 
each  other  and  with  the  spinal  cord.  Fig.  256  shows  a  lateral  view 
of  the  cerebral  axis  and  Fig.  77  (page  148)  shows  a  transparent  dia- 
gram which  indicates  the  position  and  relative  arrangement  of  the  nuclei 
within  it.  Figs.  52  to  57  (pages  111  to  117)  show  the  various  tracts 
passing  through  the  cerebral  axis. 

From  a  study  of  these  diagrams  and  from  a  consideration  of  the 
symptoms  of  lesions  in  the  cerebral  axis  already  discussed,  pages  110 
and  115,  it  is  evident  that  gross  lesions  such  as  hemorrhages,  small 
areas  of  softening  due  to  thrombi  or  emboli,  plaques  of  sclerosis,  and 
tumors  of  the  cerebral  axis,  can  be  easily  located  by  their  effects  upon 
the  cranial  nerves  and  upon  the  sensory  and  motor  tracts.  The  char- 
acteristic features  of  such  lesions  are  alternating  paralysis  or  anaesthesia 
when  the  lesion  is  unilateral,  and  paraplegia  with  cranial  nerve  symp- 
toms when  it  is  bilateral.  The  small  size  of  the  cerebral  axis  and  the 
importance  of  the  tenth  nerve  centres  to  life  make  it  evident  that  lesions 
which  are  larger  than  half  an  inch  in  diameter  are  usually  immediately 
fatal.  If,  however,  a  lesion  spares  the  tenth  nerve  nuclei,  the  patient 
may  live,  and  then  a  diagnosis  of  bulbar  lesion  is  not  difficult.  The 
prognosis  is,  however,  more  serious  than  in  tumor,  hemorrhage,  or 
softening  elsewhere  in  the  brain,  as  an  extension  of  the  lesion  may  end 
life  at  any  time,  and  regeneration  in  these  nuclei  and  tracts  does  not 
occur. 

BULBAR  PARALYSIS. 

Any  affection  which  involves  the  cerebral  axis  may  be  termed  bul- 
bar paralysis.  Thus  cases  of  hemorrhage,  which  are  rare,  or  of  soften- 
ing, which  are  common  in  the  crura,  pons,  or  medulla,  may  be  described 
"  as  acute  apoplectiform  bulbar  paralysis."  But  since  such  cases  differ 
from  cases  of  cerebral  apoplexy  in  no  respect  excepting  in  the  peculiar 
combination  of  their  symptoms,  due  to  the  location  of  the  lesion,  it 
seems  unwise  to  establish  a  special  category  of  diseases  for  these  cases. 

614 


StJLBAn  PARALYSIS. 


615 


The  facts  already  stated  in  tegard  to  the  pathology  of  apoplexy  (page 
473),  and  m  regard  to  its  symptoms,  and  especially  those  produced  by 
lesions  in  this  region  (page  494)  apply  to  this  condition  and  make  it 


Fig.  256. 


The  cerebral  axis.  The  cranial  nerves  and  their  nuclei  are  numbered  III.  to  XII.  BrcJ,  superior 
peduncle  of  cerebellum ;  Cm,  corpus  mammillare  ;  Cop,  posterior  commissure ;  DB,  decussation  of 
superior  peduncle  ;  DLm,  sensory  decussation  in  medulla  ;  DPi/,  motor  decussation  in  medulla  ;  BV, 
crossed  bundle  of  V.  nerve  ;  Fai,  internal  arcuate  fibres  ;  Fcl,  fornix  ;  Fng,  funiculus  gracilis  ;  Flp, 
posterior  longitudinal  bundle  ;  Frtf,  fasciculus  retroflexus  ;  If,  infundibulum  ;  Leo,  locus  caeruleus ; 
I/m,  lemniscus ;  JVa,  anterior  nucleus  of  thalamus ;  Nai;  nucleus  arcuatus  ;  Ng,  nucleus  gracilis  ; 
iVo,  olive  ;  Mg,  nucleus  tegmenti ;  2f,  red  nucleus  ;  Oaa,  ventral  olivary  nucleus  ;  Oae,  dorsal  olivary 
nucleus  ;  Po,  pons  ;  Pos,  superficial  transverse  fibres  of  pons  ;  Pp,  pes  pedunculi ;  Pp,  pyramid  ;  Qa, 
Qp,  corpora  quadrigemina ;  Sn,  substantia  nigra ;  Sim,  striae  acusticse ;  Stz,  stratum  zonale  of  the 
thalamus  ;  Tho,  thalamus  ;  Ust,  lower  thalamic  bundle  ;  Va,  Vicq  d'Azyr's  bundle  ;  Vg  anterior  column; 
Vma,  valve  of  Vieussens.     (Obersteiner.) 


Section  through  the  pons  Varolii,  showing  multiple  hemorrhages  which  caused  death. 
(Comte,  Des  Paralysies  Pseudo-bulbaires,  Paris,  1900.) 

unnecessary  to  do   more  than  mention  an  affection  which,  though  ex- 
tremely rare,  has  received  some  attention  in  recent  text-books.     Fig 


616  bVLbab  p analysis. 

257  shows  a  diagram  of  such  a  lesion,  a  number  of  small  hemorrhages 
in  the  pons  Varolii  having  caused  the  death  of  the  patient.  Acute 
bulbar  paralysis  has  been  already  described  in  the  chapter  upon  ence- 
phalitis ;  it  being  also  known  as  polio-encephalitis  inferior.  (See 
page  538.) 

By  bulbar  paralysis  a  disease  of  a  chronic  progressive  type  is  usually 
indicated,  also  called  glosso-labio-laryngeal  paralysis.  This  disease  is 
exactly  homologous  to  chronic  anterior  poliomyelitis,  the  lesion  being 
in  the  cerebral  axis  and  not  in  the  spinal  cord.  It  is  a  slowly  advanc- 
ing degeneration  in  the  nuclei  of  origin  of  the  motor  cranial  nerves,  one 
after  another  being  involved,  usually  from  below  upward,  but  occa- 
sionally from  above  downward.  In  some  cases  it  is  a  part  of  a  more 
widespread  aifection,  viz.,  amyotrophic  lateral  sclerosis,  and  many  cases 
which  present  the  typical  features  of  bulbar  palsy,  if  they  survive, 
develop  the  additional  symptoms  which  characterize  that  disease.  (See 
page  294.)  But  in  many  cases  the  bulbar  symptoms  are  the  only  ones 
to  present  themselves,  and  produce  a  very  typical  picture  which  is 
easily  recognized. 

Etiology.  —  The  etiology  of  bulbar  paralysis  is  obscure.  In  a  few 
eases  it  appears  to  have  been  a  congenital  aifection,  and  as,  in  these  it 
has  occurred  in  two  or  more  children  of  a  family  whose  parents  were 
related  to  one  another  or  in  children  who  showed  signs  of  degenera- 
tion, it  has  been  considered  an  hereditary  or  a  family  disease  and  due 
to  defective  development  of  the  brain.  In  one  such  case  hereditary 
syphilis  was  the  manifest  cause.  The  majority  of  the  cases  develop 
between  the  thirtieth  and  fiftieth  years  of  life,  though  no  age  is  exempt. 
Falls  and  blows  on  the  head,  emotional  strain,  and  cold  have  been 
thought  to  be  causes  of  the  disease.  Overwork  of  the  facial  muscles, 
such  as  occurs  in  players  on  wind  instruments,  has  been  thought  to  be 
a  cause. 

Pathology.  —  The  pathology  is  a  progressive  atrophy  of  the  motor 
neurones  in  the  hypoglossal,  glossopharyngeal,  spinal  accessorv,  pneu- 
mogastric,  facial,  and  motor  part  of  the  trigeminal  nerves  and  occasion- 
ally of  the  abducens  and  oculomotor  nuclei  also.  The  bodies  of  the 
neurones  undergo  a  progressive  atrophy,  lose  their  branches,  both  den- 
drites and  axones,  become  smaller,  and  finally  are  reduced  to  small  nuclei 
or  disappear  wholly.  (See  Plate  III.,  page  34.)  The  axones  degenerate 
in  the  same  manner  and  leave  an  empty  sheath.  The  muscles  to  which 
they  go  atrophy.  The  pathology  of  the  disease  is  therefore  identical 
with  that  of  chronic  anterior  poliomyelitis  {q.  v.).  This  lesion  may  be 
limited  to  the  gray  nuclei  of  the  cerebral  axis.  But  in  some  cases 
sclerosis  of  the  pyramidal  (motor)  tracts  in  the  medulla  and  pons  has 
been  found,  and  this  in  a  few  cases  could  be  traced  into  the  lateral  and 
anterior  median  columns  of  the  spinal  cord.  In  such  cases  it  is  evi- 
dent that  the  disease  was  amyotrophic  lateral  sclerosis  and  that  the 
bulbar  paralysis  was  only  the  first  stage  of  that  affection. 

Symptoms.  —  The  symptoms  of  bulbar  paralysis  usually  begin  in 
the  tongue,  causing  a  slight  defect  in  articulation,  words  which  con- 


PATHOLOGY.  617 

tain  the  Unguals,  d,  1,  n,  r,  s,  t,  being  badly  pronounced.  As  the 
disease  go^s  on  this  difficulty  of  speech  is  increased  by  the  develop- 
ment of  paralysis  in  the  pharynx,  which  makes  the  speech  nasal,  and 
of  paralysis  of  the  lips,  which  prevents  the  enunciation  of  b,  p,  v,  w,  y, 
and  finally  it  is  almost  impossible  to  understand  these  patients,  the 
vowel  sounds  alone  being  clearly  produced.  When  the  larynx  itself 
becomes  paralyzed  even  phonation  is  indistinct.  This  symptom  has 
been  termed  dysarthria  to  distinguish  it  from  aphasia.  The  paralysis 
of  the  tongue  is  attended  by  fibrillary  twitching  and  by  atrophy, 
which  throws  the  mucous  membrane  into  folds.  A  reaction  of  de- 
generation may  be  found  in  the  muscles  of  the  tongue. 

While  the  symptoms  are  developing  disturbance  in  the  act  of  chew- 
ing and  of  swallowing  appears.  The  food  cannot  be  rolled  about  in 
the  mouth  on  account  of  the  paralysis  of  the  tongue  and  cannot  be 
carried  backward  to  the  pharynx.  The  pharyngeal  muscles  are 
involved,  and  hence  their  automatic  action  is  imperfect ;  hence  the  food 
is  not  grasped  and  propelled  into  the  oesophagus,  but  may  stick  in  the 
throat  and  get  into  the  larynx.  The  automatic  closure  of  the  epiglottis 
on  arrival  of  food  near  it  is  also  impaired  by  paralysis  of  the  laryngeal 
muscles ;  hence  fluids  trickle  into  the  larynx  and  fits  of  choking 
become  frequent.  These  patients  sometimes  attempt  to  aid  themselves 
by  pushing  the  food  back  into  the  pharynx  with  the  finger  or  by  lying 
down  when  they  attempt  to  swallow  fluids.  The  paralysis  of  the 
pharynx  and  uvula  may  lead  to  regurgitation  of  fluids  through  the 
nose.  When  the  paralysis  reaches  the  muscles  of  mastication  chewing 
is  impossible,  and  semisolid  food  only  can  be  taken.  It  is  probably 
one  reason  for  the  rapid  emaciation  of  these  patients  that  the  difficulty 
of  eating  deprives  them  of  a  proper  amount  of  nourishment.  The 
difficulty  in  deglutition  forms  one  of  the  chief  dangers  of  the  disease, 
for  foreign  bodies  get  into  the  larynx  and  lungs  and  choke  the  patients 
or  set  up  pneumonia,  which  is  fatal.  For  this  reason  feeding  by  the 
stomach-tube  should  be  begun  early,  and  the  patient  should  be  in- 
structed how  to  pass  the  tube,  and  thus  should  avoid  all  acts  of 
swallowing. 

A  further  set  of  symptoms  is  referable  to  the  facial  muscles.  The 
lower  muscles  become  weak  and  atrophy,  causing  a  lack  of  facial 
expression  and  a  flattening  of  the  face  from  obliteration  of  its  folds. 
The  muscles  about  the  mouth  are  relaxed  and  the  mouth  cannot  be 
firmly  closed,  and  the  lower  lip  hangs  down.  Hence  the  saliva  runs 
out,  and  the  patient  holds  a  handkerchief  to  catch  the  drooling.  He 
can  no  longer  whistle  or  blow.  The  jaw-jerk  may  be  increased.  The 
muscles  closing  the  eye  are  very  rarely  paralyzed.  The  lower  muscles 
of  the  face  and  of  the  tongue  are  in  a  state  of  constant  fibrillary  con- 
traction causing  a  fine  tremulous,  wavy  movement  of  the  skin  and 
mucous  membrane.  As  the  facial  muscles  atrophy  a  partial  reaction 
of  degeneration  can  be  obtained.  The  paralysis  of  the  muscles  of 
mastication  leads  to  an  inability  to  close  the  jaw,  which  makes  it 
necessary  for  the  patient  to  support  it  by  the  hand.     The  tongue  in  its 


618 


BULBAR  PAEALYSIS. 


final  state  of  paralysis  lies  on  the  floor  of  the  mouth,  cannot  be  put 
out  or  moved  about,  the  soft  j)alate  hangs  motionless  and  does  not 
rise  in  speaking  or  in  swallowing ;  hence  fluids  return  through  the 
nose.  In  some  cases  the  oculomotor  nuclei  become  affected  and  various 
forms  of  strabismus  and  double  ptosis  develop.  Thus  in  the  patients 
shown  in  Figs.  258  and  259  there  was  not  only  a  paralysis  of  the 
facial  muscles  and  of  the  tongue,  but  double  ptosis  with  paralysis  of  all 
the  muscles  of  the  eyeballs. 


Fig  259. 


Patient  suflfering  from  bulbar  paralysis.  Double 
ptosis,  slight  external  strabismus,  facial  paralysis 
with  lack  of  facial  expression  and  inability  to 
close  the  lips  are  to  be  seen.  The  effort  to  open 
the  eyes  causes  a  wrinkling  of  the  forehead. 


Bulbar  paralysis.  Double  ptosis,  with  ex- 
ternal strabismus,  flattening  of  the  face, 
inability  to  close  the  mouth,  and  atrophy  of 
the  right  half  of  the  tongue  are  to  be  seen. 
(Icon,  de  la  Salpetriere.) 


Finally,  the  respiratory  movements  of  the  larynx  are  interfered 
with,  a  peculiar  stridor  is  heard  in  respiration,  the  breathing  becomes 
rapid,  and  death  by  asphyxiation  occurs.  In  the  early  stage  a  laryn- 
goscopic  examination  shows  weakness  of  the  adductors,  which  later  on 
becomes  a  total  paralysis.  In  a  few  cases  the  oculomotor  nerves  and 
the  abducens  have  been  paralyzed,  causing  ptosis,  and  strabismus  with 
all  the  characteristics  of  ophthalmoplegia  (q.  v.). 

There  is  never  any  pain  or  anaesthesia  of  the  face,  but  headache  in 
the  occiput  or  pain  in  the  nape  of  the  neck  is  sometimes  complained 
of.     Sometimes  an  increase  of  knee-jerks  is  present. 

A  very  constant  symptom  of  bulbar  paralysis  is  an  emotional 
state.  The  patients  cry  on  very  slight  provocation,  and  seem  to 
lack  a  normal  control  of  their  emotions  and  are  extremely  depressed 
at  their  condition.  In  a  few  cases  symptoms  of  spastic  paraplegia 
appear  and  the  disease  goes  on  to  amyotrophic  lateral  sclerosis.  In 
other  cases  an  atrophic  paralysis  develops  in  the  hands  and  arms,  and 
it  is  evident  that  a  chronic  anterior  poliomyelitis  has  followed  the 


DIAGNOSIS.  619 

bulbar  palsY.  It  has  already  been  stated  that  in  both  of  these  diseases 
bulbar  paralysis  may  develop  as  a  final  result.  These  facts  point  to  a 
common  origin  and  pathology  in  all  three  affections,  which  may,  there- 
fore, be  regarded  as  stages  or  varieties  of  onset  in  one  disease. 

The  course  of  bulbar  paralysis  is  slowly  progressive.  In  a  few  cases 
there  may  be  for  a  time  an  arrest  of  symptoms,  but  they  soon  reappear, 
and  the  result  is  uniformly  fatal.  The  average  duration  of  the  disease 
is  two  years,  but  I  have  seen  several  rapid  cases  which  terminated 
within  a  year  of  the  onset.  Death  is  due  to  choking,  to  pneumonia  or 
bronchitis  set  up  by  inhaling  food,  or  to  starvation. 

Diagnosis.  —  The  diagnosis  of  bulbar  paralysis  presents  no  difficulty. 
It  is  to  be  remembered,  however,  that  similar  symptoms  are  caused  by 
tumors  of  the  cerebral  axis,  though  these  are  rarely  so  located  as  to 
produce  bilateral  symmetrical  symptoms.  I  have  seen  one  case,  how- 
ever, in  which  the  entire  absence  of  general  symptoms  of  brain  tumor 
and  the  slowly  progressive  development  of  bulbar  paralysis  led  to- this 
error,  which  was  only  revealed  at  the  autopsy.  The  history  of  the  slow 
progress  of  the  symptoms  will  serve  to  distinguish  bulbar  paralysis 
from  vascular  lesions  in  the  cerebral  axis.  But  cases  of  aneurism  of 
the  basilar  artery  sometimes  present  very  similar  symptoms,  though  in 
the  few  cases  thus  far  recorded  spastic  paraplegia  has  appeared  early. 

The  occurrence  of  spinal  and  cortical  symptoms  will  distinguish 
multiple  sclerosis  from  this  disease.  In  the  course  of  multiple  neuritis 
either  of  diphtheritic  or  of  toxic  origin  cranial  nerve  symptoms  some- 
what like  those  of  bulbar  palsy  may  occur.  But  the  history  of  the 
case,  its  rapid  progress,  the  occurrence  of  other  symptoms  referable  to 
the  nerves  of  the  extremities,  and  the  rapid  recovery  leave  no  doubt 
as  to  the  diagnosis  from  bulbar  paralysis. 

There  is  a  condition  quite  like  that  of  bulbar  paralysis  which  is  pro- 
duced by  bilateral  lesions  in  the  facial  motor  area  of  the  cerebral  cor- 
tex lying  in  the  operculum  or  in  the  motor  tracts  from  those  areas  to 
the  nuclei  of  the  cranial  nerves,  especially  in  the  internal  capsule  near 
the  lenticular  nucleus  and  in  the  lenticular  loop.  This  condition  is 
naturally  a  very  rare  one,  but  it  has  been  observed.  It  has  been 
named  pseudo-bulbar  paralysis.  It  may  be  due  to  a  congenital  defect 
with  porencephalus,  and  then  the  symptoms  appear  in  infancy.  It 
may  be  due  to  multiple  sclerosis  or  to  several  separate  attacks  of  an 
apoplectic  nature,  each  causing  unilateral  symptoms,  but  the  final  result 
being  a  symptom-complex  resembling  bulbar  palsy.  The  history  of 
the  development  of  the  symptoms  should  prevent  any  mistake  of  diag- 
nosis. And  in  these  cases  the  paralysis  is  not  accompanied  by  rapidly 
advancing  atrophy,  fibrillary  tremor,  and  reaction  of  degeneration, 
which  can  be  demonstrated  in  cases  of  bulbar  paralysis.  There  is 
usually  a  weakening  of  the  mind,  a  dementia,  and  not  uncommonly  some 
form  of  aphasia.  And  in  these  cases  evidences  of  arterial  disease  aid 
the  diagnosis  as  well  as  the  non-progressive  course  of  the  symptoms. 

Finally,  it  is  necessary  to  differentiate  bulbar  paralysis  from  as- 
thenic paralysis  or  myasthenia  gravis.     The  symptoms  in  myasthenia 


620  BULB  AM  PAEALYSIS. 

gravis  are  much  like  those  of  bulbar  paralysis,  though  ptosis,  paralysis 
of  the  orbicularis  palpebrarum,  and  weakness  of  the  muscles  of  the  neck, 
which  are  very  rare  in  bulbar  palsy,  appear  early  in  myasthenia, 
Furthermore,  in  myasthenia  a  great  general  muscular  lassitude  and 
undue  fatigue  on  exertion  is  quite  constant,  so  that  a  few  movements 
exhaust  the  patient  completely,  rendering  him  incapable  of  walking  or 
of  using  his  hands.  The  rapid  onset  of  this  general  muscular  weak- 
ness is  sufficient  to  differentiate  the  disease  even  from  cases  of  amy- 
otrophic lateral  sclerosis  beginning  with  bulbar  symptoms.     The  elec- 

FiG    260. 


C.T. 


Section  of  muscle  showing  lymphoid  infiltrations.     The  letters  C.T.  indicate  a  strand  of  con- 
nective tissue  surrounded  by  the  infiltrating  cells.     (Hun.) 

trical  excitability  of  the  muscles  diminishes  progressively  as  the  current 
is  applied,  until  no  contraction  can  be  produced,  yet  after  a  period  of 
rest  it  is  found  to  have  returned.  This  peculiarity,  termed  electrical 
fatigue  of  a  muscle,  is  characteristic  of  the  disease,  as  shown  by  Jolly 
and  Murri.  The  muscles  which  are  affected  show  no  fibrillary  tremor, 
do  not  atrophy  and  show  no  reaction  of  degeneration.  There  are  no 
sensory  symptoms.  The  course  of  the  disease  differs  from  that  in 
bulbar  palsy,  for  while  the  onset  may  be  similarly  slow  and  the  various 
symptoms  may  all  be  alike  in  bulbar  and  asthenic  paralysis,  the  disease 
is  not  steadily  progressive.  Periods  of  improvement  or  even  entire 
subsidence  of  the  symptoms  occur.  The  patient  may  recover  and  be 
free  from  all  signs  of  disease  for  some  months,  though  the  recovery  is 
never  permanent,  as  almost  all  cases  die  within  a  few  years.  And  if 
the  symptoms  prove  fatal  no  lesion  in  the  nervous  system  has  been 
found.  The  only  constant  lesion  in  myasthenia  gravis  is  an  extensive 
infiltration  of  the  muscles,  and  of  all  of  the  organs  of  the  body,  by 
small  lymphoid  cells  (shown  in  Fig.  260).  The  nature  of  the  disease 
is  discussed  on  page  768. 


TREATMENT.  621 

Prognosis.  —  The  prognosis  in  bulbar  paralysis  is  very  serious. 
The  patients  die  within  three  years  of  the  onset  of  the  disease,  and  some 
cases  run  a  more  rapid  course.  The  length  of  life  may  be  prolonged 
by  care  in  feeding  these  patients. 

Treatment.  —  The  treatment  should  consist  in  keeping  up  the  nutri- 
tion and  general  health  of  the  patient  by  every  possible  means.  Good 
food,  massage,  life  in  the  open  air,  stimulating  water  treatment  by 
warm  packs  or  hot  baths,  followed  by  cool  showers  or  douches,  and  all 
forms  of  tonics  should  be  advised.  Strychnine,  arsenic,  and  phos- 
phorus in  the  form  of  hypophosphites  and  the  glycero-phosphates 
should  be  employed  freely  and  kept  up,  each  drug  being  used  a  short 
time,  and  those  selected  and  continued  which  seem  to  agree  best.  In 
the  early  stage  the  patient  should  be  carefully  instructed  in  the  passage 
of  the  stomach-tube,  and  this  must  be  insisted  upon,  as  later  in  the 
disease,  when  difficulty  in  swallowing  begins,  the  chief  danger  can  be 
avoided  by  its  use.  When  solids  can  no  longer  be  swallowed  semi- 
solid food  should  be  given  and  supplemented  by  eggs  and  milk  and 
broths  given  through  the  tube.  When  nothing  can  be  voluntarily  swal- 
lowed all  food  has  to  be  given  in  this  way.  I  have  known  a  patient 
to  be  kept  alive  over  a  year  by  this  method  of  feeding.  The  thirst  can 
be  reduced  by  large  enemata  of  salt  solution  and  by  following  each 
feeding  by  giving  water  through  the  tube.  In  the  last  stages  of  the 
disease  hypodermic  medication,  with  morphine  to  allay  the  mental  dis- 
tress, with  atropia  to  prevent  the  undue  flow  of  saliva,  and  with 
strychnine  to  support  the  vital  centres  may  be  employed. 


CHAPTER  XXXV. 

THE   CRANIAL   NERVES   AND   THEIR   DISEASES. 

The  number,  name,  and  function  of  the  cranial  nerves  are  as  follows : 

First.     Olfactory,  sense  of  smell. 

Second.     Optic,  sense  of  sight. 

Third.     Oculomotor,  motion  of  the  eyeball  up,  down,  and  in. 

Fourth.     Patheticus,  motion  of  the  eyeball  in  and  up. 

Fifth.  Trigeminal,  sensory  nerve  of  the  face ;  probably  nerve  of 
taste ;  and  motor  nerve  of  the  muscles  of  mastication. 

Sixth.     Abducens,  motion  of  the  eyeball  outward. 

Seventh.     Facial,  motor  nerve  of  the  face. 

Eighth.  Acoustic ;  cochlear  division,  nerve  of  hearing  ;  vestibular 
division,  nerve  of  equilibrium. 

Ninth.  Glossopharyngeal,  nerve  of  the  throat,  sensory  and  motor ; 
possibly  of  taste. 

Tenth.     Pneumogastric  or  vagus  nerve,  larynx,  and  vital  organs. 

Eleventh.     Spinal  accessory,  nerve  of  the  larynx  and  neck. 

Twelfth.     Hypoglossal,  nerve  of  the  tongue. 

Each  of  these  nerves  has  a  peripheral  course  from  the  surface  of  the 
head,  from  some  organ  of  sense,  or  from  some  group  of  muscles  to  its 
primary  nerve  centre  lying  upon  the  base  of  the  brain.  Plate  XXV. 
shows  the  course  and  the  distribution  of  the  superficial  nerves  of  the 
head  and  face.  The  deeper  origin  of  the  cranial  nerves  is  shown  in 
Figs.  53  to  57,  77,  78,  256,  and  261. 

The  primary  centres  or  cranial  nerve  nuclei  are  closely  joined  to  one 
another  by  association  fibres,  and  their  interaction  is  necessary  in  many 
reflex  acts,  such  as  the  sniffing  of  an  odor  or  a  sneeze,  the  turning  of 
the  eyes  and  head  toward  an  object  seen,  the  winking  caused  by  dust 
in  the  eye ;  the  motions  of  the  face  in  smiling,  frowning,  or  crying ; 
the  pose  of  the  head  in  listening,  or  quick  turning  toward  a  sound,  or 
a  start  with  winking  at  a  noise ;  the  involuntary  swallowing  of  saliva 
or  of  tasting  food,  and  the  acts  of  respiration  and  coughing. 

Each  cranial  nerve  is  also  connected  with  the  higher  centres  of  the 
brain.  Some  are  joined  directly  to  the  brain  cortex.  The  majority 
have  an  indirect  connection,  the  tract  from  the  primary  centre  to  the 
cortex  being  interrupted  in  the  basal  ganglia.  This  interruption  is 
for  thie  purpose  of  providing  for  the  numerous  unconscious  and  invol- 
untary automatic  acts  of  high  complexity  which  involve  the  coordi- 
nated action  of  numerous  widely  separated  centres. 

In  studying  the  cranial  nerves  it  is  evident,  then,  that  a  sharp  dis- 
tinction must  be  made  between  diseases  in  the  peripheral  part  of  th^ 

622 


PLATE  XXV 


y^. 


Showing  the  distribution  of  the  fifth,  seventh  and  eleventh  cranial  nerves  and  the 
cervical  and  brachial  plexuses  ;  also  the  area  of  the  middle  meningeal  artery  in  the  inner 
table  of  the  skull,  injury  to  which  is  sometimes  the  cause  of  hemiplegia;  also  the  course 
of  the  bloodvessels  in  the  neck  and  face.     (Arnold's  Atlas.) 


TKE   CRANIAL    NERVES. 


623 


nerve,  diseases  in  the  primary  centres  of  the  nerve,  diseases  in  the  intra- 
cerebral tracts  of  the  nerve,  and  diseases  of  the  secondary  or  cortical 
centres  of  the  nerve.     In  this  section  the  diseases  of  the  peripheral 


Fig.  261. 


ti  Co. 


The  base  of  the  brain  and  the  crura,  pons,  and  medulla,  showing  the  superficial  origin  of  the  crania 
nerves.  /  to  XII,  the  cranial  nerves  ;  2%,  optic  thalamus  ;  h,  pituitary  body  ;  tc,  tuber  cinereum  ;  o, 
corpora  albicantia;  P,  pes  .pedunculi ;  i,  interior  and  e  exterior  geniculate  body  ;  PV,  pons  Varolii ; 
pa,  anterior  pyramid  of  medulla;  o,  olive;  d,  decussation  of  anterior  pyramids ;  ca,  anterior  column 
of  spinal  cord ;  cl,  lateral  column  of  spinal  cord ;  Ce,  cerebellum  ;  fl,  flocculus  of  cerebellum.  (Allen 
Thompson. ) 

part  and  of  the  primary  centres  of  the  cranial  nerves  are  considered. 
The  diseases  of  the  intracerebral  tracts  and  cortical  centres  have  been 
treated  under  brain  diseases,  pages  86  to  150. 


THE  FIRST  NERVE  :    THE  OLFACTORY  NERVE. 

The  structure  and  course  of  the  olfactory  nerve  to  the  olfactory  bulb 
and  from  the  bulb  to  the  brain  is  demonstrated  in  Figs.  68  and  262, 
The  primary  neurones  and  the  finer  filaments  of  the  nerve  endings  ar^ 


624 


THE   CEANIAL  NEBVES  AND   THEIB  DISEASES. 


situated  in  the  Schneiderian  membrane  on  the  upper  and  middle 
turbinated  bones  and  on  the  septum  of  the  nose,  all  of  which  parts 
are  pigmented.  It  is  in  the  olfactory  bulb  that  the  primary  sensory 
neurones  terminate  in  a  fine  brush  about  the  glomeruli  of  the  nerve. 
(Fig.  262.)  The  course  from  the  olfactory  bulb  to  the  brain  must  be 
regarded  as  an  intracerebral  tract.     The  mitral  cells  in  the  bulb  are 

Fig,  262. 


The  olfactory  bulb  and  tract.  A,  Schneiderian  membrane  in  nose  in  which  lies  peripheral  olfac- 
tory neurone ;  B,  glomerulus  of  olfactory  bulb ;  C,  mitral  cells  with  dendrites  in  B  and  axones  in  D 
olfactory  lobe  ;  E,  granule  cells  ;  F,  cells  in  olfactory  lobe  ;  H,  G,  fibres  of  olfactory  tract.  (Ramon  y 
Cajal. ) 

the  secondary  neurones  which  receive  impulses  from  their  dendrites 
about  the  glomeruli  and  send  impulses  along  their  axones  in  the  olfactory 
tract  to  the  cortex.  Fig.  68,  page  132,  shows  the  course  of  this  tract 
to  its  various  terminal  stations  in  the  optic  thalamus  and  in  the  cortex 
of  the  uncinate  gyrus.  The  thalamic  centre  is  joined  to  the  cortex  by 
the  fornix  and  fascia  dendata.  It  is  from  the  study  of  comparative 
anatomy  that  these  facts  are  established.  In  man  the  olfactory 
apparatus  has  been  reduced  to  an  almost  rudimentary  state. 

Any  disease  that  affects  the  terminal  filaments  of  the  nerve  in  the 
mucous  membrane  of  the  nose  will  suspend  the  sense  of  smell.  This 
symptom  is  termed  anosmia.  Acute  or  chronic  catarrhal  affections  of 
the  nasal  organs,  arrest  of  the  nasal  secretion,  which  occurs  when  the 
fifth  nerve  is  diseased ;  tumors  of  the  nose,  or  polypi ;  caries  of  the 
ethmoid  bones,  or  ethmoiditis,  or  diseases  which  interrupt  the  trans- 
mission of  impulses  to  the  olfactory  bulb  will  cause  anosmia.  Menin- 
gitis upon  the  base  of  the  brain,  fractures  of  the  base  of  the  skull, 
and  tumors  of  the  bone  or  of  the  brain  in  the  anterior  fossa  are  capable 
of  tearing  or  destroying  the  fine  filaments  of  the  olfactory  nerve  or  of 
separating  them  from  their  connection  with  the  bulb.  Any  disease 
which  destroys  or  compresses  the  olfactory  bulb  or  tract  or  its  terminal 
station  in  the  cortex  will  have  the  same  effect.  An  arrest  of  activity 
of  this  centre  occurs  in  hysteria. 

The  sense  of  smell  has  much  to  do  with  our  appreciation  of  taste, 
and  when  it  is  lost  it  is  impossible  for  the  patient  to  perceive  very 
delicate  differences  of  taste  in  various  substances.     This  may  be  the 


TEE   SECOND   NEBVE :    THE    OPTIC   NERVE.  625 

symptom  fipst  noticed  by  the  patient.  Loss  of  the  sense  of  smell  is  a 
symptom  which  is  not  often  perceived  spontaneously  as  long  as  the 
disease  is  unilateral ;  hence  it  has  to  be  sought  for  by  testing  the 
patient  with  various  fragrant  substances.  The  tests  are  best  made 
with  various  perfumed  powders  or  essential  oils,  for  it  is  to  be  remem- 
bered that  ammonia,  alcohol,  and  vinegar,  which  are  commonly  used 
for  this  purpose,  produce  irritation  of  the  fifth  nerve  as  well  as  of  the 
first,  and  should  not  be  used  as  tests.  The  symptom  is  of  interest 
chiefly  as  an  indication  of  disease  in  the  anterior  fossa  of  the  skull, 
such  as  tumor  or  abscess  of  the  brain.  A  primary  isolated  neuritis 
of  the  first  nerve  has  not  yet  been  recorded.  It  occurs  only  as  a 
result  of  nasal  disease.  Atrophy  of  the  olfactory  nerve  has  been  said 
to  occur  as  a  complication  of  tabes.  It  must  be  the  terminal  lesion  in 
any  destruction  of  the  nerve  fibres.  Irritation  of  the  olfactory  organ 
or  of  its  cortical  centres  may  cause  hallucinations  of  smell  (parosmia). 
This  is  seen  in  epilepsy  and  in  insanity. 

THE  SECOND  NERVE:  THE  OPTIC  NERVE. 

Anatomy.  —  For  the  finer  anatomy  and  physiology  of  the  optic 
apparatus  and  retina  the  reader  is  referred  to  the  text-books  on 
ophthalmology. 

The  anatomy  of  the  optic  nerve  and  its  connections  with  the  brain 
are  shown  in  Fig.  263. 

Many  primary  optic  neurones  are  situated  in  the  retina,  their 
dendrites  being  connected  with  the  rods  and  cones,  and  these  send 
their  axones  to  the  primary  optic  centres  in  the  thalamus,  corpora 
geniculata,  and  corpora  quadrigemina.  It  will  be  noticed  that  some 
cell  bodies  in  these  basal  ganglia  send  their  axones  outward  to  the 
retina,  where  they  terminate  in  the  rods.  Thus  the  optic  nerve  con- 
tains fibres  which  pass  in  both  directions.  The  optic  nerves  pass 
backward  from  the  eye  to  the  optic  chiasm,  where  a  partial  decussation 
of  the  fibres  occurs,  the  majority  crossing  over  to  the  other  side  to 
enter  the  opposite  optic  tract  and  the  minority  turning  directly  into  the 
optic  tract  of  the  same  side.  The  crossing  fibres  convey  impulses 
from  the  inner  or  nasal  half  of  the  retina,  and  hence  their  injury 
causes  blindness  in  the  outer  or  temporal  portion  of  the  visual  field, 
which  is  much  the  larger  portion.  Fibres  from  the  macula  lutea, 
which  convey  central  visual  impulses,  pass  from  each  eye  to  both 
optic  tracts ;  hence  central  or  direct  vision  is  never  affected  by  a  lesion 
in  the  optic  tract.  (See  Fig.  264.)  The  optic  tracts,  containing 
fibres  from  both  eyes,  curve  about  the  crura  cerebri  and  terminate  in 
the  primary  optic  centres,  as  shown  in  the  diagram.  A  lesion  of  one 
optic  nerve  causes  blindness  of  one  eye.  A  lesion  of  the  optic  chiasm 
causes  partial  or  total  blindness,  according  to  the  number  and  position 
of  the  optic  fibres  destroyed.  If  the  lesion  involves  one  lateral  part 
only  of  the  chiasm  (Fig.  264,  ft),  the  fibres  from  the  temporal  half  of 
the  retina,  are  involved,  and  a  unilateral  nasal  hemianopsia  is  caused, 
40 


626 


TBE   CRANIAL  NEEVES  AND   THEIR  DISEASES. 


that  is,  a  blindness  in  the  inner  portion  of  the  visual  field  of  one  eye. 
If  the  lesion  involves  the  anterior  or  posterior  part  only  of  the  chiasm, 
the  fibres  which  come  from  the  nasal  halves  of  the  retinae  are  involved, 


Fig.  263. 


Diagram  of  the  optic  nerves  and  tracts.  On  tlie  right  of  the  figure  the  masses  of  gray  matter  in  the 
basal  ganglia  are  shown.  Palv,  pulvinar  of  the  optic  thalamus  ;  Pn,  posterior  nucleus  of  the  optic  thal- 
amus lying  between  the  corpus  geniculatum  externum  cge,  and  the  corpus  geniculatum  internum  cgi  ; 
IC,  internal  capsule ;  F,  fillet ;  By,  red  nucleus  of  the  tegmentum  ;  FED,  peduncle  of  the  cerebrum ; 
Sn,  substantia  nigra  ;  OT,  optic  tract ;  JT,  optic  chiasm  ;  //,  optic  nerve  ;  1,  2,  fibres  from  the  retina  to 
the  pulvinar  of  the  optic  thalamus  ;  1,  centripetal ;  2,  centrifugal ;  7,  8,  fibres  between  the  optic  thal- 
amus and  the  occipital  cortex  ;  3,  4,  fibres  between  the  retina  and  the  corpus  geniculatum  externum ; 
9,  10,  corresponding  fibres  to  the  occipital  cortex  ;  5,  6,  fibres  between  the  retina  and  the  corpus  quad- 
rigeminum  anterior  ;  11,  12,  corresponding  fibres  to  the  occipital  cortex  —  for  the  sake  of  distinctness 
the  decussating  optic  fibres  only  are  traced  ;  13,  cell  of  the  superficial  gray  matter  of  the  CQA  passing 
to  the  nucleus  of  the  third  nerve  (16)  ;  14,  cell  of  the  deep  gray  of  CQA  passing  to  the  third  nerve 
nucleus  ;  15,  cell  of  the  deep  gray  of  CQA  sending  fibre  into  the  fillet ;  17,  fibre  from  the  red  nucleus, 
terminating  about  14 ;  18,  fibre  from  the  fillet,  terminating  about  13  ;  OL,  occipital  lobe  of  the  brain 
with  its  cortex,  containing  both  cells  and  terminal  brushes  of  the  visual  tract,  (Starr,  Atlas  of  Nerve 
Cells.) 


THE    SECOND   NEEVE:    THE    OPTIC   NEBVE.  627 

and  a  bilateral  temporal  hemianopsia  is  caused,  that  is,  a  blindness  in 
the  outer  portion  of  the  visual  field  of  both  eyes.  This  is  termed 
heteronymous  hemianopsia.  If  the  lesion  involves  the  optic  tract 
after  the  decussation  a  blindness  in  one-half  of  both  eyes  is  caused, 
and  the  patient  is  deprived  of  seeing  anything  to  one  side  of  the  middle 
line.     This  is  termed  homonymous  hemianopsia. 


The  relation  in  the  optic  nerves,  optic  chiasm,  and  optic  tracts  of  the  fibres  from  the  nasal  half  (/»), 
temporal  half  {ft),  and  central  spot  (/to)  of  the  retina  ;  Nil,  optic  nerve ;  £11,  optic  tract ;  xll,  optic 
decussation.  The  left  figure  shows  the  fibres  which  cross  ;  the  right  figure  shows  those  which  do  not 
cross;  P,  pupillary  fibres.     (Dejerine. ) 

In  none  of  these  forms  of  hemianopsia  is  central  vision  impaired.  It 
is  only  aifected  in  lesions  of  the  nerve  itself.  The  acuteness  of  vision 
is  best  tested  by  Snellen's  test  types,  type  No.  xx  as  seen  at  twenty 
feet  being  taken  as  indicating  normal  vision  ^. 

The  extent  of  the  visual  field  is  to  be  determined  for  each  eye  sepa- 
rately. This  is  to  be  done  by  having  the  patient  fix  each  eye  in  turn 
on  that  of  the  examiner,  the  other  eye  being  covered,  and  then  notice 
whether  he  can  see  the  hand  of  the  examiner  or  a  small  white  object 
held  by  him  as  it  is  passed  outward  from  the  centre  of  vision  on  all 
sides.  The  retina  is  less  sensitive  in  its  peripheral  parts,  and  the  less 
the  illumination  of  the  test  object  the  less  distinctly  is  it  seen.  The 
luminosity  of  blue,  red  and  green  are  less  than  white  in  the  order 
named  ;  hence  the  visual  field  is  greatest  for  white,  next  for  blue,  next 
for  red,  and  least  for  green. 

Contraction  of  the  visual  field  for  white  or  colors  as  well  as  defect 
in  the  visual  field  is  an  important  symptom. 

The  optic  nerve  contains  some  fibres  which  pass  to  the  motor 
mechanism  controlling  the  action  of  the  iris.  (Fig.  264,  P.)  Hence 
impressions  of  light  cause  a  narrowing  of  the  pupil.  If  one  optic 
nerve  only  is  destroyed  the  pupil  reflex  is  lost  in  that  eye  when  it  is 
exposed  to  light.  But  as  this  reflex  is  bilateral,  light  in  the  unaifected 
eye  will  cause  a  contraction  of  the  pupil  in  the  blind  eye.  The  fibres 
which  sul^serve  this  pupil  reflex  decussate  at  the  chiasm.     Hence  if 


628  TEE  CBANIAL  NESVES  AND   THEIS  DISEASES. 

one  optic  tract  be  injured,  its  function  suspended,  we  have  not  only  a 
bilateral  hemianopsia,  but  also  an  interference  with  the  pupil  reflex. 
This  is  difficult  to  determine,  for,  as  already  stated,  the  reflex  is  a 
bilateral  one,  and  light  thrown  on  the  unaffected  parts  of  one  of  the 
retinae  is  capable  of  causing  a  prompt  narrowing  of  both  pupils.  If, 
however,  the  patient  be  examined  in  a  dark  room,  the  eyes  be  only 
faintly  illuminated,  so  that  the  pupils  are  fairly  dilated,  and  then  a 
ray  of  light  be  thrown  by  a  mirror  from  one  side  carefully  upon  the 
blind  side  only  of  the  retina,  no  pupil  reflex  will  ensue.  This  indi- 
cates infallibly  a  lesion  of  the  optic  tract,  or  basal  ganglia,  as  it  does 
not  occur  in  cases  where  the  hemianopsia  is  due  to  lesion  elsewhere  in 
the  visual  tract,  as,  for  example,  in  the  occipital  lobe.  It  is  termed 
Wernicke's  test  for  a  hemiopic  pupillary  reflex. 

In  lesions  of  the  optic  tract  the  hemianopsia  affects  color  sense  as 
well  as  white  and  object  vision.  It  is  only  in  affections  of  the  visual 
centres  in  the  cortex  of  the  cerebral  hemisphere  that  hemianopsia  for 
color,  with  preservation  of  sight  for  white  and  objects,  has  been 
observed.     This  is  called  hemichromatopsia.     (See  page  122.) 

Diseases  of  the  Optic  Nerve. 

Hypersemia  and  anaemia  of  the  optic  nerve  accompany  correspond- 
ing conditions  of  the  brain,  but  unless  very  extreme,  are  not  easily 
recognized,  even  by  experts,  as  the  normal  tint  of  the  retina  and  the 
normal  calibre  of  the  bloodvessels  differ  in  different  individuals  within 
very  wide  limits,  like  the  color  of  the  hair  and  of  the  skin. 

Growers  states  that  a  state  of  hypersemia  may  be  admitted  when  the 
disk  is  dull  red  or  brick  color  and  when  the  edge  of  the  disk  cannot 
be  distinguished  from  the  rest  of  the  retina.  Such  a  condition  is 
usually  associated  with  a  hypersensitiveness  to  light  and  pain  on  using 
the  eyes.  The  causes  of  hypersemia  are  overaction  or  strain  of  the 
eyes  due  to  refractive  errors,  or  the  use  of  the  eyes  in  intense  glare  and 
heat,  or  inflammation  of  the  conjunctiva,  iris,  or  choroid,  or  some 
toxsemia,  or  some  form  of  cerebral  or  spinal  disease.  The  condition 
has  been  observed  in  many  different  types  of  insanity,  but  no  form  of 
insanity  is  particularly  characterized  by  this  symptom.  It  is  a  matter 
of  dispute  whether  the  chronic  forms  of  spinal  disease  which  lead  to 
atrophy  produce  a  preliminary  hypersemia  or  ansemia.  I  have  never 
observed  any  indication  of  either  condition  in  cases  which  I  have 
observed. 

The  pallor  of  ansemia  is  almost  as  great  as  that  of  atrophy,  and  in 
the  early  stage  of  atrophy  they  caunot  be  distinguished.  The  causes 
of  ansemia  of  the  disk  are  those  of  general  ansemia.  A  sudden  ansemia 
due  to  embolism  of  the  central  artery  of  the  retina  always  causes  sud- 
den blindness  in  one  eye,  and  the  contrast  between  the  appearance  of 
the  two  eyes  serves  to  establish  the  existence  of  unilateral  ansemia. 
The  arteries  in  one  retina  are  seen  to  be  small  and  empty.  A  moder- 
ate degree  of  ansemia  may  be  caused  by  disease  of  the  bloodvessels  and 


DISEASES  OF  THE  OPTIC  NERVE.  629 

may  lead  to  temporary  blindness.  This  was  the  case  in  a  woman  sixty 
years  old,  who  had  aortic  obstruction  and  extensive  general  endarteritis 
not  specific  in  origin.  She  had  suffered  from  unilateral  headaches  for 
some  years.  On  November  1st  she  took  a  coal-tar  remedy  for  head- 
ache, which  probably  depressed  the  heart,  and  within  a  few  hours  she 
noticed  a  great  dimness  of  vision  in  the  right  eye.  This  increased 
rapidly ;  in  two  days  she  was  nearly  blind,  and  on  December  1st  the 
right  eye  was  totally  blind.  Three  days  later  vision  began  to  return 
in  this  eye,  and  by  January  1st  was  fairly  good.  But  during  the  latter 
part  of  November  the  left  eye  began  to  fail,  and  by  the  middle  of 
December,  while  the  right  eye  was  recovering,  the  left  eye  was  totally 
blind.  In  February  the  left  eye  also  improved,  and  she  finally  recov- 
ered vision  in  both  eyes.  During  all  this  time  the  disks  were  pale,  the 
arteries  small  but  not  obliterated,  and  the  veins  were  distended.  She 
could  see  better  when  lying  flat  on  her  back  than  when  standing.  The 
absence  of  visible  neuritis,  the  marked  arterial  anaemia,  and  the  exist- 
ence of  general  endarteritis  with  aortic  obstruction,  and  the  recovery, 
prove  the  symptoms  to  have  been  due  to  circulatory  conditions.  The 
treatment,  which  consisted  of  full  doses  of  iodide  of  potassium,  90  grains 
daily,  with  strychnine  and  digitalis,  was  attended  not  only  by  relief  of 
the  blindness,  but  also  of  the  headaches,  which  were  probably  due  to 
the  same  cause. 

Thrombosis  of  the  Retinal  Artery  may  cause  local  ansemia  of  the 
retina,  and  may  be  followed  by  oedema  and  hemorrhagic  effusion,  a 
condition  exactly  like  that  which  occurs  in  the  brain.  This  was  the 
condition  found  in  the  following  case  :  A  boy,  aged  thirteen  years,  had 
had  a  continued  fever  for  six  weeks  with  temperature  between  99°  and 
102°  F.,  supposed  to  be  enteric,  but  probably  malarial,  as  the  blood 
examination  showed  the  presence  of  two  generations  of  tertian  malarial 
parasites.  He  then  developed  dimness  of  vision  in  the  left  eye  and 
then  in  the  right.  His  optic  disks  were  swollen  three  diopters,  with 
flame-like  appearance  about  the  disk.  In  the  left  eye  a  wedge-shaped 
area  extended  up  and  in  from  the  nasal  side  of  the  disk,  and  was  color- 
less ;  the  retinal  artery  on  this  side  was  small ;  the  veins  were  normal, 
numerous  dots  about  the  macula  indicating  plastic  exudation.  This 
condition  slowly  improved.  Later  a  marked  picture  of  thrombosis 
of  the  retinal  veins  developed  in  the  right  eye,  with  a  narrowing  of 
the  arteries  and  much  plastic  exudation  about  the  disk,  and  a  few 
retinal  hemorrhages.  This  too  gradually  subsided,  and  after  four 
months  the  optic  nerves  presented  a  fairly  normal  appearance  and  his 
vision  had  returned.  The  origin  was  presumably  malarial  infection, 
as  nephritis  was  not  present,  and  there  were  never  any  signs  of  cerebral 
disease. 

I  have  seen  thrombosis  of  retinal  arteries  associated  with  symptoms 
of  general  cerebral  arterial  disease  which  subsequently  caused  death.  It 
is  U)  be  regarded,  therefore,  as  a  condition  of  prognostic  importance  as 
indicating  a  widespread  arterial  disease.  The  treatment  must  always 
be  directed  to  a  removal  of  the  cause. 


630  TEE  CBANIAL  NEEVES  AND   THEIR  DISEASES. 

(Edema  of  the  Optic  Nerve.  "  Choked  Disk."  —  The  optic  nerve 
at  its  exit  from  the  eyeball  receives  a  double  sheath,  one  layer  of  which 
is  continuous  with  the  dura  mater  and  the  other  with  the  pia  mater. 
When  fluid  accumulates  under  pressure  within  the  cranium  it  therefore 
finds  its  way  into  the  space  between  these  sheaths,  distending  the  dural 
sheath  and  compressing  the  nerve  at  its  exit  from  the  eyeball.  Such 
compression  is  felt  earlier  by  the  lymphatics  and  veins  than  by  the 
harder  nerve  fibres,  and  the  consequent  result  is  a  dropsical  eifusion 
into  the  intraocular  portion  of  the  nerve  from  transudation  of  fluid 
from  the  obstructed  vessels.  This  causes  a  swelling  of  the  optic  disk 
visible  to  the  ophthalmoscope  and  causing  an  appearance  shown  in  Fig. 
265.     Such  a  swelling  may  exist  for  some  time  without  any  impairment 

Fig.  265. 


Swollen  disk  in  a  case  of  chronic  meningitis.     (Liebreich.) 

of  vision,  hence  the  importance  of  ophthalmoscopic  examinations  in  cases 
without  ocular  symptoms.  The  experiments  of  Merz^  have  demon- 
strated that  this  swelling  of  the  nerve  may  come  and  go  rapidly  and 
may  vary  greatly  in  degree,  according  to  the  varying  amount  of  intra- 
cranial presisure.  The  oedema  is  always  attended  by  venous  congestion, 
but  no  inflammatory  changes  may  occur,  and  it  is  therefore  erroneous 
to  identify  choked  disk  with  optic  neuritis.  It  is  not  infrequently 
accompanied  by  hemorrhages  in  the  retina.  Choked  disk  will  occur 
under  any  condition  which  obstructs  the  circulation  in  the  eye  or  in- 
creases the  intracranial  pressure.  It  is  very  common  in  tumors  and 
^  Merz,  Arch,  of  Ophthalmology,  July,  1901. 


DISEASES  OF   TEE  OPTIC  NEEVE.  631 

abscesses  of  the  brain,  occasionally  occurs  temporarily  after  large 
hemorrhaged,  and  not  infrequently  accompanies  Bright's  disease  or 
those  affections  in  which  general  oedema  is  a  symptom. 

The  only  positive  means  of  diagnosis  is  by  ophthalmoscopic  exami- 
nation. In  many  cases  when  the  choked  disk  has  lasted  for  some  time 
vision  is  impaired  and  the  visual  field  is  diminished  in  extent.  In  a 
few  cases  it  produces  no  symptoms.  In  some  cases  it  causes  a  rapidly 
advancing  blindness.  But  in  any  case  this  blindness  may  pass  away 
as  the  oedema  of  the  nerve  subsides,  provided  a  true  neuritis  or  atrophy 
does  not  ensue.  Choked  disk  is  usually  bilateral,  but  may  commence 
in  one  eye  some  time  before  it  appears  in  the  other.  In  some  cases  it 
goes  on  to  neuritis  and  to  atrophy.  Cases  have  been  recorded  of 
choked  disk  without  other  symptoms  and  without  apparent  cause.  In 
two  such  cases,  reported  by  Brudenel  Carter,^  unsuspected  tumor  of  the 
brain  and  latent  abscess  of  the  sphenopalatine  fossa  were  eventually 
proven  to  be  the  cause. 

Optic  Neuritis.  Etiolog"y. —  Optic  neuritis  occurs  occasionally  with- 
out apparent  cause,  and  must  be  regarded  as  an  idiopathic  affection.  It  . 
has  been  known  to  be  hereditary,  and  develops  about  the  age  of  twenty 
and  goes  on  to  blindness.  In  the  majority  of  cases,  however,  it  is 
secondary  to  other  conditions.  It  may  be  due  to  general  poisons  in  the 
blood,  such  as  produce  multiple  neuritis,  namely,  alcohol,  lead,  arsenic, 
mercury,  and  tobacco.  It  is  the  pathological  state  which  is  present  in 
so-called  toxic  amblyopia  and  in  the  affection  described  by  ophthal- 
mologists as  retrobulbar  neuritis ;  where  the  fibres  passing  from  the 
macula  lutea  are  especially  involved.  It  may  occur  as  a  sequel  of  any 
of  the  infectious  diseases,  especially  malaria.  It  also  occurs  occasion- 
ally as  a  symptom  of  severe  chronic  anaemia,  of  pernicious  anaemia, 
leucocythsemia,  and  diabetes.  It  may  be  syphilitic  in  origin.  It  is  a 
frequent  symptom  of  nephritis.  It  is  supposed  to  be  caused  by  rheu- 
matism, by  gout,  or  by  taking  cold.  Optic  neuritis  may  be  secondary 
to  a  meningitis,  in  which  case  the  inflammation  extends  directly  along 
the  sheath  of  the  nerve.  Its  most  common  cause,  however,  is  from 
pressure  within  the  skull  exerted  either  directly  by  tumor  or  abscess  of 
the  brain,  or  syphilitic  exudations  upon  the  base  of  the  brain,  or  by 
the  pressure  of  fluid  in  the  ventricles.  Much  discussion  has  been  de- 
voted to  the  cause  of  optic  neuritis  in  intracranial  disease.  It  may  be 
due  to  the  increase  of  pressure  within  the  head,  producing  oedema  of 
the  nerve  trunk  and  a  secondary  neuritis,  as  Parinaud  affirms.  This 
theory  is  confirmed  by  the  fact  of  the  rapid  subsidence  of  all  the  ap- 
pearances of  optic  neuritis  in  cases  of  brain  tumor  when  the  skull  is 
opened  and  the  intracranial  pressure  relieved.  Thus  in  one  case 
recorded  by  Harvey  Gushing^  a  swelling  of  seven  diopters  was  entirely 
reduced  within  twenty-four  hours  of  an  operation  and  within  a  week 
the  optic  neuritis  had  wholly  disappeared.  It  may  be  due  to  a  direct 
infection  of  the  nerve  by  producits  of  disease  within  the  brain,  as  Leber 
and  Deutschmann  have  long  maintained,  although  the  rapid  subsidence 

1  Clifford  Alllnitt'H  System  of  Medicine,  vol.  vi.,  p.  842. 
*  Surgery,  Gynaicology  and  Obstetrics,  Oct.,  1905. 


632  TEE  CRANIAL  NEEVES  AND   THEIB  DISEASES. 

after  relief  of  pressure  throws  doubt  upon  this  theory.  In  all  these 
cases  optic  neuritis  is  usually  bilateral,  though  in  tumors  of  the  brain, 
upon  the  base,  with  direct  pressure  upon  the  nerve,  it  is  possible  for  the 
nerve  on  the  side  of  the  tumor  to  be  affected  much  sooner  than  the  other. 
Symptoms.  —  The  symptoms  of  optic  neuritis  are  a  sudden  or  gradual 
failure  of  sight,  objects  becoming  blurred,  as  if  a  fog  were  between  the 

Fig.  266. 


Acute  optic  neuritis.  Note  disk  much  swollen ;  estimated  at  +  7  D.  Edge  indistinct ;  vessels 
obscured  at  edge  ;  large  number  of  hemorrhages  around  the  disk,  patches  of  oedema  in  the  retina,  veins 
very  tortuous.     (Posey.) 

eye  and  the  object.  Some  patients  complain  of  sensitiveness  to  light 
and  of  distress  in  using  the  eyes.  There  is  also  a  diminution  of  the 
visual  field  and  a  lack  of  distinctness  of  color  vision,  blue  being  lost 
first.  In  some  cases  irregular  blind  spots  develop  in  the  visual  field. 
It  is  rare  for  total  blindness  to  occur  as  the  result  of  optic  neuritis 
within  six  months  of  the  onset.  The  progress  of  the  blindness  is  con- 
tinuous unless  the  cause  can  be  removed. 

Diagnosis.  —  A  diagnosis  can  be  made  only  by  an  ophthalmoscopic 
examination.  The  figures  on  Plate  XXVI.,  show  the  appearance  of  a 
normal  retina  and  of  a  nerve  in  the  early  stage  of  optic  neuritis.  (See 
also  Fig.  266.)  It  will  be  noticed  that  the  changes  are  very  marked 
in  the  bloodvessels  of  the  retina,  the  arteries  being  smaller  and  the  veins 
being  distinctly  congested.  Their  point  of  exit  in  the  disk  is  often 
obscured.  It  will  be  noticed  that  the  outline  of  the  disk  in  the  early 
stage  is  no  longer  clear  and  in  the  later  stage  is  completely  obliterated. 
The  peculiar  striated  appearance  about  the  disk  is  quite  evident  in  the 
fiarures,  beins:  more  marked  in  the  later  stage  of  the  disease,  and  this 
striation  is  partly  yellowish  and  partly  red,  in  contrast  to  the  uniform 


PLATE  XXVI 

Fig.   1. 


Normal  Eye-ground  (average  tint).     (Norris  and  Oliver.) 


Fig.  2. 


Ophthalmoscopic  Appearances  in  Early  Stage  of  Optic  Neuritis. 
(Norris  and  Oliver.) 


DISEASES  OF   THE  OPTIC  NERVE.  633 

color  of  the  retina  in  health.  In  the  later  stage  hemorrhages  in  the 
retina  are  Evident,  being  indicated  by  red  or  yellowish-white  plaques. 
Small  whitish  or  yellow  spots  are  also  seen  which  are  due  to  exudation 
of  inflammatory  products.  There  is  an  actual  exudation  within  the 
nerve  trunk,  so  that  the  optic  disk  is  actually  swollen.  Its  height  can 
be  estimated  by  the  ophthalmoscope  (1  diopter  equals  f  mm.),  but  the 
first  evidence  of  this  swelling  is  the  disappearance  of  the  clear  central 
space  known  as  the  physiological  cup.  If  the  condition  subsides  there 
is  a  gradual  reduction  of  this  swelling  and  little  by  little  a  return  to 
the  normal  appearance  of  the  disk.  While  the  diagnosis  of  the  exist- 
ence of  optic  neuritis  is  easily  made  by  the  aid  of  the  ophthalmoscope, 
it  is  by  no  means  easy  to  determine  in  every  case  to  what  the  condi- 
tion is  due.  If  to  the  symptoms  of  neuritis  there  are  added  headache, 
malaise,  attacks  of  vertigo,  disorders  of  digestion,  with  nausea  and 
vomiting,  and  some  irregularities  of  circulation,  and  the  patient's 
health,  strength,  and  nutrition  suffer  the  question  at  once  arises  whether 
these  are  the  symptoms  of  tumor  of  the  brain,  chronic  tubercular  men- 
ingitis, general  arterial  disease,  nephritis,  or  merely  of  general  anaemia. 
I  have  seen  two  patients  suffering  from  these  symptoms  during  several 
months  and  not  a  little  distressed  mentally  lest  they  might  be  develop- 
ing a  fatal  affection.  Both  were  young  women  suffering  from  anaemia 
and  also  from  very  irregular  painful  menstruation  due  to  endometritis. 
But  in  both  the  severity  of  the  nervous  symptoms  had  awakened  the 
fear  of  brain  tumor.  Both  eventually  recovered  under  general  tonic 
treatment.  In  such  cases  the  diagnosis  can  be  reached  only  after  a 
considerable  time,  the  lack  of  development  of  local  brain  symptoms, 
especially  in  the  cranial  nerves,  the  existence  of  normal  powers  of  sen- 
sation and  motion,  and  of  normal  reflexes  being  negative  symptoms  of 
great  value.  On  the  other  hand,  the  appearance  of  localizing  brain 
symptoms  may  confirm  the  suspicion  that  the  neuritis  was  the  first  sign 
of  serious  intracerebral  disease,  especially  of  tumor  or  abscess.  I  have 
seen  this  condition  as  an  early  symptom  of  serious  arterial  disease,  which 
led  rapidly  to  multiple  cerebral  thrombosis  with  aphasia  and  hemiplegia 
and  death.  It  is  well  known  that  a  very  common  cause  of  optic  neu- 
ritis is  Bright's  disease,  and  it  follows  that  an  examination  of  the  urine 
should  be  made  in  every  case.  It  is  never  to  be  forgotten  that  abuse 
of  alcohol  and  tobacco  combined  may  be  the  cause  of  optic  neuritis'and 
may  also  produce  other  nervous  symptoms.  I  have  seen  such  a  con- 
dition in  young  women  addicted  to  champagne  and  cigarettes,  as  well 
as  in  many  young  men  of  nervous  constitution  who  were  victims  of 
the  same  habits.  It  is  evident,  therefore,  that  in  any  case  of  optic 
neuritis  great  caution  should  be  exercised  in  giving  a  prognosis,  every 
etiological  factor  should  be  investigated,  and  the  course  of  the  case 
should  be  watched  with  extreme  care.  The  lesion  in  optic  neuritis  is 
similar  to  that  already  described  as  a  diffuse  neuritis  (page  164)  there 
being  a  congestion  of  the  vessels,  an  effusion  of  serum  and  blood 
cells,  of  leucocytes,  and  nuclei  in  the  endoiicurium,  as  well  as  a 
})arenchymut()us  (hsgencration  of  tlie  nerve  fibres. 

Prognosis.  —  The  prognosis  in  the  affection  will  depend  entirely  upon 


634  TEE  CBANIAL  NEBVES  AND   THEIB  DISEASES. 

the  possibility  of  removing  the  cause.  Even  when  the  optic  neuritis 
has  been  quite  extreme  recovery  is  still  possible.  Thus,  after  absorp- 
tion or  removal  of  tumors  of  the  brain  I  have  frequently  seen  recovery 
of  vision  when  the  blindness  had  been  so  extreme  as  to  incapacitate 
the  patient  from  recognizing  even  the  largest  letters  upon  the  test  card. 

Treatment.  —  The  treatment  for  the  atfection  is  the  removal  of  the 
cause,  if  possible,  and  complete  rest  of  the  eyes,  which  should  be  shaded 
from  the  light.  The  ophthalmologists  prescribe  strychnine  by  hypo- 
dermic injections  or  by  the  mouth  in  large  doses,  -^q-  grain,  increased 
at  each  injection  until  in  ten  days  |-  grain  is  given,  and  this  drug  will 
certainly  stimulate  the  nerve  to  activity,  even  when  in  a  state  of 
neuritis.  Whether  such  stimulation  is  desirable  may  be  open  to 
question,  and  certainly  this  treatment  has  no  eflfect  in  other  forms  of 
neuritis,  excepting  a  temp3rary  effect  of  stimulation  of  the  functions 
of  the  nerve.  The  use  of  mercurial  inunctions  and  of  full  doses  of 
iodide  of  potassium  is  recommended  both  in  syphilitic  and  non-syphi- 
litic cases.  The  local  depletion  by  wet  cups  or  leeches  to  the  temple 
is  often  beneficial  in  the  early  stage.  In  anssmic  cases  general  tonics 
are  of  benefit. 

Gushing  advises  that  in  all  cases  of  optic  neuritis  or  of  choked  disk, 
whether  from  intracranial  pressure  or  from  Bright's  disease,  a  relief 
of  pressure  be  secured  either  by  frequently  repeated  lumbar  punctures 
or  by  decompressing  operations  upon  the  cranium.  In  this  way  sight 
may  be  saved.     See  page  613. 

Optic  Nerve  Atrophy.  Etiology. — Optic  neuritis  in  many  cases 
goes  on  to  a  gradual  atrophy  of  the  optic  nerve,  which  is  then  spoken 
of  as  consecutive,  and  in  such  cases  the  atrophy  is  the  terminal  result. 
Increased  pressure  within  the  cranium  may  produce  atrophy  without 
any  primary  neuritis,  the  nerve  appearing  to  undergo  a  degeneration 
from  the  start,  there  being  no  initial  process  of  congestion,  such  as 
occurs  in  neuritis.  A  secondary  atrophy  also  occurs  after  lesions  of 
the  nerve  in  the  orbit  or  after  affections  of  the  optic  chiasm  and  tract. 
Optic  atrophy  may,  however,  occur  as  a  primary  affection  without  any 
preceding  intracranial  pressure  or  disease.  Some  cases  are  congenital 
or  develop  very  soon  after  birth  from  actual  congenital  defective 
development  of  the  optic  nerve.  In  these  cases  other  nervous  symp- 
toms usually  indicate  an  imperfect  development  of  the  nervous  system, 
such  as  imbecility  and  idiocy,  or  spastic  paralysis.  These  cases  have 
been  described  most  fully  by  Sachs.^  Vogt  has  observed  the  same  con- 
dition developing  in  children  at  the  age  of  eight  or  ten.  In  many  cases 
primary  optic  atrophy  is  a  condition  associated  with  other  diseases, 
though  it  can  hardly  be  called  secondary  to  them.  This  is  notably  the 
case  in  locomotor  ataxia,  in  disseminated  or  multiple  sclerosis,  and  in 
paresis.  In  all  of  these  diseases  the  occurrence  of  optic  atrophy  is  an 
exceedingly  serious  complication  and  probably  due  to  the  same  cause 
which  produces  the  original  affection.  It  may  be  the  first  lesion  pro- 
duced by  this  cause.     Thus  in  locomotor  ataxia  10  per  cent,  of  the  cases 

^  Amaurotic  Family  Idiocy,  Journal  of  Nervous  and  Mental  Disease,  1887,  p.  541, 
and  1903,  p.  1. 


PLATE   XXVII 


Primary  Atrophy  of  Optic  Nerve  (Spinal       Post-papillitic  or  Consecutive  Atrophy  of 
Atrophy).     Modified  from  Haab.  the  Optic  Nerve.   Modified  from  Juler. 


Embolic  Atrophy  of  the  Optic  Nerve.     (De  Schweinitz,  in  Dercum's  Nervous  Diseases.) 


DISEASES  OF   THE  OPTIC  NEEVE.  635 

begin  with  optic  atrophy.  And  in  multiple  sclerosis  52  per  cent,  of  the 
patients  de^i^elop  this  complication.  Optic  atrophy  may  occur  as  the 
result  of  poisoning  by  tobacco,  alcohol,  lead,  and  quinine.  It  may 
occur  as  the  result  of  the  various  infectious  diseases  and  of  diabetes, 
and  in  some  cases  has  been  ascribed  to  cold. 

Symptoms.  —  The  symptoms  of  optic  atrophy  are  a  gradually  increas- 
ing blindness,  a  diminution  of  the  visual  field,  progressively  increasing 
from  without  inward,  so  that  finally  only  central  vision  is  possible,  and 
very  often  a  defective  color  vision,  the  fields  for  various  colors  being 
either  progressively  reduced  in  extent  or  altered  in  their  extent.  In 
some  cases  irregularly  situated  blind  spots,  sometimes  central,  develop 
—  the  so-called  central  scotoma.  Pain  in  the  eyes  and  discomfort  after 
their  use  or  after  exposure  to  strong  light  are  often  felt. 

The  appearances  in  optic  atrophy  are  demonstrated  in  Plate  XXVII. 
where  both  gray  and  white  atrophy  are  shown.  The  ophthalmoscopic 
appearances  in  optic  nerve  atrophy  are  not  uniform.  When  the  atrophy 
is  secondary  to  the  optic  neuritis  there  are,  first,  the  ordinary  changes 
observed  in  the  neuritis,  and  then,  as  the  swelling  and  congestion  sub- 
side, the  disk  once  more  becomes  evident,  has  a  clearer  and  clearer  out- 
line, but  an  unusually  white  appearance,  until  finally  it  presents  a 
perfectly  white  spot  in  the  retina.  The  arteries  are  noticeably  dimin- 
ished in  calibre.  When  the  optic  atrophy  is  primary  and  is  not  pre- 
ceded by  a  neuritis  there  is  a  gradual  progressive  change  in  the  appear- 
ance of  the  normal  disk,  which  becomes  more  sharply  outlined,  loses  its 
pink  tint,  and  becomes  more  and  more  gray,  or  even  clear  white  in  color, 
the  arteries  and  veins  preserving  their  normal  calibre.  After  a  time  the 
lamina  cribrosa  at  the  bottom  of  the  disk  may  become  evident,  the  ap- 
pearance presented  resembling  a  fine  sieve.  The  lesion  in  optic  atrophy 
is  a  progressive  parenchymatous  neuritis  without  any  interstitial  change. 

Diagnosis.  —  The  diagnosis  of  the  underlying  condition  in  a  case  of 
optic  atrophy  is  of  as  great  importance  as  in  a  case  of  optic  neuritis. 
Toxic  amblyopia  should  be  first  excluded.  Symptoms  of  organic  brain 
or  spinal-cord  disease  should  be  looked  for.  The  mental  characteristics 
of  the  patient  should  be  considered,  and  changes  indicative  of  begin- 
ning paresis  should  not  be  overlooked.  And  the  case  should  be  watched 
for  a  long  period  before  giving  a  diagnosis  of  primary  optic  atrophy. 
More  than  one-half  of  the  cases  eventually  develop  some  form  of  spinal 
sclerosis. 

Prognosis.  —  The  prognosis  in  optic  nerve  atrophy  is  much  more 
serious  than  in  optic  neuritis,  for  while  the  latter  may  go  on  to 
recovery,  the  former  is  progressive,  and  treatment,  unless  directed  to 
the  cause,  is  not  successful. 

Treatment. — The  progress  of  the  case  may  be  delayed  by  the  use 
of  tonics,  by  everything  which  improves  the  general  health,  and  by 
the  hypodermic  use  of  strychnine,  from  ^  up  to  |-  grain  daily,  the 
dose  being  increased  slowly,  or  of  the  chloride  of  gold,  or  nitrate  of 
silver,  or  of  nitroglycerin.  Recently  hypodermic  injections  of  normal 
salt  solution  beneath  the  conjunctiva  havfi  been  recommended.  They 
have  not  been  useful  in  my  cases. 


636 


TEE  CMANIAL  NE:rVES  AND  THEIH  DISEASES. 


THE  THIRD,  FOURTH  AND  SIXTH  NERVES :   THE  MOTOR  NERVES 
OF  THE  EYEBALL.     OPHTHALMOPLEGIA. 

The  eyeball  and  eyelid  are  moved  by  numerous  muscles  supplied  by 
these  three  nerves,  as  follows  : 

The  third  nerve  supplies  the  ciliary  muscle,  sphincter  iridis,  levator 
palpebrse,  superior  rectus,  internal  rectus,  inferior  rectus,  inferior 
oblique. 

The  fourth  nerve  supplies  the  superior  oblique. 

The  sixth  nerve  supplies  the  external  rectus. 

The  branches  of  the  cavernous  plexus  of  sympathetic  nerves  sup- 
ply the  dilator  pupillse.  These  nerves  arise  from  groups  of  cells 
lying  upon  the  floor  of  the  aqueduct  of  Sylvius  and  of  the  fourth 
ventricle  of  the  brain.     (Fig.  267.)     The  groups  are  closely  joined  to 

Fig.  267. 


Sagittal  section  through  the  cerebral  axis,  to  show  the  nuclei  of  the  ocular  nerves  in  the  floor  of 
the  aqueduct  of  Sylvius  and  the  fourth  ventricle,  and  the  course  of  the  nerves  to  their  exit.  The 
various  groups  of  cells  from  which  the  third  nerve  arises  are  seen.  RN,  red  nucleus  of  tegmentum  ; 
L,  lemniscus  (sensory  tract)  ;  CC,  motor  tract  in  the  crus  cerebri  seen  to  traverse  the  pons  and  enter 
the  anterior  pyramid  of  the  medulla. 

each  other  by  association  fibres,  the  longest  of  which,  between  the 
third  and  sixth  nerve  nuclei,  pass  in  the  posterior  longitudinal 
bundle. 

The  location  of  the  nuclei  of  the  oculomotor  nerves  and  the  function 
of  each  group  of  cells  has  been  the  subject  of  much  discussion,  and 
unanimity  of  view  has  not  yet  been  reached,  though  the  subject  has 
been  approached  from  the  side  of  comparative  anatomy  (Spitzka,  Edin- 
ger),  embryology  (von  Gudden,  Edinger),  physiological  experimenta- 
tion (Hensen  and  Volchers,  Bernheim),  and  pathology  (Westphal, 
Kahler,  Pick,  and  Starr),  as  well  as  from  the  histological  standpoint 
(Kolliker,  Perlia,  Bruce). 

The  third  nerve  nucleus  consists  of  a  long  column  of  cells  which 
begin  in  the  gray  matter  of  the  wall  of  the  third  ventricle  near  the 


MOTOB  NEBVES  OF  THE  EYEBALL.     OPHTHALMOPLEGIA.      637 

posterior  commissure  on  the  median  surface  of  the  optic  thalamus,  and 
extends  for  almost  a  centimetre  in  length  under  the  floor  of  the  aque- 
duct of  Sylvius  downward  to  the  level  of  the  corpora  quadrigemina 
posterior.  Just  posterior  to  this  column  lies  the  group  of  cells  giving 
origin  to  the  fourth  nerve,  and  about  3  cm.  below  in  the  pons  is  the 
sixth  nerve  nucleus.  The  column  of  cells  making  up  the  third  nerve 
nucleus  can  be  divided  into  a  number  of  groups.  These  groups,  their 
connections,  and  their  special  functions  are  shown  in  Figs.  267  and 


Fig.  268. 
Nucl.  med.  ant. 


Nucl.  Edinger-  Westphal 


Levels  of  sections  frmn 
which  the  figures  ac- 
companying Perlia's 
article  were  taken. 


Nucl.  lal.  ant 
(Darkschewitsch) 


Nucl.  dors.  I  {ant.) 


Nucl.  vent.  I  {ant. 

Nucl.  centralis 

Nucl.  dors.  II  {post.). 
[v.  Gudden) 


Nucl.  vent.  II  ( post. ) 


Nucl.  Trochl. 


Scheme  of  the  nuclei  of  the  oculomotor  nerve.     (After  Perlia,  Arch.  f.  Ophth.,  Leipzig, 
Bd.  XXV.,  Abth.  iv.,  S.  297.) 

268.  That  each  group  controls  a  single  muscle  is  proven  by  the  fact 
that  small  lesions  limited  to  different  parts  of  the  column  of  cells  and 
affecting  certain  groups  or  their  nerve  roots  only  produce  different 
forms  of  paralysis  of  the  eyeball.  A  study  of  the  combination  of 
paralyses  observed  in  various  cases  enabled  the  writer  (in  1888^)  to 
offer  the  following  scheme  of  the  order  in  which  the  muscles  are  rep- 
resented in  these  groups  : 

1  .Joum,  of  Nerv,  and  Ment.  Dis.,  vol.  xv.,p.  301, 


638  THE   CBANIAL  NEBVES  AND    THEIE  DISEASES. 

Table  XVII. — Arrangement  of  III.  Nerve  Nuclei. 
Eight  Side. 


Median  line. 


Ciliary  muscle.  Sphincter  iridis. 

Eectus  internus.         Levator  palpebrae. 

Rectus  superior. 
Rectus  inferior.  Obliquus  inferior. 

Obliquus  superior. 


Rectus  externus. 
Subsequent  observations  have  lent  support  to  this  localization. 

Third  Nerve  Paralysis. 

The  third  nerve,  after  arising  from  its  groups  of  cells  upon  the  floor 
of  the  aqueduct  of  Sylvius,  sends  its  various  roots  through  the  teg- 
mentum of  the  crus  cerebri,  which  traverse  on  their  way  the  red  nu- 
cleus of  the  tegmentum  and  make  their  exit  in  a  large  bundle  on  the 
inner  side  of  the  crus  in  close  apposition  to  the  third  nerve  of  the  op- 
posite side.  Lesions  of  the  groups  of  cells  of  origin  of  the  third  nerve 
may  produce  a  partial  paralysis  of  the  ocular  muscles.  So,  too,  may 
lesions  of  the  roots  of  the  third  nerve  in  their  passage  through  the  crus. 
But  lesions  of  the  nerve  after  its  exit  cause  paralysis  of  all  the  muscles 
which  it  supplies.  The  third  nerve  passes  from  its  superficial  origin 
in  the  crus  forward  to  the  sphenoidal  fissure,  and  thus  into  the  orbit, 
where  it  divides  up  in  the  various  small  branches  going  to  the  muscles 
already  named  and  to  the  ciliary  ganglion. 

Etiology.  —  Lesions  upon  the  base  of  the  brain  lying  near  the  crus 
or  between  it  and  the  exit  of  the  nerve  into  the  orbit  are  particularly 
liable  to  produce  paralysis  of  the  third  nerve.  Meningitis,  either  sim- 
ple or  tubercular,  abscess  of  the  brain,  and  hemorrhages  on  the  base 
often  involve  the  nerve.  In  meningitis  in  infants  it  is  often  the  nerve 
first  affected.  Syphilitic  exudations  upon  the  base  of  the  brain  and  a 
syphilitic  neuritis  with  infiltration  of  the  sheath  of  the  nerve  are  the 
most  common  causes  of  third  nerve  palsy.  Tumors  of  the  brain  lying 
in  this  locality  or  lying  in  such  position  as  to  displace  the  brain  axis, 
and  thus  cause  traction  upon  the  nerve,  may  also  produce  third  nerve 
paralysis.  In  both  these  conditions  other  symptoms  of  the  disease 
accompany  the  ocular  palsy  and  give  a  clue  to  the  diagnosis.  The  ocu- 
lomotor nerve  is  rarely  injured  within  the  orbit,  inasmuch  as  it  is  well 
protected,  but  occasionally  in  fractures  of  the  base  the  nerve  will  be 
torn,  and  severe  falls  and  blows  upon  the  head  have  been  known  to 
cause  the  bruising  of  the  nerve  against  a  sharp  edge  of  the  sphenoidal 
fissure.  Thus  in  a  patient  under  my  own  observation  a  fall  from  a  car- 
riage upon  the  side  of  the  head  produced  a  sudden  paralysis  of  the  third 
nerve  which  remained  about  six  months  and'  then  gradually  recovered. 
Tumors  within  the  orbit  and  anything  producing  extreme  exophthal- 
mus  may  by  pressure  or  stretching  involve  one  or  more  branches 
of  the  oculomotor  nerve,  and  thus  cause  a  partial  paralysis.  Thus  in 
a  patient  with  exophthalmic  goitre  and  very  extreme  exophthalmus  I 
have  seen  an  inability  to  turn  the  eye  inward  and  upward  which  sub- 
sided when  the  exophthalmus  disappeared.     Oculomotor  palsy  may 


TRIBB  NEBVE  PAEALYSIS. 


639 


occur  as  a  complication  of  diphtheria  and  of  typhoid  fever,  and,  in  fact, 
it  has  bee^  recorded  in  many  different  forms  of  multiple  neuritis ; 
under  these  circumstances  it  is  usually  bilateral.  German  authorities 
afl&rm  that  a  temporary  oculomotor  palsy  may  be  produced  by  poison- 


FiG.  269. 


A  section  through  the  cms  cerebri,  showing  the  origin,  course,  and  exit  of  the  oculo- 
motor nerves.     (Edinger.) 


ing  with  sausage,  fish,  or  meat  that  is  diseased.  Lastly,  it  is  supposed 
that  the  eifect  of  cold  may  produce  a  sudden  oculomotor  palsy  of  tem- 
porary duration,  but  this  is  certainly  very  rare. 

Symptoms.  —  The  symptoms  of  third  nerve  paralysis  are  ptosis  or 
falling  of  the  upper  eyelid,  dilatation  of  the  pupil,  and  loss  of  reflex 
action  both  to  light  and  to  accommodation  and  a  turning  outward  and 
slightly  downward  of  the  eyeball  owing  to  the  unopposed  contraction 
of  the  external  rectus  and  superior  oblique  muscles.  The  strabismus 
is  attended  by  double  vision  and  often  by  vertigo  and  uncertainty  of 
gait.  There  is  an  inability  to  turn  the  eyeball  upward,  downward,  or 
inward,  owing  to  the  paralysis  of  the  superior,  inferior,  and  internal 
recti  muscles.  The  paralysis  of  the  inferior  oblique  muscle  is  also 
present,  but  does  not  produce  a  visible  deflection  of  the  eye  when  the 
superior  oblique  is  capable  of  acting.  Ptosis  is  complete,  so  that  the 
patient  is  unable  to  open  the  eye  sufiiciently  to  see,  although  he  usually 
makes  efl'orts  in  this  direction  by  elevating  the  eyebrow  to  the  utmost 
limit ;  hence  in  such  patients  wrinkling  of  the  forehead  is  more  pro- 


640 


THE  CBANIAL  NERVES  AND   THEIB  DISEASES. 


nounced  on  the  side  of  the  paralysis.  Fig.  270  shows  this  condition. 
In  cases  where  the  cause  can  be  removed  by  treatment  the  prognosis  is 
good,  and  even  in  cases  of  injury  and  disease  of  the  nerve  from  cold, 

recovery,    as    a   rule,    ensues. 
Fig.  270.  The    nerve   is   inaccessible   to 

any  form  of  electrical  treat- 
ment, therefore  it  is  impos- 
sible to  treat  the  paralysis. 
The  Pupil  and  Its  Nervous 
Mechanism.  —  The  action  of 
the  pupil  to  light  and  to  ac- 
commodation is  a  reflex  act  in 
which  the  optic,  oculo-motor, 
and  sympathetic  nerves  take 
part. 

The  contraction  of  the  pupil 
to  light  is  produced  by  im- 
pulses passing  from  the  retina 
to  the  corpora  quadrigemina 
anterior ;  thence  to  the  ante- 
rior group  of  cells  in  the  third 
nerve  nucleus,  thence  in  the 
third  nerve  to  the  ciliary  gan- 
glion, from  which  the  short 
ciliary  nerves  go  to  the  iris, 
which  contracts  by  closure  of 
its  sphincter. 

Any  lesion  which  breaks  this  reflex  arc  will  arrest  the  contraction 
of  the  pupil  to  light.  The  dilatation  of  the  pupil  in  darkness  or  in 
distant  vision  is  produced  by  impulses  passing  to  the  dilator  pupillse 
through  the  sympathetic  nerves  from  the  cilio-spinal  centre  in  the 
eighth  cervical  and  first  dorsal  segments  of  the  spinal  cord.  These 
spinal  centres  are  connected  with  the  corpora  quadrigemina  by  nerve 
fibres  which  pass  in  the  tegmentum  of  the  pons  and  medulla  and  in  the 
antero-lateral  columns  of  the  upper  cord.  The  sympathetic  nerve  fibres 
leave  the  spinal  cord  in  the  first  dorsal  nerve  root,  pass  to  the  superior 
cervical  ganglion,  thence  in  the  carotid  or  cavernous  plexus  to  the 
Gasserian  ganglion  of  the  fifth  nerve,  and  thence  by  way  of  the  first 
branch  of  the  fifth  nerve  and  the  long  ciliary  nerves  to  the  iris.  The 
cilio-spinal  centre  in  the  spinal  cord  receives  impulses  from  many 
directions,  any  one  of  which  is  capable  of  producing  a  dilatation  of  the 
pupil.  Thus  irritating  impulses  coming  in  through  the  lower  cervical 
and  upper  dorsal  posterior  spinal  nerve  roots  from  the  skin  of  the  neck, 
or  if  of  sufficient  intensity  from  any  region  of  the  body,  can  cause  such 
a  dilatation.  It  is  possible  that  the  total  absence  of  such  impulses  when 
the  posterior  nerve  roots  in  the  cervical  cord  are  injured  causes  the  per- 
manent contraction  of  the  pupil,  called  spinal  myosis.  Impulses  also 
reach  the  cilio-spinal  centre  from  the  cortex  of  the  bra,in  and  from  many 


Ptosis  and  external  strabismus  due  to  paralysis  of  the 
right  third  nerve.     (Icon,  de  la  Salpetriere. ) 


THIBD  NEBVE  P  ABA  LYSIS. 


641 


subcortical  centres  in  the  basal  ganglia  and  medulla.  Attention  directed 
to  a  distant''  object,  with  voluntary  fixation  of  the  eyes,  or  emotional 
changes,  fear  or  anger,  or  any  irritation  of  the  medulla  which  excites 
the  respiratory  and  vasomotor  centres  may  cause  dilatation  of  the  pupil. 
It  is  the  last-named  cause  which  produces  a  contracted  pupil  in  men- 
ingitis and  cerebral  disease.  A  general  lowering  of  the  nervous  con- 
dition such  as  accompanies  neurasthenia,  hysteria,  epilepsy,  and  anaemia 
is  usually  attended  by  a  dilatation  of  the  pupil.  The  exact  path  from 
the  optic  centres  to  the  spinal  centre  is  as  yet  undiscovered.  A  per- 
manent loss  of  power  of  dilatation  of  the  pupil  in  darkness  is  known 
to  occur  only  when  the  cilio-spinal  centre  in  the  cord  or  the  sympa- 
thetic nerve  in  the  neck  or  its  branches  to  the  dilator  pupillse  are 
destroyed  by  disease.     This  occurs  in  tabes,  in  paresis,  in  multiple 

Fig,  271. 


Diagram  of  the  visual  tract  and  its  connection  with  the  third  nerve.  JV.o.,  optic  nerve ;  ehs., 
chiasm  ;  tr.  o.,  optic  tract ;  gen.  Za<., corpus  geniculatum  externum  ;  bra.,  brachum  conjunctivum  ;cg.  a., 
corpus  quadrigeminum  anierioT  ; pulv.,  ulvinar ;  ^rera.  m.,  corpus  geniculatum  internum;  tor.,  teg- 
mentum; a.s.,  aqueduct  of  Sylvius.     (Jacob.) 


sclerosis,  and  in  bulbar  palsy,  and  in  myelitis  of  this  locality.  The 
hemiopic  pupillary  reflex  of  Wernicke  has  been  described  on  page  628. 
When  a  patient  presents  anomalies  in  the  action  of  the  pupils  the  pos- 
sibility of  his  being  under  the  influence  of  some  drug  which  afiects  their 
action  must  be  considered  before  a  conclusion  is  reached  that  a  local  or 
general  disease  of  the  nervous  system  is  present. 

Argyll-Robertson  Pupil.  —  This  is  a  condition  of  loss  of  reflex  of  the 
pupil  t()  light,  while  its  action  in  accommodation  is  preserved.  (See 
pages  3/3G  and  045.) 

41 


.642  THE  CBANJAL  NEBVES  AND   THEIB  DISEASES. 

Paralysis  of  the  Fourth  Nerve.     Patheticus  Paralysis. 

Paralysis  of  the  fourth  nerve  is  extremely  rare  aud  is  ouly  to  be 
detected  by  a  careful  examination  for  double  images  with  a  red  glass 
over  one  eye.  The  upright  image  is  then  seen  to  be  upright  by  the 
normal  eye,  but  appears  to  be  displaced  outward  and  turned  obliquely 
by  the  affected  eye.  The  fourth  nerve  supplies  the  superior  oblique 
muscle  and  is  of  all  the  nerves  the  one  best  protected  within  the  brain. 
It  arises  from  a  small  group  of  cells  beneath  the  aqueduct  of  Sylvius, 
and  decussates  with  its  fellow  in  the  roof  of  the  aqueduct  just  beneath 
the  corpora  quadrigemina  posterior,  and  makes  its  exit  immediately 
after  its  decussation  on  the  dorsal  surface  of  the  crus  lying  upon  the 
superior  peduncle  of  the  cerebellum.  (Fig.  267.)  It  then  curves 
around  the  crus,  lying  upon  the  upper  border  of  the  pons  Varolii,  and 
so  passes  forward  upon  the  side  of  the  optic  tract  and  enters  the  orbit 
through  the  sphenoidal  fissure.  Any  of  the  causes  which  produce 
paralysis  of  the  third  nerve  may  cause  paralysis  of  the  fourth.  Snyder  ^ 
reports  several  cases  due  to  injury,  attended  by  vertigo.  Isolated  paral- 
ysis of  the  fourth  nerve  without  affection  of  the  third  or  sixth  is 
an  important  symptom  of  tumor  of  the  cerebellum  or  of  exudation  on 
the  under  surface  of  the  anterior  lobe  of  the  cerebellum.  In  a  patient 
seen  in  Meynert's  clinic  at  Vienna,  who  presented  many  symptoms  of 
cerebellar  disease,  the  existence  of  an  isolated  fourth  nerve  paralysis 
made  it  probable  that  the  cerebellar  lesion  lay  in  the  anterior  and  ven- 
tral part  of  the  cerebellum,  a  diagnosis  which  was  confirmed  by  autopsy. 

Paralysis  of  the  Sixth  Nerve.     Abducens  Paralysis. 

The  symptom  produced  by  paralysis  of  the  sixth  nerve  is  internal 
strabismus.  This  nerve  supplies  the  external  rectus,  and  when  it  is 
paralyzed  the  internal  rectus,  being  unopposed,  turns  the  eye  inward, 
producing  strabismus  and  an  inability  to  turn  the  eye  outward. 
Double  vision  always  accompanies  the  strabismus,  and  the  pupil  is 
usually  somewhat  contracted  on  account  of  the  position  of  the  eyeball, 
but,  of  course,  reacts  to  light.  The  sixth  nerve  arises  from  a  group 
of  cells  upon  the  floor  of  the  fourth  ventricle,  passes  ventrally  through 
the  pons  Varolii,  making  its  exit  in  the  groove  between  the  pons  aud 
medulla,  and  then  passes  forward  beneath  the  pons  to  enter  the  orbit 
through  the  sphenoidal  fissure.  As  it  has  the  longest  course  of  any 
cranial  nerve,  disease  at  any  part  of  the  base  of  the  brain  is  liable  to 
involve  it,  hence  abducens  paralysis  is  a  frequent  symptom  of  intra- 
cranial disease,  such  as  basilar  meningitis,  syphilis  of  the  base,  tumors 
of  the  brain,  and  fractures  at  the  base  of  the  skull.  The  same  causes 
which  produce  oculomotor  paralysis  may  cause  abducens  paralysis, 
and  it  is  equally  inacessible  to  any  treatment.  The  sixth  nerve  nucleus 
and  the  root  of  the  nerve  are  often  involved  in  disease  of  the  pons 
Varolii.     (See  pages  117  and  615.) 

lArch,  of  Ophthalmologj,  vol.  XXXVI.,  p.  988. 


PABALYSIS  OF  THE  SIXTH  NERVE.    ABDUCENS  PARALYSIS.    643 


The  diagnosis  between  an  affection  of  the  abducens,  due  to  disease 
in  the  ner^  trunk  or  due  to  disease  in  the  brain,  can  only  be  made 
from  the  presence  of  other  symptoms.  Paralysis  of  the  face  on  the 
same  side  as  the  ocular  palsy,  and  of  the  arm  and  legs  upon  the  oppo- 
site side,  indicates  a  lesion  in  the  pons  or  on  the  base,  producing  uni- 
lateral pressure  on  the  pons.  In  the  patient  whose  face  is  shown  in 
Fig.   272   the  paralysis  of  the 

sixth  nerve  was  associated  with  Fig.  272. 

paralysis  of  the  seventh  and 
eighth  nerves  on  the  same  side. 
This  patient  had  suffered  from  a 
fracture  of  the  base  of  the  skull, 
but  had  recovered  from  all 
the  symptoms  excepting  those 
present  in  these  three  nerves. 

The  Conjugate  Motions  of 
the  Eyes. —  In  all  acts  of  vision 
the  eyes  move  together,  either 
being  converged  to  a  near  object, 
diverged  in  looking  at  a  distance, 
or  turned  together  in  any  di- 
rection. 

In  convergence  the  internal 
recti  act  together  and  there  is  a 
contraction  of  the  pupil. 

In  divergence  the  external 
recti  act  together  and  there  is  a 
dilatation  of  the  pupil. 

In  conjugate  lateral  motions  opposite  muscles  in  the  two  eyes  act 
together.  In  motions  upward  and  downward  homogeneous  muscles 
act  together.  All  these  movements  are  primarily  reflex  and  automatic, 
the  sensations  of  objects  upon  the  retina  causing  the  appropriate  action. 
For  such  acts  the  interaction  of  the  various  nuclei  of  the  optic  and 
motor  nerves  of  the  eyeballs  is  all  that  is  necessary.  And  the  very 
complete  system  of  association  fibres  joining  these  various  nuclei  to 
one  another  provides  for  such  combinations  of  movement.  Lesions 
which  destroy  these  association  fibres,  especially  lesions  in  the  posterior 
longitudinal  bundle,  interfere  with  conjugate  lateral  motions.  Lesions 
in  the  nuclei  themselves  necessarily  suspend  consensual  motions  in  any 
direction,  both  reflex  and  voluntary.  The  connections  of  these  nuclei 
are  shown  in  Fig.  267. 

Conjugate  movements  may  also  be  voluntary  in  origin.  The  motor 
centres  in  the  cerebral  cortex  for  ocular  movements  are  located  in  the 
anterior  part  of  the  motor  zone.  (See  page  92.)  They  send  their 
impulses  down  to  the  subcortical  centres  by  a  tract  which  passes 
through  the  knee  of  the  internal  capsule,  occupies  a  position  near  the 
median  line  in  the  foot  of  the  cms  cerebri  and  decussates  in  the  raph6 
before  it  ends  in  the  oculomotor  nuclei.     The  tract  conveying  impulses 


Paralysis  of  the  left  sixth,  seventh,  and  eighth  nerves 
from  a  fracture  at  the  base  of  the  skull. 


644 


THE   CRANIAL  NEBVES  AND    THEIB  DISEASES. 


of  conj  agate  lateral  motion  passes  directly  to  the  sixth  nerve  nuclei, 
and  thence  an  impulse  is  sent  back  to  the  opposite  third  nerve  by  way 
of  the  posterior  longitudinal  bundle.  Fig.  273  shows  this  tract  and 
demonstrates  the  fact  that  lesions  at  different  parts  of  it  cause  paralysis 
of  lateral  conjugate  motion  of  the  two  eyes. 

Irritation  of  the  motor  centres  in  the  cortex  causes  involuntary  turn- 
ing of  the  eyes  away  from  the  side  of  the  lesion.  This  happens  in  epilepsy 
when  the  focus  of  irritation  which  starts  the  fit  is  unilateral.  Paralysis 
of  the  motor  centres  in  the  cortex,  or  destruction  of  the  tract  which 
conveys  their  impulses  will  eventually  result  in  conjugate  deviation  of 
the  eyes  toward  the  side  of  the  lesion,  for  the  eyes  being  once  turned  in 


Fig.  273. 


Diagram  to  illustrate  the  mechanism  of  conjugate  movements  of  the  eyes.  P,  pons  Varolii  ;  ///, 
oculomotor  nuclei ;.  VI,  abducens  nuclei ;  S.i.,  rectus  internus  ;  R.e.,  rectus  externus.  A  tract  from  the 
cortex  passes  to  VI.  A  tract  connects  each  F/ ^  ith  the  opposite ///.  C,  centre  of  convergent  move- 
ment of  both  eyes —  a  part  of  ///;  A,  lesion  in  the  left  half  of  the  brain,  causing  a  loss  of  voluntary 
turning  of  both  eyes  to  the  right ;  B,  lesion  in  the  left  half  of  the  pons,  causing  a  loss  of  voluntary 
turning  of  both  eyes  to  the  left ;  C,  lesion  in  the  left  posterior  longitudinal  bundle,  causing  a  loss  of 
voluntary  conjugate  motion  of  the  eyes  to  the  right ;  D,  lesion  in  the  left  VI  nucleus,  causing  a  loss  of 
voluntary  turning  of  the  eyes  to  the  left. 

that  direction  remain  fixed,  there  being  no  possibility  of  a  voluntary 
correction  of  this  position.  Hence  in  severe  attacks  of  apoplexy  when 
this  tract  is  destroyed  the  patient  is  said  to  look  toward  his  lesion. 

The  diagnosis  of  the  situation  of  a  lesion  producing  forced  conjugate 
deviation  of  the  eyes  can  only  be  made  from  the  existence  of  other 
symptoms  of  cerebral  or  pons  disease.  These  symptoms  have  been 
more  fully  discussed  in  Chapter  V. 

Rheumatic  Ocular  Palsy.  —  A  paralysis  affecting  one  or  more  of 
the  ocular  muscles  must  not  be  too  hastily  ascribed  to  a  lesion  of  the 
nerve.     These  muscles  are  subject  to  rheumatic  affections  like  other 


OPHTHALMOPLEGIA.  645 

muscles,  an^  occasionally  a  supposed  ophthalmo])legia  turns  out  to  be 
a  rheumatic  myositis,  and  subsides  promptly.  The  external  rectus  is 
the  muscle  most  frequently  affected. 

Recurrent  Oculomotor  Palsy.  —  A  rare  condition  consisting  of 
combined  paralysis  of  several  of  the  muscles  of  temporary  duration, 
but  commonly  recurring  several  times  in  the  course  of  a  short  attack, 
has  been  described  and  named  recurrent  oculomotor  palsy.  The  oculo- 
motor nerve  is  the  one  usually  affected,  but  the  fourth  and  sixth  may 
also  be  involved.  The  attacks  of  double  vision  are  attended  by  head- 
ache, nausea,  vomiting,  and  by  a  fever, and  occasionally  by  conjunctivitis. 
They  have  been  termed  ophthalmic  migraine  by  Charcot.  In  one  case 
seen  by  me  the  cause  was  probably  malarial  infection  and  the  attacks 
ceased  under  quinine.  Darquier^  and  Knapp^  have  collected  and 
analyzed  about  forty  cases.  The  lesion  is  probably  a  vascular  one, 
congestion  or  oedema  either  in  the  nuclei  or  in  the  nerve  trunks.  In 
one  case  a  vascular  tumor  was  found  on  the  trunk  of  the  third  nerve. 

Ophthalmoplegia. 

Disease  in  the  nuclei  of  the  nerves  that  supply  the  muscles  of  the 
eyeballs  and  paralyze  them  is  termed  ophthalmoplegia.  It  has  been 
divided  into  ophthalmoplegia  interna  and  externa,  according  to  the 
muscles  afPected.  In  ophthalmoplegia  interna  the  action  of  the  pupil 
is  afPected.  In  ophthalmoplegia  externa  the  motion  of  the  eyeballs 
and  of  the  levator  palpebrse  are  impaired. 

Ophthalmoplegia  Interna. — Ophthalmoplegia  interna  is  a  very 
rare  condition.  It  may  be  unilateral  or  bilateral.  It  may  be  acute  or 
chronic  in  its  onset.  There  are  no  cases  with  autopsy  on  record  and 
the  condition  as  a  symptom  is  a  curiosity. 

Loss  of  reflex  to  light  while  reflex  action  in  accommodation  is  pre- 
served, called  the  Argyll-Robertson  pupil,  or  reflex  iridoplegia,  occurs 
as  a  symptom  in  tabes,  in  paresis,  in  disseminated  sclerosis,  in  soften- 
ing or  atrophy  of  the  brain,  in  syphilis  of  the  brain,  in  hydrocephalus, 
in  tumors  of  the  third  ventricle  or  adjacent  basal  ganglia,  and  as  a 
congenital  condition.  It  is  supposed  to  point  to  a  suspension  of  action 
in  the  association  neurones  between  the  optic  terminals  and  the  oculomo- 
tor ciliary  nuclei.  These  axones  pass  through  the  gray  wall  of  the  aque- 
duct of  Sylvius,  and  in  all  these  diseases  except  tabes  microscopic 
lesions  in  this  region  have  been  observed.  There  is  as  yet  no  satisfac- 
tory explanation  for  the  occurrence  of  this  symptom  in  tabes.  The 
condition  is  usually  eventually  bilateral,  but  may  be  unilateral  at  first. 
Loss  of  action  of  the  pupil  in  accommodation  is  known  to  occur  with- 
out loss  of  action  to  light.  This  is  a  rare  condition,  is  usually  uni- 
lateral and  due  to  a  lesion  in  the  cervical  sympathetic. 

Ophthalmoplegia  Externa.  —  This  term  is  applied  to  any  form  of 
paralysis  of  the  muscles  moving  the  eyeball,  not  due  to  a  lesion  in  the 

'  Annales  d'oculistlque,  Oct.,  1893,  tome  ex.,  p.  258. 
2  Boston  Med.  and  >Surg.  Jour.,  Sept.,  1894. 


646 


THE  CBANIAL  NEUVES  AND   THE  IB  DISEASES. 


periphery  of  one  nerve  alone.  It  is  usually  bilateral.  It  is  usually 
partial,  a  few  only  of  the  ocular  muscles  being  involved.  It  may, 
however,  become  total,  under  which  circumstances  the  eyeballs  are  mo- 
tionless. Inasmuch  as  the  muscles  affected  are  numerous,  almost  every 
possible  combination  of  paralysis  of  different  muscles  has  been  observed. 
It  is  not,  therefore,  necessary  to  specify  the  different  position  of  the 
eyeballs  possible  and  the  different  forms  of  double  vision  possible  in 
ophthalmoplegia. 

Etiology.  —  The  symptoms  may  be  produced  either  by  lesions  in- 
volving the  roots  ol  the  oculomotor  nerves  in  their  passage  through 


Fig.  274. 


Fig.  275. 


Normal  oculomotor  nucleus.  1,  ventral  nu- 
cleus ;  2,  median  nucleus ;  3,  lateral  nucleus ;  4, 
posterior  longitudinal  bundle  ;  5,  6,  nerve  roots ;  7, 
raphe. 


Atrophy  of  oculomotor  nucleus.  1,  ventral 
nucleus ;  la,  Dorsal  nucleus ;  2,  3,  Edinger- 
Westphal  nucleus.     (Siemerling. ) 


the  cerebral  axis,  or  by  lesions  limited  to  their  nuclei.  Lesions  involv- 
ing the  roots  are  very  coromon.  They  are  due,  as  a  rule,  to  hemorrhage 
from,  or  thrombosis  or  embolism  in  the  small  arteries,  and  especially 
in  their  terminal  branches  within  the  crus  cerebri.  These  arteries 
enter  through  the  posterior  perforated  space,  and  are  terminal  arteries. 
Tumors,  spots  of  sclerosis,  or  syphilitic  exudations  in  this  region 
cause  the  same  effect.  The  majority  of  cases  on  record  of  ophthal- 
moplegia externa  which  have  been  examined  pathologically  have  been 
produced  in  this  manner.  Such  lesions  necessarily  cause  a  destruction 
of  other  nerve  tracts  passing  through  the  cerebral  axis  in  addition  to 


OPHTHALMOPLEGIA. 


647 


the  oculomotor  roots.  It  is  from  the  presence  of  symptoms  of  such 
destruction  that  the  pathological  diagnosis  is  to  be  reached.  Thus 
hemiansesthesia,  hemiataxia,  or  hemiplegia  of  various  types  have  been 
observed  in  association  with  partial  ophthalmoplegia.  And  such  an 
affection  of  the  sensory  and  motor  tracts  in  the  cerebral  axis  may  be 
bilateral,  causing  very  extensive  and  complex  groups  of  symptoms  of 
which  the  oculomotor  palsies  may  be  the  least  noticeable,  though  they 
may  be  the  most  important  as  a  guide  to  the  localization  of  the  lesion, 
(See  Figs.  53  to  56.)  The  condition  may  also  be  congenital  ;  Brad- 
burne  ^  has  recorded  an  hereditary  type,  examples  being  seen  in  five 
generations  in  one  family.  Lesions  involving  the  nuclei  of  the  oculo- 
motor nerves  may  be  either  acute  or  chronic,  and  resemble  in  charac- 
ter the  lesions  of  anterior  poliomyelitis. 

Pathology.  —  Acute  ophthal-  Fig.  276. 

moplegia  externa  may  occur  from 
hemorrhage  in  the  nuclei,  from 
thrombosis  or  embolism  in  ter- 
minal vessels  supplying  the 
nuclei,  or  from  inflammatory 
processes,  probably  infectious  in 
origin,  in  the  nuclei.  It  may 
also  be  the  result  of  poisoning 
by  bad  food,  Wernicke  has 
named  this  condition  polioen- 
cephalitis acuta.  (See  page 
538.)  The  lesion  found  is  a 
congestion  of  the  vessels,  exuda- 
tion from  them  of  small  cells 
and  leucocytes  and  serum,  swell- 
ing and  degeneration  of  all  pos- 
sible degrees  in  the  motor  cells, 
with  destruction  of  their  den- 
drites and  axones,  and  conse- 
quent degeneration  in  the  nerve 
roots  and  nerves.  This  lesion 
may  be  limited  to  the  oculomotor  nuclei,  or  it  may  be  extensive  in  the 
cerebral  axis,  affecting  other  cranial  nerves,  in  which  case  the  ophthal- 
moplegia is  only  a  part  of  an  acute  bulbar  paralysis.  A  chronic  atro- 
phy of  the  nuclei  has  also  been  described  and  is  shown  in  Fig.  275. 
These  lesions  resemble  in  every  particular  the  lesions  of  anterior 
poliomyelitis. 

Symptoms.  —  The  onset  of  acute  ophthalmoplegia  is  usually  sudden, 
with  severe  cerebral  symptoms,  vertigo,  vomiting,  headache  and  even 
delirium  and  coma.  The  patient  is  always  somnolent  for  some  days, 
and  occasionally  develops  an  optic  neuritis  as  well  as  the  ocular  palsy. 
In  a  few  cases  other  symptoms  of  bulbar  paralysis  have  developed, 
and  a  fatal  result  followed.     In  many  the  general  symptoms  subside 

'Lancet,  Feb.  3,  1912. 


Patient  suffering  from  chronic  ophthalmoplegia 
externa.  The  wrinkling  of  the  forehead  in  the  effort 
to  open  the  eyes  is  noticeable.  The  external  strabis- 
mus can  be  seen. 


648  THE   CBANIAL  NEBFES  AND    THEIE  DISEASES. 

in  the  course  of  two  weeks,  and  the  only  permanent  symptoms  are  the 
irregular  ocular  paralyses.  In  some  cases  the  symptom  is  the  first  one 
of  a  beginning  myasthenia  gravis.  (See  page  769.)  The  aifection 
may  be  arrested  in  its  progress,  but  usually  leaves  a  chronic  condition 
of  paralysis. 

Chronic  ophthalmoplegia  externa  may  be  the  terminal  result  of  an 
acute  attack,  or  it  may  be  a  primary  condition.  In  a  number  of  cases 
it  has  been  a  congenital  condition.  If  primary  it  begins  gradually  and 
makes  slow  but  steady  progress,  one  muscle  after  another  being  affected. 
It  may  vary  in  intensity,  some  muscles  recovering  as  others  are  in- 
volved. Fig.  276  shows  the  appearance  presented  by  a  patient  suf- 
fering from  this  disease.  In  this  case  the  disease  has  remained 
stationary  for  six  years.  It  may  even  go  on  to  recovery,  the  mus- 
cles gradually  regaining  their  power.  Such  recovery  occurs  in  the 
minority  of  the  cases.  In  the  cases  which  recover  it  is  probable  that 
the  degeneration  is  only  slight  in  degree,  sufficient  to  arrest  the  func- 
tion of  the  cells  for  a  time.  In  this  condition  also  a  general  chronic 
bulbar  paralysis  may  eventually  develop.  It  is  not  uncommon  to  find 
some  defective  action  of  the  orbicularis  palpebrarum  and  frontalis 
muscles  supplied  by  the  facial  nerve,  associated  with  ptosis ;  or  some 
affection  of  the  muscles  of  mastication.  It  is  rare  for  bulbar  palsy  to 
advance  to  the  oculomotor  nerves,  though  it  is  not  rare  for  it  to  develop 
subsequently  to  their  paralysis.  This  is  probably  because  a  fatal  result 
is  reached  early  in  bulbar  palsy.  As  a  curious  confirmation  of  the 
statement  that  chronic  ophthalmoplegia  is  quite  homologous  to  chronic 
anterior  poliomyelitis,  the  case  of  Kalicher  may  be  cited  in  which,  in 
the  course  of  six  months,  the  paralysis  extended  from  the  eyes  to  the 
face,  and  then  to  the  arms  and  to  the  legs  until  total  paralysis  was 
present.  The  lesion  in  this  case  was  chiefly  vascular,  the  vessel  walls 
being  thickened,  showing  fatty  degeneration  and  unusual  dilatations, 
and  hemorrhagic  extravasation  being  found  everywhere  in  the  gray 
matter.  Chronic  ophthalmoplegia  may  be  the  first  symptom  of  tumor 
in  the  corpora  quadrigemina  or  of  multiple  sclerosis. 

Prognosis.  —  The  prognosis  in  partial  ophthalmoplegia  externa  is  not 
as  unfavorable  as  might  be  supposed.  Over  one-half  of  the  patients 
recover.  This  seems  to  show  that  in  the  majority  of  cases  the  lesion 
is  a  vascular  one  and  does  not  cause  a  degeneration  of  the  nuclei.  In 
the  total  chronic  cases  the  prognosis  is  bad. 

Treatment.  —  The  treatment  of  acute  ophthalmoplegia  is  by  the  use 
of  ice  to  the  back  of  the  neck,  aconite,  and  phenacetin,  and  the  admini- 
stration of  hot  baths  daily  to  produce  free  sweating.  At  the  same 
time  brisk  laxatives  and  diuretics  are  to  be  used,  and  if  the  patient  is 
plethoric  leeches  to  the  temples  or  behind  the  ears  are  to  be  applied. 
The  eyes  should  be  kept  in  perfect  rest.  In  chronic  cases  it  is  best  to 
correct  diseases  of  the  bloodvessels  by  the  use  of  heart  stimulants, 
nitroglycerin,  and  iodide  of  potassium,  and  later  to  stimulate  the  nerves 
by  the  use  of  strychnine.  In  cases  where  syphilis  has  been  a  possible 
cause  a  mercurial  treatment,  followed  by  the  free  use  of  iodide,  is  to  be 
prescribed. 


THE  FIFTH  NEBVE:   TBIGEMINAL  NEBFE.  649 

JTHE  FIFTH  NERVE:  TRIGEMINAL  NERVE. 

Anatomy.  —  The  trigeminal  nerve  is  made  up  of  two  parts,  a  motor 
and  a  sensory  portion,  the  latter  being  much  larger  than  the  former. 
The  motor  part  arises  from  a  group  of  cells  lying  in  the  upper  part  of 
the  pons  Varolii  in  the  lateral  portion  of  the  formatio  reticularis.  (Fig. 
277.)  It  passes  outward  through  the  formatio  reticularis,  joins  the 
sensory  root,  lies  under  the  Gasserian  ganglion,  and  leaves  the  skull 
with  the  inferior  maxillary  branch.  It  passes  to  the  muscles  of  mas- 
tication, viz.,  the  masse ters,  temporals,  zygomatic,  digastric,  and  myelo- 
hyoid  muscles,  which  close  the  mouth  and  move  the  lower  jaw.  It 
also  sends  a  filament  to  the  tensor  tympani  muscle.  The  sensory  part 
of  the  nerve  develops  from  the  neurones  in  the  Gasserian  ganglion. 
This  large  ganglion  lies  upon  the  nerve  at  the  side  of  the  pons  (Fig. 
261)  upon  the  base  of  the  skull.  It  has  an  extensive  distribution  upon 
the  face  and  in  the  head,  as  shown  in  Plate  XXV.  (p.  622),  and  all 
sensations  from  these  parts  come  in  through  the  nerve.  The  various 
areas  of  the  skin  of  the  face  connected  with  the  three  great  divisions 
are  shown  in  Plate  IV.  (page  40).  Its  terminal  central  axones  enter 
the  pons  from  the  ganglion  and  terminate  in  a  long  column  of  gray 
matter  which  is  identical  in  its  structure  with  the  substantia  gela- 
tinosa  of  the  spinal  cord,  and  lies  in  the  lateral  part  of  the  for- 
matio reticularis,  from  the  upper  part  of  the  pons  to  the  lowest  part 
of  the  medulla,  where  it  is  continuous  with  the  posterior  horn  of 
the  spinal  cord.  As  these  axones  enter  the  pons  a  few  bifurcate, 
but  the  majority  turn  downward,  and  some  pass  all  the  way  down 
to  the  lowest  level  of  the  medulla,  lying  at  the  side  of  the  column 
of  gray  matter  just  mentioned.  Hence  in  cross-sections  of  the  pons 
and  medulla  this  root  of  the  fifth  nerve  is  cut  across  and  appears 
as  a  semilunar-shaped  white  tract.  (Fig.  55.)  It  should  be  called 
the  descending  root,  as  sensory  impulses  coming  in  pass  down  through 
it  to  reach  the  gray  matter.  But  as  this  cranial  nerve  was  formerly 
described  as  arising  in  the  medulla  and  pons,  like  the  motor  nerves,  it 
was  originally  named  the  ascending  root  of  the  fifth.  The  axones  end 
in  bushes  within  the  substantia  gelatinosa,  and  thence  new  sensory  fila- 
ments arise  which  pass  upward  to  the  cortex.  These  appear  to  decus- 
sate in  the  upper  part  of  the  pons,  as  unilateral  lesions  of  the  pons 
below  the  entrance  of  the  fifth  nerve  produce  anaesthesia  of  the  face  on 
the  side  of  the  lesion  only.  They  then  join  the  general  sensory  tract 
in  the  lemniscus  and  formatio  reticularis  and  pass  to  the  cortex  in  the 
lower  third  of  the  sensory  motor  area.      (See  page  106.) 

There  is  a  long  semilunar-shaped  group  of  cells  which  lies  on  the 
lateral  border  of  the  gray  matter  lining  the  aqueduct  of  Sylvius,  in  the 
substance  of  the  tegmentum,  and  extends  for  four  millimetres  beneath 
the  corpora  quadrigemina  posterior.  These  cells  resemble  the  cells  of 
the  column  of  Clarke  in  the  spinal  cord  and  are  large  and  flask-shaped. 
They  send  axones  downward  in  a  long  descending  nerve  root  which  is 
semilunar  in  cross-section  and  which  enters  the  fifth  nerve  root  oppo- 
site its  exit  at  the  level  of  the  motor  nucleus  of  the  nerve.     Some 


650 


THB   CBANIAL  NERVES  AND   THEIR  DISEASES. 


authorities '  ascribe  motor  functions  to  this  group  of  cells  and  root, 
though  they  were  found  to  be  normal  in  a  case  where  paralysis  of  the 


Fig.  277. 


Scheme  of  the  neurones  making  up  the  fifth  or  trigeminal  nerve.     (Edinger.) 
^  W.  A.  Turner,  Allbutt's  System  of  Medidne,  vol.  VI.,  p.  787. 


THE  FIFTH  NEBVE:   TBIGEMINAL  NEEVE.  651 

muscles  of  jtnastication  was  present  and  where  the  motor  nucleus  of 
the  nerve  was  degenerated.  Other  authorities  ascribe  trophic  functions 
to  this  group  of  cells  and  root,  and  affirm  that  they  are  diseased  in 
cases  of  hemiatrophy  of  the  face.  This  statement  is  made  by  Mendel  ^ 
on  the  basis  of  one  case  only.  Others  still  regard  them  as  a  part  of 
the  sensory  root. 

The  sense  of  taste  is  a  part  of  the  function  of  the  fifth  nerve,  for 
complete  division  of  this  nerve  behind  the  Gasserian  ganglion  suspends 
this  sense.  The  nerves  through  which  this  sense  is  conveyed  are  two  : 
1.  The  anterior  two-thirds  of  the  tongue  sends  sensations  of  taste 
through  the  lingual  nerves  and  chorda  tympani,  which  passes  into  the 

Fig.  278. 


The  facial  nerve  and  its  connections,  within  the  aqueduct  of  Fallopius.  1,  fifth  nerve,  with  the 
Gasserian  ganglion ;  2,  ophthalmic  division  of  the  fifth  nerve ;  3,  superior  maxillary  division  of  the 
fifth  nerve  ;  4,  lingual  nerve  ;  5,  sphenopalatine  ganglion  ;  6,  otic  ganglion  ;  7,  submaxillary  ganglion  ; 
8,  facial  nerve  in  the  aqueduct  of  Fallopius ;  9,  great  superficial  petrosal  nerve ;  10,  small  superficial 
petrosal  nerve  ;  11,  stapedius  branch  of  facial  nerve  ;  12,  branch  of  communication  with  pneumogastric 
nerve ;  13,  branch  of  communication  with  glossopharyngeal  nerve  ;  14,  chorda  tympani.     (Dalton. ) 

Fallopian  canal  in  association  with  the  facial  nerve,  but  leaves  it  at 
the  geniculate  ganglion,  thence  passes  by  the  Vidian  or  great  super- 
ficial petrosal  nerve  to  Meckel's  ganglion  (sphenopalatine),  and  thence 
to  the  superior  maxillary  nerve,  and  thus  into  the  second  trunk  of  the 
trigeminal.  2.  The  posterior  third  of  the  tongue  sends  sensations  of 
taste  through  the  peripheral  branches  of  the  glossopharyngeal  nerve, 
thence  through  the  small  superficial  petrosal  nerve  or  nerve  of  Jacob- 
son  to  the  otic  ganglion,  and  thence  by  the  inferior  maxillary  nerve 
into  the  third  trunk  of  the  trigeminal.      (See  Fig.  278.) 

The  protected  situation  of  all  these  nerves  accounts  for  the  rarity 
of  loss  of  the  sense  of  taste.  I  have  seen  it  once,  however,  in  a  case 
of  severe  chronic  trigeminal  neuralgia  and  anaesthesia  probably  due  to 
destructive  lesion  of  the  root  of  the  nerve.  I  have  also  observed  a 
tf;tal  loss  of  taste  on  one  side  of  the  tongue  and  pharynx  after  division 
of  the  roots  of  the  nerve  and  after  excision  of  the  Gasserian  ganglion 
done  for  the  relief  of  neuralgia  in  several  patients.     Davies  ^  who  has 

'Nonrol.  f:!entralblatt,  vol.  VII.,  p.  14. 

*  The  Function  of  the  Trigeminal  Nerve.     Brain,  1907,  p.  219. 


652  TBE   CBANIAL   NERVES   AND    THEIR  DISEASE iS. 

studied  the  results  of  resection  of  the  Gasserian  ganglion  in  107  eases 
reports  an  impairment  of  taste  in  27  and  a  loss  of  taste  in  34  cases. 
In  46  cases  taste  was  unaflPected. 

Paralysis  of  the  Fifth  or  Trigeminal  Nerve. 

A  lesion  of  the  motor  portion  of  the  trigeminal  nerve  causes  par- 
alysis of  the  muscles  of  chewing.  The  lower  jaw  falls  open  in  such 
cases,  and  hence  both  speech  and  swallowing  are  interfered  with.  The 
patient  usually  supports  the  jaw  with  the  hand,  and  thus  assists  both 
acts.  It  is  to  be  remembered,  however,  that  these  muscles  act  sym- 
metrically on  both  sides  and  together,  and  hence  the  destruction  of 
one  motor  branch  of  the  nerve  does  not  cause  very  great  disability. 
A  little  branch  from  the  motor  portion  passes  to  the  tensor  tympani 
through  the  otic  ganglion,  and  hence,  when  the  nerve  is  paralyzed,  the 
ear  drum  cannot  be  stretched,  and  consequently  deafness  appears. 
This  may  be  accompanied  by  tinnitus  aurium.  When  the  muscles 
are  paralyzed  a  gradual  atrophy  may  occur  which  leads  to  a  sinking 
of  the  temple  and  a  thinness  of  the  face,  over  the  muscles  which  are 
paralyzed,  and  at  the  same  time  reaction  of  degeneration  may  appear 
in  these  muscles.  Miiller  and  Schultze  both  describe  a  slight  difficulty 
in  swallowing  due  to  a  paralysis  of  the  muscles  about  the  hard  palate 
as  having  occurred  in  two  cases.  In  both  these  cases  there  was  a 
slight  deviation  of  the  uvula  toward  the  paralyzed  side.  These  symp- 
toms were  referred  to  a  paralysis  of  the  spheno-stapedius  muscle,  which 
receives  a  branch  from  the  motor  portion  of  the  trigeminal  nerve, 
k  These  symptoms  of  paralysis  of  the  fifth  nerve  are  of  some  interest, 
'as  the  nerve  is  occasionally  divided  by  surgeons  in  the  operation  for 
trigeminal  neuralgia,  and  it  is  chiefly  in  such  cases  that  the  symptoms 
hitherto  described  have  appeared.  It  is  needless  to  add  that  in  such 
cases  the  motor  branch  of  the  trigeminal  nerve  should  be  carefully 
avoided  by  the  surgeon,  so  that  the  paralytic  symptoms  may  be 
prevented. 

Trismus  or  tonic  spasm  of  the  muscles  of  mastication  is  usually  an 
affection  associated  with  general  convulsions  or  is  a  symptom  of  the  in- 
fection of  tetanus  or  lockjaw.  It  is  not  to  be  regarded  as  a  local  dis- 
ease of  the  motor  branch  of  the  fifth  nerve.  Occasionally  a  spasm  of 
these  muscles  upon  one  side  of  the  body  occurs  in  connection  with 
facial  spasm,  and  may  be  regarded  as  a  local  affection  quite  similar  in 
its  origin  to  blepharospasm  and  facial  tic.  Under  these  circumstances 
it  is  usually  a  reflex  manifestation  of  some  sensory  disturbance  about^ 
the  jaw  or  throat,  and  until  the  origin  of  such  reflex  irritation  is  ascer- 
tained and  removed  treatment  will  not  be  successful. 

Tonic  spasm  of  the  masseters  has  been  known  to  occur  after  long- 
continued  stretching  of  the  muscles,  as  in  a  case  reported  by  Peterson, 
where  it  developed  after  a  long  dental  operation.  It  has  been  known 
to  occur  after  a  condition  of  paralysis  in  the  muscles,  as  in  a  case  re- 
ported by  McConnell.  Occasionally  a  fixation  of  the  jaw  and  inability 
to  open  the  mouth  occur  as  a  symptom  in  hysteria. 


THE  FIFTH  NEBVE :   TRIGEMINAL  NEBVE.  653 

Clonic  spasms  of  the  muscles  of  mastication  occur,  causing  a  chat- 
tering of  the  teeth,  as  in  a  malarial  chill  or  in  an  extreme  condition  of 
fear,  or  a  grating  of  the  teeth  if  the  spasm  is  more  slow  and  forcible. 
It  occurs  occasionally  in  paralysis  agitans  and  in  old  age.  Even  in 
health  every  one  has  bitten  the  tongue  or  bitten  the  cheek  unintention- 
ally by  an  unexpected  movement  of  the  jaw.  The  disease  is  of  no 
moment  excepting  in  children,  when  grinding  of  the  teeth  at  night  often 
causes  much  annoyance  to  the  parents  and  sometimes  disturbs  the 
child's  sleep.  This  may  be  due  to  any  disease  which  produces  an  irri- 
tation of  the  nervous  system,  such  as  irritation  of  the  intestine  by 
worms,  irritation  of  the  genitals  by  smegma,  irritation  of  the  throat  by 
adenoids,  or  irritation  of  the  ear  by  wax.  Occasionally  grinding  of 
the  teeth  is  an  early  sign  of  irritation  upon  the  base  of  the  brain  by  a 
beginning  basilar  or  tubercular  meningitis,  and  in  many  cases  of  this 
disease  the  symptom  appears  at  some  time  in  the  progress  of  the  case. 
It  has  been  known  to  occur  as  a  symptom  of  tumor  of  the  base  of  the 
brain.  The  spasm  is  uniformly  bilateral.  In  the  vast  majority  of 
children's  cases,  the  symptom  is  outgrown,  and  it  should  not  cause  any 
alarm  excepting  in  connection  with  other  symptoms  of  tetanus  or  of 
tubercular  meningitis. 

All  these  forms  of  facial  spasm  are  to  be  treated  as  in  facial  tic. 
(See  page  761.) 

Bernhardt  has  recorded  one  case  of  congenital  paralysis  of  the 
trigeminal  nerve  which  was  accompanied  by  abducens  and  facial  palsy, 
and  this  appears  to  be  a  unique  case  in  the  literature.  Injuries  of  the 
fifth  nerve  upon  the  base  of  the  brain  by  tumors,  syphilitic  or  tuber- 
cular exudations  in  the  meninges,  or  fractures  of  the  base  are  very 
rare.  When  the  Gasserian  ganglion  is  involved  herpes  zoster  upon  the 
face  may  appear.  A  neuritis  of  the  trigeminal  nerve  following  cold 
has  not  been  described,  yet  it  is  not  at  all  unlikely  that  very  many 
cases  of  trifacial  neuralgia  (see  page  729)  are  really  due  to  this  cause. 
Hemorrhage  or  softening  in  the  pons  Varolii  or  medulla  at  any  point 
in  the  fifth  nerve  nucleus,  or  tumors,  or  patches  of  sclerosis  in  the  same 
locality  may  cause  paralysis  of  the  nerve.  Thus  the  symptoms  may  be 
present  in  any  form  of  gross  lesion  in  the  cerebral  axis.  It  also  ap- 
pears as  a  symptom  in  some  cases  of  bulbar  paralysis.  In  these  cases 
the  existence  of  other  symptoms  of  bulbar  disease  will  enable  a  diag- 
nosis of  the  site  of  the  lesion  to  be  reached.  It  has  also  been  observed 
in  syringomyelia  when  the  gliomatous  degeneration  extended  into 
the  pons. 

The  symptoms  of  paralysis  of  the  sensory  portion  of  the  trigeminal 
nerve  are  numbness  and  anaesthesia  of  the  face.  In  Plate  XXV.,  the 
exit  of  the  three  great  branches  of  the  sensory  portion  upon  the  face 
are  shown,  and  in  Plate  IV.,  their  distribution  to  the  skin  is  shown. 
The  first  branch  supplies  the  tear  ducts  and  glands,  and  any  loss  of  its 
function  is  followed  by  a  dryness  of  the  eye.  The  second  branch  con- 
trols the  sensation  of  the  mucous  membrane  of  the  nose  and  mouth,  and 
dryness  of  these  parts  accompanies  this  injury.     The  second  and  third 


654 


THE   CBANIAL  NEBVES  AND   THEIB  DISEASES. 


branches  contain  all  the  fibres  concerned  in  taste,  and  hence  lesion  of 
these  parts  results  in  the  loss  of  this  sense.  The  vasomotor  and  trophic 
functions  of  the  fifth  nerve  are  important,  and  trophic  symptoms  are 
exceedingly  common  in  cases  of  any  inj  ury  of  the  fifth  nerve.  Whether 
these  are  due  to  the  suspension  of  sensation  may  be  a  matter  of  discus- 
They  consist  of  ulcerations  of  the  cornea,  falling  of  the  teeth. 


sion. 


changes  in  the  hard  gums  and  in  the  mucous  membrane  of  the  mouth 


Fig.  279. 


Fig.  280. 


Hemiatrophy  of  the  face.  The  condition  de- 
veloped slowly  during  five  years,  and  then  came 
to  a  standstill  at  the  point  shown. 


Hemiatrophy  of  the  face, 
developed    in    two    years. 
Mailhouse. ) 


The  condition  had 
(Kindness   of    Dr. 


and  nose,  with  undue  dryness  and  tendency  to  ulceration  and  the  forma- 
tion of  crusts,  and  also  the  development  of  herpes  zoster,  which  may 
occur  in  the  nasal  and  buccal  cavities  as  well  as  on  the  skin.  The  fifth 
nerve  supplies  the  tongue  with  sensation  as  well  as  with  taste,  and  un- 
noticed injuries  of  the  tongue  in  chewing  are  not  uncommon  when  the 
fifth  nerve  is  anaesthetic.  A  loss  of  the  reflexes  of  the  face  occurs 
in  paralysis  of  the  fifth  nerve,  so  that  winking  does  not  occur  when  the 
eye  is  irritated  ;  tears  do  not  flow,  sneezing  cannot  be  produced  by 
inhalation  of  irritating  substances  or  by  tickling  of  the  nose.  The 
sense  of  smell  is  somewhat  impaired,  partly  on  account  of  the  dryness 
of  the  mucous  membrane  of  the  nose,  and  partly  because  many  irritat- 
ing substances  like  ammonia,  alcohol,  and  substances  which  irritate  the 
mucous  membrane  are  really  perceived  through  the  trigeminal  nerve. 
It  is  possible  that  the  secretion  of  saliva  and  swallowing  upon  the 
paralyzed  side  may  be  suspended. 

The  common  form  of  disease  of  the  fifth  nerve  is  trigeminal  neu- 
ralgia. This  has  been  described  on  page  729  et  seq.  It  is  there  shown 
that  such  neuralgia  is  usually  due  to  a  lesion  of  the  Gasserian  ganglion. 

Hemiatrophy  of  the  Face.  —  A  gradually  advancing  atrophy  of 
one-half  of  the  face  has  been  observed.    It  is  a  very  rare  afiectiou.    It 


TEE  SEVEN TE  NERVE:  TEE  FACIAL  NERVE.  655 

is  characterized  by  a  very  slowly  progressive  atrophy  affecting  the  skin, 
the  subcutaneous  fat,  the  muscles,  and  the  bones  in  about  equal  degree. 
As  a  rule,  this  condition  develops  in  youth  ;  it  has  not  been  observed 
before  the  age  of  ten  years,  and  is  rare  after  the  age  of  twenty  years. 
For  some  time  it  may  escape  notice,  but  gradually  the  asymmetry  of 
the  face  becomes  more  and  more  evident,  the  skin  is  manifestly  thin, 
and  is  often  slightly  pigmented,  the  cheek  sinks  in  upon  the  affected 
side,  the  temple  also  sinks,  and  when  the  disease  is  thoroughly  devel- 
oped, as  in  the  case  shown  in  Fig.  280,  palpation  reveals  the  decided 
atrophy  of  all  the  tissues.  Sometimes  the  hair  on  the  affected  side  falls 
out,  but  this  is  not  a  uniform  result.  Sensibility  is  in  no  way  affected 
and  the  muscles  are  not  paralyzed,  although  as  they  become  thin  they 
are  weaker  than  those  of  the  healthy  side.  After  several  years  the 
condition  appears  to  come  to  a  standstill,  and  the  disease  does  not  lead 
to  a  fatal  termination.  Occasionally  the  disease  makes  rapid  progress, 
as  in  the  patient  shown  in  Fig.  280.  In  this  man  the  disease  had  been 
in  progress  only  two  years.  He  suffered  from  spasms  of  the  masseters 
and  much  pain  in  the  face,  but  had  no  anaesthesia  and  no  other  symp- 
toms of  intracranial  disease.  The  pathology  of  this  disease  is  unknown. 
There  is  no  known  treatment. 


THE  SEVENTH  NERVE:  THE  FACIAL  NERVE. 

The  facial  nerve  is  the  great  motor  nerve  of  the  face.  It  arises  from 
a  large  nucleus  which  lies  deep  in  the  formatio  reticularis  of  the  pons 
Varolii.  (Fig.  77.)  From  this  nucleus  the  fibres  pass  backward  and 
inward  toward  the  floor  of  the  ventricle,  curve  about  the  nucleus  of  the 
sixth  nerve,  forming  an  angle  known  as  the  knee  of  the  facial,  and  then 
turn  outward,  traverse  again  the  formatio  reticularis  of  the  pons,  and 
make  their  exit  in  the  groove  between  pons  and  medulla  in  close  prox- 
imity to  the  eighth  nerve.  It  then  enters  the  internal  auditory  foramen 
of  the  j)etrous  portion  of  the  temporal  bone  and,  after  passing  through 
the  bony  canal  called  the  aqueduct  of  Fallopius,  issues  from  the  stylo- 
mastoid foramen  upon  the  face  just  under  the  lobe  of  the  ear.  It  there 
divides  into  many  branches  which  supply  the  various  muscles  of  the 
face.  (Plate  XXV.)  Just  before  entering  the  auditory  foramen  a 
ganglion  is  seen  to  lie  on  the  nerve.  This  is  the  geniculate  ganglion. 
As  motor  nerves  do  not  possess  such  ganglia,  it  is  evident  that  at  this 
point  some  sensory  fibres  must  be  associated  with  the  motor  fibres. 
These  are  partly  sensory  fibres  belonging  to  the  chorda  tympani  —  a 
sensory  nerve  of  taste  which  joins  the  seventh  nerve  in  the  Fallopian 
canal  and  leaves  it  at  the  ganglion  to  pass  by  way  of  the  Vidian  or 
superficial  petrosal  nerve  to  the  superior  maxillary  branch  of  the  fifth ; 
and  partly  sensory  nerves  of  touch  supplying  the  auditory  canal  and 
the  skin  just  in  front  of  the  ear,  as  recently  shown  by  J.  R,  Hunt.' 
Occasionally,  when   inflammatory  processes   attack  the   seventh   nerve 

'  Ilcqjetic  Inflammations  of  the  Geniculate  Ganglion,  Jour.  Nerv.  and  Ment.  Dis., 
Feb.,  1907,  alsoAmer,  Jour,  Med.  Sci.,  Aug.,  1908. 


656  THE   CEANIAL  NEEVES  AND   THEIE  DISEASES. 

in  the  canal  where  this  sensory  nerve  lies  beside  it,  its  fibres  are 
involved,  and  then  the  sense  of  taste  is  lost  on  the  anterior  two-thirds 
of  the  tongue.  When  the  sensory  fibres  are  involved  neuralgic  pain 
may  be  felt  just  in  front  of  the  ear  or  in  the  auditory  canal  or  herpes 
may  appear  in  these  places.  A  small  nerve  lies  at  the  side  of  the  facial 
between  the  ganglion  and  the  pons  and  enters  it  between  the  seventh 
and  eighth  nerves.  This  is  the  nerve  of  Wrisberg.  It  is  probably  a 
sensory  nerve.  It  passes  toward  the  glossopharyngeal  nucleus,  where 
it  ends.  It  has  been  thought  to  be  a  vestige  of  the  extensive  sensory 
facial  branch  found  in  fishes.  Fig.  278  shows  the  various  branches  of 
the  nerve  in  its  course.  The  successive  invasion  of  each  branch  from 
without  inward  by  a  progressive  neuritis  enables  oile  to  locate  the 
process  of  a  neuritis  in  the  nerve,  as  will  be  indicated  in  the  section 
on  diagnosis.  The  facial  nucleus  is  closely  joined  to  many  other 
cranial  nerve  nuclei  by  association  fibres.  One  distinct  branch  from 
the  oculomotor  nucleus  joins  the  facial  nucleus  and  sends  some  fila- 
ments directly  into  the  nerve  trunk.  Thus  an  anatomical  basis  for 
the  many  reflex  and  automatic  acts  in  which  the  muscles  of  the  head 
and  face  take  part  is  secured.  Thus  in  the  acts  of  winking,  breathing, 
in  motions  attendant  upon  sensations  of  sight,  and  sound,  and  taste, 
and  smell,  as  well  as  in  the  various  bodily  sensations,  changes  of  the 
facial  expression  are  constantly  seen  which  are  automatic.  Mental 
states  are  also  reflected  in  the  face  unconsciously,  and  this  is  secured 
by  an  intimate  association  of  the  facial  nuclei  and  the  centres  in  the 
optic  thalamus.  Any  disease  in  the  pons,  such  as  hemorrhage,  soften- 
ing, sclerosis,  or  tumor  will  suspend  these  reflex  acts.  The  facial  nu- 
cleus is  also  directly  joined  to  the  cerebral  cortex,  receiving  impulses 
from  the  lower  third  of  the  motor  area  by  a  tract  which  traverses  the 
knee  of  the  internal  capsule  and  lies  in  the  median  part  of  the  motor 
tract  in  the  crus  cerebri.  Its  fibres  leave  the  motor  tract  in  the  pons, 
pass  backward  in  the  raph6,  where  they  partially  decussate  with  those 
of  the  opposite  side,  and  end  in  the  nucleus.  Thus  each  hemisphere 
is  joined  to  both  nuclei.  Lesions  in  the  course  of  this  tract  arrest 
voluntary  movements  of  the  face,  but  do  not  suspend  the  reflex  acts. 
(See  Figs.  48  and  49  and  page  104.) 

Facial  Paralysis.  Pathology.  —  As  facial  paralysis  usually  recovers, 
but  few  pathological  observations  are  available.  Markowski^  found 
in  a  case  of  facial  palsy  due  to  cold  that  the  nerve  was  normal  from 
the  medulla  to  the  geniculate  ganglion,  but  from  the  ganglion  down- 
ward into  the  periphery  there  was  a  well-marked  degeneration.  The 
neuritis  was  parenchymatous  and  not  interstitial.  The  peripheral 
branches  contained  many  fully  degenerated  fibres,  a  few  remaining 
globules  of  myelin  in  a  state  of  disintegration,  and  many  little  fat 
cells.  Some  of  the  branches  contained  normal  as  well  as  degenerated 
nerve  fibres,  and  very  few  degenerated  fibres  were  found  in  the  super- 
ficial petrosal  and  stapedius  nerves.  The  entire  process  was  a  pure 
degenerative  neuritis  without  any  participation  of  the  neurilemma.    A, 

lArch.  f,  Psjch.,  Bd,  xxiii.,  p.  367. 


TEE  SEVEN TK  NEBVE :  THE  FACIAL  NEBVE.  657 

similar  lesion  was  found  by  Darkschewitch  and  Tichonow  ^  and  by 
G.  Alexander.^  Dejerine  and  Theohari  ^  have  examined  a  nerve  after 
facial  palsy  and  found  a  well-marked  degeneration  in  all  the  branches 
of  the  nerve,  though  the  lower  branch  was  more  affected  than  the 
others.  There  was  no  evidence  of  interstitial  changes.  While  the 
root  of  the  facial  nerve  showed  no  degeneration,  examination  of  the 
nucleus  by  the  Nissl  stain  demonstrated  a  disappearance  of  the  fine  Nissl 
bodies  and  a  shining  appearance  such  as  is  found  in  cells  whose  function 
is  suspended.     This  was  also  seen  by  Flatau^  in  another  case. 

Etiology.  —  While  facial  palsy  due  to  maldevelopment  in  foetal  life 
has  been  occasionally  described,  the  majority  of  congenital  cases  are 
due  to  pressure  exerted  upon  the  facial  nerve  during  delivery.  This 
pressure  may  be  produced  by  forceps  applied  to  the  head  or  by  a  mis- 
placed hand  or  shoulder  pressing  upon  the  face  during  labor.  Bern- 
hardt^ has  recorded  a  number  of  such  cases,  and  every  obstetrician  has 
met  with  them.  In  some  of  these  cases  the  orbicularis  oris  has  not 
been  paralyzed.  Children  occasionally  suffer  from  facial  paralysis  fol- 
lowing a  blow  on  the  ear  or  an  attack  of  the  mumps.  Tubercular 
swelling  of  the  glands  of  the  neck,  causing  pressure  on  the  nerve,  is 
an  occasional  cause  of  facial  palsy  in  children,  but  the  most  common 
cause  in  children  is  otitis  media  producing  an  inflammation  of  the 
nerve  in  the  Fallopian  canal  adjacent  to  the  inflamed  ear.  This  also 
is  a  cause  of  facial  palsy  in  the  adult  in  7  per  cent,  of  the  cases.  In- 
tracranial disease,  such  as  tumors  of  the  brain,  or  exudations  upon  the 
base  of  the  brain,  or  syphilitic  inflammation  of  the  nerve  trunk  within 
the  cranium  compressing  the  nerve,  may  produce  paralysis.  Fracture 
at  the  base  of  the  skull  and  caries  of  the  petrous  portion  of  the  tem- 
poral bone,  involving  the  nerve  in  its  course  have  also  produced  facial 
palsy.  The  usual  cause  of  facial  paralysis  is  supposed  to  be  exposure 
to  cold.  Philip  ascribed  facial  palsy  to  this  cause  in  72  per  cent,  of 
the  cases.  Remak  ®  found  this  a  cause  in  45  per  cent,  of  his  cases,  but 
Thomas  ^  describes  it  as  a  cause  in  only  one-quarter  of  his  cases.  In 
many  of  my  cases  a  history  of  a  draught  upon  the  side  of  the  face  has 
been  obtained,  and  it  seems  probable  that  this  may  produce  such  con- 
gestion of  the  nerve  as  to  lead  to  facial  paralysis.  The  disease  does 
not  occur  more  frequently  in  winter  than  in  summer,  but  Bernhardt* 
found  it  more  common  in  women  than  in  men  and  more  common  in 
men  who  were  shaven  than  in  those  who  wore  a  beard.  The  Germans 
call  all  cases  due  to  exposure  to  cold  "  rheumatic,"  but  there  appears 
to  be  no  distinct  relation  between  facial  palsy  and  rheumatism.  In 
very  many  cases  no  ostensible  cause  can  be  ascertained. 

The  most  plausible  explanation  of  the  occurrence  of  facial  palsy  is 
that  offered  by  Philip  ^  who  believes  that  a  congenital   narrowing  of 

'Neurol.  Centralbl.,  1893,  p.  329.  2 Arch.  f.Psych.,  xxxv.,  p.  72. 

3  J.e  Somaine  Medicale,  1897,  p.  453.  *  Zeitsch.  f.  klin.  Med.,  1897. 

•■■'Berliner  klin.  Woch.,  1899,  No.  31.  «Eemak,  Neurol.  Centralbl.,  1899. 

'  Tliomas,  .Jonr.  Amer.  Med.  Assoc,  1898,  No.  21.      ^Dissert.,  Bonn,  1890. 
« Berliner  klin.  Woch.,  1888,  No.  7,  and  1892,  No.  30. 

42 


658  TEE  CEANIAL  NEEVES  AND   THEIB  DISEASES. 

the  stylomastoid  foramen  in  certain  persons  predisposes  them  to  a  com- 
pression of  the  nerve  upon  any  slight  congestion  of  its  trunk.  It  is 
probable  that  an  unnaturally  large  nerve  lying  in  a  normal  foramen 
might  produce  some  such  tendency.  A  certain  number  of  cases  of 
facial  palsy  develop  during  the  night,  when  pressure  upon  the  pillow, 
combined  with  venous  stasis  in  the  canal,  might  produce  a  pressure  in 
such  individuals.     One  attack  of  facial  palsy  predisposes  to  another, 

Fig.  281.  Fig.  282. 


Facial  paralysis  of  the  right  side.    Attempt  to  Facial  paralysis  of  the  risbt  side.    Attempt  to 

raise  the  eyebrows.  close  the  eyes. 

and  in  7  per  cent,  of  the  cases  there  is  a  recurrence  of  the  disease. 
Facial  paralysis  may  accompany  any  form  of  multiple  neuritis,  and  is 
then  often  bilateral.  I  have  seen  such  cases  in  adults  suffering  from 
alcoholic  multiple  neuritis,  also  from  severe  lead  palsy,  and  several 
cases  in  children  after  diphtheria. 

Symptoms.  —  The  symptoms  of  facial  paralysis  are  very  noticeable. 
There  is  a  total  paralysis  of  all  tlie  muscles  upon  one  side  of  the  face, 
including  the  forehead  and  the  muscles  which  close  the  eye.  The 
forehead  is  smooth,  its  wrinkles  are  no  longer  evident,  and  the  eye- 
brow is  immovable.  Attempts  at  frowning  are  unsuccessful  on  the 
paralyzed  side,  the  eye  remaining  open,  and  when  the  patient  is  told  to 
close  it  he  merely  rolls  the  eyeball  upward,  leaving  the  sclera  visible. 
He  is  not  aware,  however,  that  he  has  not  closed  the  eye.  The  reflex 
act  of  winking  is  abolished,  and  the  consequent  exposure  of  the  eye- 
ball often  leads  to  conjunctivitis.  As  the  lower  lid  is  not  approxi- 
mated to  the  eyeball,  tears  are  no  longer  directed  into  the  tear  duct, 
and  may  run  down  upon  the  cheek.  The  nasolabial  fold  of  the  cheek 
is  flat,  the  corner  of  the  mouth  hangs  down,  and  there  is  a  total  lack 
of  the  normal  play  of  facial  expression  during  emotion  or  in  conver- 
sation. The  flat,  expressionless  countenance  gives  a  mask-like  appear- 
ance to  the  face  and  attracts  attention  at  once.  In  the  act  of  respira- 
tion the  ala  of  the  nose  is  not  dilated  and  the  cheek  often  flaps.     All 


TEE  SEVENTH  NEBVE :  THE  FACIAL  NEBVE.  659 

voluntary  motions  about  the  mouth  are  suspended,  hence  whistling, 
blowing/  pursing  of  the  lips,  drinking,  or  moving  the  food  about  in 
the  mouth  during  the  act  of  chewing  are  imperfectly  performed,  and 
the  pronunciation  of  labials  is  often  indistinct.  The  patient  often 
bites  the  cheek  in  the  act  of  chewing,  as  the  buccinator  fails  to  act. 
Those  who  can  move  the  ears  lose  this  power.  Occasionally,  when  the 
mouth  is  opened,  the  tongue  appears  to  protrude  toward  the  healthy 
side.  This  deviation  is  often  apparent  rather  than  real,  the  mouth 
being  opened  unevenly.  If,  however,  the  deviation  of  the  tongue  is 
real,  it  is  due  to  a  paralysis  of  the  muscles  attached  to  the  hyoid 
bone,  allowing  this  to  fall  upon  the  paralyzed  side.  Occasionally  in 
facial  palsy  a  deviation  of  the  uvula  has  been  noticed.  One  side  of 
the  uvula  may  be  elevated,  and  it  is  then  turned  away  from  the 
paralyzed  side,  or  it  may  fall  toward  the  paralyzed  side.  This  symp- 
tom is  a  rare  one,  and  I  have  seen  it  only  occasionally.  While 
mentioned  by  Erb  and  others,  it  has  been  denied  by  Horsley  and 
Beevor,  who  consider  it  a  mere  accident  and  not  a  symptom  of  facial 
palsy.  It  is  quite  certain  that  the  motor  innervation  of  the  palate 
comes  from  the  vago-accessorius.  Schultze  ^  has  found  such  a  deviation 
in  but  one  case  in  55.  In  some  cases  of  facial  palsy  the  sense  of  taste 
is  affected  upon  the  anterior  two-thirds  of  the  tongue  upon  the 
paralyzed  side.  This  may  be  present,  however,  without  attracting  the 
notice  of  the  patient.^  It  can  be  discovered  by  applying  sweet,  salty, 
or  bitter  solutions  to  the  tongue  with  a  fine  camel's-hair  brush  or  by 
passing  a  galvanic  current  through  the  tongue,  when  the  galvanic  taste 
is  no  longer  produced.  This  symptom  is  only  present  when  the  lesion 
in  the  nerve  is  a  deep  one  and  involves  that  part  of  it  within  the 
Fallopian  canal,  where  the  chorda  tympani  joins  it.  An  unusual 
acuteness  of  hearing  and  tinnitus  aurium  ar^  occasionally  complained 
of.  They  are  due  to  the  tension  of  the  drum  membrane  consequent 
upon  paralysis  of  the  stapedius  muscle.  Under  these  circumstances 
the  lesion  is  very  deep  in  the  Fallopian  canal  or  upon  the  base  of  the 
brain.  If  the  paralysis  is  an  accompaniment  of  middle-ear  disease, 
or  if  the  auditory  nerve  is  affected  upon  the  base  of  the  brain,  where 
it  lies  in  close  apposition  to  the  facial  nerve,  deafness  and  vertigo  will 
be  associated  with  paralysis.  The  patients  sometimes  complain  of 
pain  behind  the  ear  at  the  time  of  the  onset  of  the  affection,  and  are 
often  tender  to  pressure  at  the  exit  of  the  nerve.  They  sometimes 
have  pain  in  the  side  of  the  neck,  and  occasionally  herpes  appears 
below  the  ear  or  on  the  side  of  the  neck. 

Facial  palsy  occasionally  develops  simultaneously  on  both  sides. 
This  is  very  rare,  but  I  have  seen  several  cases.  In  one  case  the  left 
side  was  affected  three  days  after  the  right  side.  The  absence  of  other 
bulbar  symptoms  and  the  recovery  in  these  cases  proved  them  to  be 
peripheral. 

After  the  paralysis  has  been  present  for  some  little  time  stiffness  in 

'  Lehrbuch  der  Nervenkrankheiten,  Bd.  i.,  p.  5. 
''Lermoyez,  M.,  Annales  des  Mai.  de  F  Oreille,  1899,  p.  564, 


660  THE   CBANIAL  NERVES  AND    THEIB  DISEASES. 

the  muscles  of  the  face  is  often  felt,  but  it  is  rarelj  associated  with 
neuralgic  pain.  If  the  case  does  not  go  on  to  recovery  the  facial 
muscles  atrophy  and  contract,  producing  a  very  distressing  sense  of 
stiffness  in  the  side  of  the  face.  This  contracture  of  the  muscles  may 
restore  the  original  lines  of  the  face  and  the  original  facial  expression, 
and  may  even  elevate  the  corner  of  the  mouth  slightly,  so  that  at  the 
first  glance  the  paralysis  may  be  thought  to  be  in  the  well  side,  but 
any  attempt  at  voluntary  movement  will  demonstrate  the  immobility 
of  the  truly  paralyzed  and  contractured  muscles. 

The  electrical  contractility  in  the  facial  muscles  is  usually  altered. 
In  some  cases  the  faradic  contractility  is  preserved  and  the  galvanic 
contractility  is  slightly  increased,  so  that  the  muscles  respond  to  a 
weaker  current  than  those  upon  the  normal  side.  In  these  cases 
recovery  occurs  within  three  or  four  weeks.  In  cases  of  medium 
severity,  however,  there  is  a  partial  reaction  of  degeneration.  The 
faradic  contractility  is  very  much  diminished  or  even  lost,  the  galvanic 
contractility  is  heightened,  so  that  only  a  weak  current  is  necessary  to 
produce  contractility,  and  the  cathode  closing  contraction  remains 
greater  than  the  anode  closing  contraction.  In  the  course  of  six  weeks 
the  galvanic  contraction  becomes  normal ;  after  two  months  the  faradic 
contractility  returns,  and  these  patients  recover  in  between  two  and 
three  months.  In  severe  and  permanent  cases  there  is  a  total  reaction 
of  degeneration,  a  complete  loss  of  faradic  contractility,  and  a  gradual 
reduction  in  the  galvanic  contractility,  so  that  very  strong  currents  are 
necessary  to  produce  any  effect,  and  here  the  anode  closing  contrac- 
tion is  greater  than  the  cathode  closing  contraction,  and  the  opening 
contractions  may  be  equivalent  to  the  closure  contractions.  Such  a 
reaction  of  degeneration  may  remain  for  a  whole  year,  and  even  then 
recovery  may  ensue.  In  fact,  Remak  records  a  case  of  recovery  after 
three  years'  continued  reaction  of  degeneration.  The  electrical  reac- 
tions, therefore,  are  important  not  only  as  symptoms  but  as  prognostic 
indications  in  this  affection. 

The  course  of  the  case  varies  in  different  conditions  in  accordance 
with  the  severity  of  the  initial  lesion.  In  the  majority  of  cases  all 
symptoms  develop  within  a  few  hours  of  the  onset,  and  remain  prac- 
tically stationary  for  from  two  to  three  weeks,  during  which  time  no 
voluntary  movement  at  all  is  possible.  In  the  lighter  cases  the  symp- 
toms then  gradually  subside,  and  recovery  results  in  four  to  six  weeks. 
In  more  severe  cases  such  recovery  does  not  occur  under  six  months. 
In  the  severer  type  a  year  may  ensue  before  any  relief  is  evident,  and 
in  a  few  cases  no  recovery  occurs,  the  condition  becoming  permanent, 
the  muscles  becoming  contractured,  and  very  often  being  the  seat  of 
twitching  and  spasms  which  are  annoying.  It  is  in  these  severer  cases 
that  conjunctivitis,  unless  prevented  by  antiseptic  alkaline  lotions  to  the 
eye,  occurs  as  a  complication. 

Diagnosis. — The  diagnosis  of  facial  palsy  presents  no  difficulties  as 
the  symptoms  are  very  distinct.  But  facial  paralysis  may  occur  not 
only  from  a  lesion  of  the  nerve  trunk,  but  from  a  lesion  of  the  brain 


TEE  SEVENTH  NEBVE:  THE  FACIAL  NERVE.  661 

at  any  poipt  in  the  motor  tract  between  the  cortex  and  the  facial  nerve 
nucleus.  Facial  paralysis  produced  by  a  cortical  lesion  or  by  a  lesion 
in  the  motor  tract  above  the  level  of  the  nucleus  of  the  facial  nerve  is 
less  complete  than  facial  paralysis  from  lesion  in  the  nerve,  and  is  not 
attended  by  complete  paralysis  of  the  act  of  closing  the  eye.  Winking 
is  always  possible,  and  the  muscles  of  the  forehead  are  less  commonly 
involved.  In  emotional  excitement  the  facial  muscles  act  normally  so 
that  the  play  of  expression  occurs  even  though  voluntary  movement  is 
suspended.  In  this  form  also  there  are  no  changes  in  the  electrical 
contractility  in  the  paralyzed  muscles.  While  it  is  possible  that  an 
isolated  facial  paralysis  from  cortical  or  subcortical  disease  may  occur, 
such  paralysis  is  usually  the  accompaniment  of  hemiplegia,  and  hence 
is  not  likely  to  be  mistaken  for  paralysis  due  to  a  lesion  in  the  course 
of  the  trunk  of  the  nerve. 

Facial  paralysis  due  to  a  lesion  of  the  facial  nucleus  in  the  pons 
Varolii  is  almost  uniformly  attended  by  other  symptoms  of  pons  dis- 
ease, such  as  (a)  a  paralysis  of  the  sixth  nerve,  which  lies  adjacent  to 
the  seventh  nerve  nucleus ;  or  (6)  an  implication  of  the  motor  or  sen- 
sory tract  passing  to  the  opposite  arm  and  leg,  which  pass  close  by  the 
situation  of  the  facial  nucleus,  thus  producing  alternating  hemiplegia 
or  hemianaesthesia  (Fig.  49),  or  (c)  the  symptoms  of  bulbar  paralysis ; 
or  (d)  the  symptoms  of  muscular  dystrophy.  Gowers  has  reported  a 
case  of  isolated  infantile  palsy  of  the  face  analogous  to  infantile  spinal 
paralysis.  In  this  case  the  orbicularis  oris  escaped.  The  character- 
istics of  the  facial  paralysis  when  the  nucleus  is  aifected  are  the  same 
as  those  when  the  nerve  trunk  is  diseased,  but  there  is  no  affection  of 
taste. 

The  diagnosis  of  disease  upon  the  base  of  the  brain  between  the 
exit  of  the  facial  nerve  from  the  pons  and  its  entrance  into  the  inter- 
nal auditory  foramen  can  only  be  made  from  the  presence  of  other 
symptoms  of  intracranial  disease  such  as  are  due  to  pressure  upon  the 
side  of  the  pons  or  to  implication  of  other  cranial  nerves,  notably  the 
sixth  or  the  eighth.  The  diagnosis  of  the  location  of  the  lesion  in  the 
course  of  the  nerve  within  the  Fallopian  canal  is  not  difficult.  If  the 
nerve  is  affected  near  the  geniculate  ganglion  or  prior  to  the  giving 
off  of  the  little  branch  to  the  stapedius  muscle,  tinnitus  aurium  and 
acuteness  of  hearing  will  be  noticeable  symptoms.  They  will  not  be 
present,  however,  if  the  lesion  is  nearer  to  the  exit  of  the  nerve  than 
the  point  from  which  this  branch  arises.  If  the  nerve  is  injured  in 
the  Fallopian  canal  between  the  point  at  which  it  is  joined  by  the 
chorda  tympani  and  the  point  at  which  this  nerve  is  given  off,  a  loss 
of  taste  in  the  anterior  two-thirds  of  the  tongue  will  be  present.  Such 
a  loss  of  taste  does  not  occur  if  the  lesion  is  near  to  the  exit  of  the 
nerve  at  the  stylomastoid  foramen  or  is  in  the  face  after  its  exit  from 
this  bony  canal.     (See  Fig.  278.) 

Prognosis.  —  The  prognosis  in  facial  paralysis  depends  upon  the  cause 
and  the  severity  of  the  attack.  In  lesions  of  the  nucleus  within  the  brain 
or  of  the  trunk  on  the  base  of  the  brain,  and  in  lesions  of  the  nerve 


Q62  TBE   CHANIAL  NESrES   AND   THEIB  DISEASES. 

following  otitis  media,  recovery  is  rare.  When  the  cause  is  cold  or  is 
unknown,  the  majority  of  patients  get  well.  The  severity  can  be  deter- 
mined with  a  fair  degree  of  accuracy  by  the  electrical  examination, 
as  already  described,  and  this  examination  often  aids  the  prognosis. 

Treatment.  —  If  the  patient  is  seen  within  two  or  three  days  of  the 
onset  of  the  disease  it  is  well  to  apply  a  small  blister  over  the  exit 
of  the  nerve  beneath  the  ear.  If  the  cause  of  the  affection  can  be 
ascertained  and  can  be  removed,  especially  if  it  is  otitis  media,  this 
should  be  done,  but  in  the  majority  of  cases  it  is  impossible  to  ascer- 
tain a  cause,  and  hence  the  treatment  must  be  symptomatic.  It  should 
consist  of  massage  of  the  face,  done  by  the  patient  with  the  tips  of  his 
fingers  frequently  during  the  day,  and  this  can  be  aided  by  placing  one 
finger  in  the  mouth  and  so  compressing  and  kneading  the  muscles 
between  the  two  fingers.  The  muscles  should  be  treated  with  elec- 
tricity daily,  that  current  being  used  which  produces  a  contraction. 
But  it  is  not  advisable  to  continue  electrical  treatment  longer  than  six 
months,  for  in  the  cases  which  last  beyond  this  time  the  electrical 
stimulation  favors  the  development  of  contractures.  When  contrac- 
tures occur  in  the  chronic  cases  warm  applications  of  water  and  mas- 
sage may  alleviate  the  sensation  of  stiffness.  It  is  useless  to  apply  the 
faradic  current  when  the  muscles  do  not  respond  to  it.  Iodide  of 
potassium  in  five-grain  dose  after  meals  and  small  doses  of  strychnine 
have  been  thought  by  some  writers  to  hasten  recovery. 

In  congenital  cases  which  show  no  tendency  in  the  course  of  two 
years  to  recover,  and  in  chronic  cases  which  have  remained  in  a  sta- 
tionary condition  for  a  year  it  is  possible  to  resort  to  surgical  treat- 
ment. The  facial  nerve  is  cut  at  its  exit  from  the  stylo-mastoid  fora- 
men ;  the  hypoglossal  nerve  is  exposed  behind  the  internal  jugular 
vein  and  above  the  level  of  the  posterior  belly  of  the  digastric  muscle 
and  the  facial  nerve  is  implanted  into  a  longitudinal  slit  in  the  hypo- 
glossal. The  junction  of  the  nerves  is  wrapped  in  Cargile  membrane 
so  that  connective  tissue  will  not  interfere  with  the  union  of  the  nerves. 
The  result  is  that  nerve  fibres  grow  from  the  twelfth  into  the  sheath  of 
the  seventh  eventually  producing  a  regeneration  of  that  nerve  and  return 
of  control  in  the  muscles  of  the  face.  There  is  usually  a  coincident 
paralysis  of  the  tongue  which  gives  little  or  no  inconvenience.  Taylor 
and  Pierce  Clark  have  reported  some  success  with  this  operation.^ 

THE  EIGHTH  NERVE:    THE  AUDITORY  NERVE. 

The  eighth  or  auditory  nerve  consists  of  two  distinct  portions,  the 
cochlear  and  vestibular  nerves,  which,  though  joined  in  the  trunk,  are 
separate  from  one  another  both  in  their  peripheral  termination  and  in 
their  central  nuclei    and  also  as  to  function. 

I.  The  cochlear  part  of  the  nerve  is  the  nerve  of  hearing.  Like  all 
nerves  of  special  sense,  it  has  a  special  mechanism  in  the  periphery,  the 
organ  of  Corti,  which  is  a  sort  of  natural  harp,  its  strings  vibrating  to 

^  See  Journal  of  the  American  Medical  Association,  March  24,  1906. 


TKn  niGETE  NEBVE:  THE  AUDlTO^Y  I^EW^.  663 

different  tones,  each  string  being  in  reality  an  epithelial  cell  connected 
with  a  ner'^e  filament  whose  neurone  body  lies  within  the  cochlea  and 
sends  a  central  filament  into  the  auditory  nerve.  The  terminations  of 
this  nerve  in  the  nuclei  of  the  medulla  and  their  connections  with  the 
brain  have  already  been  described.     (Page  128.) 

Deafness.  —  Deafness  is  usually  due  to  some  disease  within  the  ear 
affecting  the  peripheral  fibres  of  the  nerve.  But  affections  of  the 
acoustic  nerve  produce  disturbances  of  hearing. 

Deafness  due  to  disease  of  the  cochlear  fibres  of  the  acoustic  nerve 
is  distinguished  from  deafness  in  the  outer  or  middle  ear  by  the  loss 
of  power  of  perceiving  sound  through  the  bones  of  the  head.  In  a 
normal  condition  the  sound  of  the  tuning-fork  can  be  heard  both  when 
held  to  the  ear  and  when  applied  to  the  teeth,  or  forehead,  or  petrous 
portion  of  the  temporal  bone.  The  sound  of  the  tuning-fork  is  nor- 
mally heard  longer  when  the  vibrations  are  conveyed  through  the  air 
than  when  they  are  conveyed  through  the  bone.  If  there  is  disease 
of  the  middle  ear,  producing  deafness,  the  tuning-fork  is  heard  through 
the  bone,  but  not  when  held  near  to  the  ear.  If  the  deafness  is  due 
to  disease  of  the  auditory  nerve  the  tuning-fork  is  no  longer  heard  when 
applied  to  the  bone,  or  is  heard  less  distinctly  than  when  held  outside 
of  the  ear.  In  auditory  nerve  deafness  the  higher  pitched  sounds  are 
less  distinctly  heard  than  low  notes.  This  may  be  tested  by  the  Galton 
whistle.  In  auditory  nerve  deafness  hearing  is  not  increased  in  a  noise 
as  it  is  when  disease  lies  in  the  middle  ear.  In  auditory  nerve  deaf- 
ness inflation  of  the  ear  by  Politzer's  method  does  not  improve  the 
hearing. 

In  auditory  nerve  deafness  changes  to  the  electrical  reactions  are 
also  present,  which  are  not  found  in  deafness  from  otitis  media. 
These  tests  usually  produce  vertigo,  and  hence  are  difficult  of  appli- 
cation, but  in  the  normal  state  the  cathode  closure  produces  a  slight 
sound  which  is  distinctly  louder  than  that  caused  by  the  anode  closure. 
If  the  auditory  nerve  is  diseased  its  reaction  is  changed.  The  anode 
closure  sound  is  greater  than  the  cathode  closure  sound,  and  the  sound 
may  be  heard  when  the  current  is  broken.  In  the  majority  of  cases 
of  auditory  nerve  deafness  the  vestibular  nerve  is  also  affected  and 
vertigo  is  associated  with  the  deafness. 

Deafness  from  acoustic  nerve  disease  or  degeneration  occurs  occa- 
sionally primarily.  It  may  be  a  congenital  condition  due  to  some 
defective  development  either  in  the  nerve  or  in  the  organ  of  Corti, 
under  which  circumstances  the  child  is  never  able  to  hear  and  never 
learns  to  speak.  This  is  the  common  cause  of  deaf-mutism.  It  is  an 
interesting  fact  that  such  deaf-mutes  are  not  made  giddy  by  ordinary 
methods  and  do  not  suffer  from  seasickness.  In  other  cases  an  atrophy 
of  the  acoustic  nerve  appears  to  be  due  to  a  congenital  defect  in  its 
power  of  nutrition,  and  deafness  comes  on  in  early  adult  life.  This  is 
a  peculiarly  hereditary  affection,  many  members  of  the  same  family 
being  subject  to  the  disease.  Thus  I  have  known  of  one  large  family 
in  which  it  has  appeared  through  four  generations,  each  generation 


664  TEE   CSANIAL  NERVES  AND   TEEIB  DISEASES. 

having  had  several  members  who  developed  the  affection.  I  know 
another  family  in  which  four  of  seven  sons  have  inherited  the  deafness 
of  their  mother,  who  had  inherited  her  deafness  from  her  father. 
They  have  several  uncles  and  cousins  who  are  deaf.  Such  cases  must 
be  due  to  a  defective  vitality  in  the  nerve  trunk,  and  are  to  be  regarded 
as  primary  auditory  atrophy,  the  nerve  dying  before  the  rest  of  the 
body,  just  as  the  teeth,  hair,  and  genital  organs  die  before  the  rest  of 
the  body.  Syphilis,  either  hereditary  or  acquired,  may  cause  acoustic 
nerve  atrophy. 

Deafness  from  acoustic  atrophy  may  develop  in  the  course  of  loco- 
motor ataxia,  of  general  paresis,  and  of  disseminated  sclerosis.  I 
have  also  seen  deafness  from  auditory  atrophy  develop  in  the  course 
of  tumors  of  the  brain  lying  upon  the  base  in  such  position  as  to 
involve  the  auditory  nerve.  In  syphilis  of  the  base  of  the  brain 
deafness  occasionally  develops.  Injuries  of  the  head  or  ear  may 
cause  deafness.  This  is  usually  attended  by  vertigo  and  is  due  to 
hemorrhage  in  the  ear.  Such  hemorrhages  may  occur  spontaneously 
in  the  infectious  diseases  or  in  states  of  anaemia.  The  use  of  quinine 
may  cause  degeneration  of  the  eighth  nerve.  A  functional  deafness 
occurs  in  hysteria  from  suspension  of  activity  in  fibres  of  the  auditory 
nerve  or  in  its  centres.  This  form  of  deafness  comes  on  more  sud- 
denly and  is  more  complete  than  any  other  type. 

The  prognosis  in  deafness  from  auditory  disease  is  unfavorable, 
though  occasionally  counter-irritation  over  the  petrous  portion  of  the 
temporal  bone,  and  treatment  by  strychnine,  is  accompanied  by  some 
improvement,  but  the  nerve  is  not  open  to  any  direct  treatment,  and 
electrical  excitation  is  of  no  benefit.  Hypodermic  injections  of 
pilocarpine,  ^  grain,  are  said  to  be  of  some  service.  Charcot  recom- 
mended quinine,  15  grains  a  day,  to  be  kept  up  some  weeks,  but  the 
majority  of  aural  surgeons  are  opposed  to  this  form  of  treatment. 

Tinnitus  Aurium.  —  Irritation  of  the  auditory  nerve  in  the  organ 
of  Corti,  or  in  the  middle  ear,  or  in  the  course  of  the  nerve,  may  pro- 
duce subjective  sounds  which  are  appreciated  as  a  ringing  or  roaring 
in  the  ears  or  head,  or  a  buzzing  sound  supposed  to  be  outside  of  the 
ear.  This  usually  indicates  disease  of  the  organ  of  Corti,  either  of  the 
nature  of  disturbances  in  the  circulation  or  of  a  beginning  auditory 
atrophy,  and  if  it  is  followed  by  progressive  deafness  the  diagnosis  of 
auditory  atrophy  may  usually  be  made.  In  at  least  80  per  cent,  of 
the  cases  of  tinnitus  aurium  some  central  deafness  can  be  detected  by 
tests  with  the  Galton  whistle  or  the  tuning-fork,  even  when  the  patient 
is  not  aware  that  he  is  deaf.  Tinnitus  aurium  may,  however,  be  due 
to  anaemia.  Thus,  fainting  is  frequently  preceded  by  a  sound  in  the 
ear.  Gout  is  a  cause  of  this  symptom.  A  chronic  anaemia  with  re- 
duction in  the  number  of  red  corpuscles  may  cause  tinnitus.  In  these 
cases  the  tinnitus  is  probably  due  to  vibrations  of  the  blood  current  in 
the  carotid  artery,  and  can  be  arrested  temporarily  by  pressure  upon 
the  carotid  artery.  Intracranial  aneurism  causes  a  sound  similar  to 
this.     It  is  because  of  this  blood  origin  of  tinnitus  that  the  symptom 


TEE  EIGHTH  NERVE:  THE  AUDITORY  NERVE.  665 

develops  in  states  of  cachexia  and  in  states  of  general  malnutrition. 
Direct  irritation  of  the  auditory  nerve,  such  as  occurs  in  all  forms  of 
otitis  media  and  otitis  externa,  may  produce  tinnitus,  and  anything 
which  interferes  with  the  free  circulation  of  air  in  the  external  auditory 
canal,  such  as  covering  the  ear  with  the  hand  or  applying  a  shell  to 
the  ear,  will  give  rise  to  a  perception  of  unusual  sounds.  Some  drugs, 
especially  quinine  and  the  salicylates,  cause  tinnitus,  and  if  too  long 
continued,  total  deafness.  In  hemorrhagic  affections  of  the  ear 
(Meniere's  disease),  where  the  disease  lies  in  the  second  or  vestibular 
division  of  the  auditory  nerve,  both  tinnitus  and  deafness  are  present. 
A  cracking  noise  due  to  contraction  of  the  muscles  near  or  about  the 
Eustachian  tube  is  not  the  same  as  tinnitus. 

The  treatment  of  tinnitus  aurium  is  very  unsatisfactory  unless  the 
cause,  such  as  anaemia,  or  gout,  or  malnutrition,  or  local  disease  in  the 
ear,  can  be  removed.  But  when  the  tinnitus  is  the  first  symptom  of 
an  auditory  atrophy  no  cure  can  be  expected.  Bromides  are  of  some 
service,  as  are  also  belladonna  and  cannabis  indica. 

II.  The  second  division  of  the  auditory  nerve  is  called  the  vestibular 
nerve.  It  is  the  nerve  of  equilibrium.  It  arises  from  the  bipolar  cells 
of  the  ganglion  of  Scarpa,  whose  dendrites  come  from  the  ampullae  of 
the  semilunar  canals  and  utricle  of  the  labyrinth.  The  axones  of  these 
cells  pass,  with  the  cochlear  division  of  the  nerve,  to  the  side  of  the 
pons,  where  they  enter  directly  the  lateral  portion  of  the  pons  (Fig. 
83)  and  terminate  about  three  groups  of  cells  :  the  cells  of  Deiters' 
nucleus,  the  cells  of  Bechterew's  nucleus,  and  the  cells  of  the  central 
or  posterior  nucleus  lying  upon  the  floor  of  the  fourth  ventricle. 

The  cells  of  Deiters'  and  Bechterew's  nuclei  have  numerous  con- 
nections with  other  parts  of  the  central  nervous  system,  especially 
with  the  cerebellum,  with  the  nuclei  of  the  oculomotor  nerves  (sixth, 
fourth  and  third),  with  various  nuclei  in  the  tegmentum,  and  with  the 
olivary  bodies.  Some  axones  of  Deiters'  cells  pass  downward  through 
the  formatio  reticularis  to  the  motor  nuclei  of  the  cervical  nerves  in 
the  spinal  cord,  and  are  probably  connected  either  directly  or  by  means 
of  association  fibres  with  the  nuclei  of  the  nerves  of  the  head  and 
back.  Its  connection  with  the  cerebellum  is  made  by  fibres  which 
pass  by  way  of  the  inferior  and  middle  peduncles  of  the  cerebellum  to 
the  flocculus  and  vermis.  Each  nucleus  is  also  connected  by  means 
of  direct  and  indirect  neurones  with  the  cerebellum  of  the  opposite 
side,  which  it  reaches  through  the  corpus  trapezoideus  and  transverse 
fibres  of  the  pons.  The  posterior  nucleus  of  the  vestibular  nerve  is 
connected  by  direct  tracts  through  the  corpus  trapezoideus  and  fillet 
and  through  the  formatio  reticularis  with  the  upper  portion  of  the  brain 
axis,  the  corpora  quadrigemina,  corpora  geniculata,  and  optic  thalami. 
It  is  also  connected  with  the  cerebellum  by  tracts  which  pass  with 
those  from  Deiters'  nerve.  The  existence  of  a  tract  to  the  cortex 
through  the  internal  capsule  has  not  yet  been  demonstrated,  and  no 
cerebral  centre  or  area  controlling  equilibrium  has  been  discovered. 
The  weight  of  evidence  in  favor  of  the  cerebellum  as  the  chief  organ 


666  T:S^  CMANTA.T'   NlSBfES  AND   THElB  DlSJSAftP..^. 

of  equilibrium  is  overwhelming,  and  it  seems  needless  to  suppose  that 
a  cerebral  area  will  be  discovered.  It  is  evident,  therefore,  that  the 
semilunar  canals  in  which  impulses  are  received  whose  object  is  to 
make  us  aware  of  our  position  in  space  and  to  direct  the  whole  system 
of  movements  by  which  our  equilibrium  is  preserved,  have  a  most 
widespread  connection  with  the  central  nervous  system  and  an  espe- 
cially close  connection  with  the  cerebellum. 

A  moment's  consideration  of  the  phenomena  of  equilibrium  will 
convince  us  of  the  great  importance  and  complexity  of  the  nervous 
mechanism  controlling  it.  The  body  is  constantly  held  in  a  state  of 
balance,  and  no  movement  of  any  degree  can  be  made  in  any  part 
without  a  compensatory  movement  to  preserve  the  centre  of  gravity. 
The  slightest  variation  in  our  position  which  would  tend  to  the  loss  of 
balance  is  immediately  perceived  and  immediately  corrected.  Further- 
more, the  balance  is  maintained  by  an  interaction  of  visual  sensations, 
labyrinthine  impressions,  and  muscular  sensations,  all  of  which  enter 
into  this  function,  as  can  be  seen  by  the  disturbance  of  balance  oc- 
casioned by  double  vision,  or  by  labyrinthine  disease,  or  by  a  loss  of 
the  muscular  sense,  as  in  locomotor  ataxia.  While  consciousness  takes 
cognizance  of  this  complex  matter  of  equilibrium,  the  primary  centres 
which  receive  the  impressions  and  regulate  the  activity  that  they 
arouse  are  located  in  the  cerebellum.  This  is  the  great  organ  of  equi- 
librium, and  it  therefore  receives  impressions  directly  from  the  eyes, 
from  the  semilunar  canals,  and  from  the  muscles  of  the  limbs.  The 
most  important  impulses  in  the  preservation  of  equilibrium,  however, 
are  derived  from  the  semilunar  canals  through  the  vestibular  nerve, 
and  aflFections  of  this  nerve  are  attended  by  very  marked  and  very 
intense  symptoms  of  vertigo.  Vertigo  may  be  due,  as  is  usually  the 
case,  to  affections  of  the  terminal  organs  in  the  semilunar  canals,  or  to 
affections  of  the  vestibular  portion  of  the  auditory  nerve  in  its  course 
to  the  cerebellum,  or  to  lesions  of  the  pons,  or  to  disease  of  the  cere- 
bellum. Disease  of  the  crura  not  involving  the  corpora  quadrigemina, 
or  of  the  superior  peduncles  of  the  cerebellum,  through  which  the  ocu- 
lar impulses  reach  the  cerebellum,  also  produces  vertigo. 

Vertigo  is  a  symptom  of  uncertainty  of  position,  attended  by  a  sensa- 
tion as  if  the  body  itself  were  being  revolved  in  any  one  of  all  possible 
directions,  or  as  if  objects  outside  of  the  body  were  turning  rapidly. 
It  causes  balancing  movements,  oscillation  of  the  eyeballs,  and  stag- 
gering in  the  gait.  It  is  attended  by  great  uncertainty  and  distress  of 
mind,  the  patient  grasping  at  adjacent  objects  for  support  as  he  feels 
himself  falling,  or  throwing  himself  flat  upon  the  floor  and  clinging  to 
anything  in  the  vicinity  for  support.  A  distinction  is  sometimes  made 
clinically  between  the  sensations  of  objects  outside  of  one's  self  turning, 
called  objective  vertigo,  or  of  one's  body  being  in  a  state  of  revolution, 
called  subjective  vertigo.  But  this  distinction  of  symptoms  cannot  be 
referred  to  any  difference  in  the  lesion  in  any  part  of  the  mechanism. 
Vertigo  may  be  produced  by  a  rapid  revolution  of  the  body  or  by  the 
passage  of  a  galvanic  current  through  the  head.     It  is  thought  that 


t:b:b  ninth  neeve:  the  glossophabyngeal  Nebve.     667 

hemorrhages  in  or  destruction  of  the  superior  vertical  or  sagittal  semi- 
lunar canaf  (anterior,  of  Retzius)  produce  a  sense  of  revolution  in  the 
entire  body,  as  if  the  head  were  going  down  and  the  feet  up,  the  head 
going  toward  the  side  of  the  lesion.  It  is  thought  that  hemorrhages 
in  the  inferior  or  horizontal  semilunar  canal  (external,  of  Retzius)  pro- 
duces a  sensation  of  rotation  of  the  body  around  its  vertical  axis  toward 
the  side  aifected.  It  is  thought  that  a  hemorrhage  in  the  posterior 
vertical  or  frontal  semilunar  canal  (posterior,  of  Retzius)  produces  a 
sensation  of  falling  toward  the  side  of  the  lesion.  But,  inasmuch  as 
these  three  canals  open  into  one  another,  further  investigation  is  neces- 
sary in  order  to  establish  these  assertions. 

Barany  has  called  attention  recently  '  to  the  uniform  association  of 
nystagmus  with  labyrinthine  disease  and  has  discovered  a  caloric  test 
of  great  value.  When  in  a  normal  person  cold  or  hot  water  is  thrown 
by  a  Politzer  syringe  into  the  outer  ear  without  exerting  pressure, 
rotary  and  horizontal  nystagmus  is  produced ;  if  the  labyrinth  is  de- 
stroyed this  never  occurs.  When  vertigo  is  caused  by  labyrinthine 
disease  nystagmus  is  always  present. 

Vertigo  is  usually  associated  with  intense  vomiting  and  great  men- 
tal distress.  A  rapid  action  of  the  heart  and  general  relaxation  of  the 
bloodvessels,  causing  profuse  sweating  and  a  feeling  of  great  faintness, 
commonly  attend  vertigo.  It  seems  probable  that  anything  which 
causes  an  intense  irritation  of  the  nuclei  of  the  vestibular  nerve  pro- 
duces a  sympathetic  disturbance  of  the  pneumogastric  centre  which  lies 
adjacent  to  it.  Conversely,  we  know  that  irritation  coming  from  the 
pneumogastric  nerve  to  the  brain,  such  as  occurs  in  gastric  disorders  or 
in  heart  disease,  may  produce  a  secondary  vertigo. 

Gastric  vertigo  is  easily  differentiated  from  vertigo  due  to  vestibular 
disease  by  the  absence  of  tinnitus  and  deafness  or  pain  in  the  ear. 
Vertigo  due  to  cerebellar  affection  is  also  rarely  followed  by  deafness 
or  by  pain  in  the  ear.     See  also  page  898. 

THE  NINTH  NERVE:   THE  GLOSSOPHARYNGEAL  NERVE. 

This  is  a  mixed  nerve  containing  both  sensory  and  motor  fibres.  Its 
sensory  branches  come  from  the  tongue,  larynx,  and  respiratory  organs 
and  middle  ear.  It  is  the  nerve  of  common  sensibility  of  the  pharynx 
and  palate  and  of  the  middle  ear.  It  is  thought  that  it  conveys  the 
sensations  of  taste  from  the  posterior  third  of  the  tongue,  those  from 
the  anterior  two-thirds  passing  in  through  the  lingual  nerve.  But  as 
division  of  the  root  of  the  trigeminal  nerve  has  caused  a  total  loss  of 
taste  in  some  patients,  there  is  some  doubt  as  to  this  function.  (See 
page  651.)  The  ganglia  of  origin  are  the  ganglion  jugulare  on  the 
glossopharyngeal  nerve  and  the  two  ganglia  on  the  vagus  nerve  (Plate 
XXVIII.).  The  sensory  fibres  enter  the  side  of  the  medulla  just  outside 
of  the  upper  part  of  the  olivary  body,  and  terminate  partly  in  the  gray 
matter  on  the  floor  of  the  fourth  ventricle  and  partly  in  a  thin  column 
of  gray  matter  like  the  substantia  gelatinosa,  lying  along  the  inside  of 
1  E.  E5,rdny.     Phys.  u.  Path,  des  BoKenpranKapparatus,  Deuticke.  Wien.  1907. 


668  TBE   CHANIAL  NEBVES  AND   TEEIB  DISEASES. 

the  solitary  bundle  in  the  formatio  reticularis.  The  sensory  fibres  on 
entering  the  medulla  form  a  part  of  this  bundle  with  other  sensory 
fibres  of  the  vagus  nerve  and  turn  downward,  passing  as  low  as  the 
upper  cervical  segment  of  the  spinal  cord.  The  solitary  bundle  is 
therefore  similar  in  its  structure  to  the  descending  root  of  the  fifth 
nerve.  It  has  also  been  called  the  respiratory  bundle  of  Krause,  as 
its  division  suspends  respiratory  motions.  Its  fibres  end  in  tufts  which 
lie  in  the  substantia  gelatinosa  which  borders  its  inner  surface.  From 
the  cells  of  this  substance  new  fibres  arise  which  enter  the  formatio 
reticularis  and  the  lemniscus,  and  so  ascend  to  the  brain  ;  but  their 
termination  in  the  cortex  has  not  yet  been  ascertained.  (See  Fig. 
56,  page  116.) 

The  motor  branches  of  the  glossopharyngeal  nerve  arise  from  a 
column  of  cells  known  as  the  nucleus  ambiguus,  which  lies  in  the 
lateral  part  of  the  formatio  reticularis.  (Fig.  66.)  They  curve  upward, 
inward,  and  backward,  then  outward,  forming  a  knee  like  that  of  the 
facial  nerve.  They  issue  from  the  side  of  the  medulla  posterior  to  the 
olivary  body  and  pass  out  of  the  skull  through  the  jugular  foramen. 
They  go  to  the  muscles  of  the  larynx,  oesophagus,  and  pharynx,  and 
preside  over  the  functions  of  respiration,  swallowing,  and  phonation. 
(See  also  Fig.  77,  page  148.) 

The  origin  of  both  the  motor  and  sensory  fibres  of  the  glosso- 
pharyngeal nerve  are  so  intimately  mingled  with  those  of  the  vagus 
nerve  that  separation  between  them  is  impossible.  The  following  table 
of  Spencer  shows  the  probable  functions  of  these  two  nerves : 

Table  XVIII.  —  The  Distribution  of  the  Ninth,  Tenth,  and 
Eleventh  Nerves. 

Afferent.  Afferent. 

Upper  roots,  ninth  nerve.    Kespiratory  regulating  fibres  ;  Cricothyroid,       stylopharyn- 

respiratory  exciting  fibres  ;  geal,  oesophagus,  pharyngeal 

(inspiration)  inferior  laryn-  contractors, 
geal. 

Middle  roots,  tenth  nerve.    Eespiratory  inhibitory  fibres  ;  Gastric    branches,    bronchial 

(expiration)  bronchial.  muscles. 

Afferent.  Efferent. 
Lower  roots,  tenth  nerve.    None.  Inferior  laryngeal,  cardiac  in- 
accessory  eleventh  nerve.  hibitory,  levator  palati. 
Spinal  accessory  eleventh   None.  Sternomastoid,  trapezius, 
nerve,  spinal  part. 

THE  TENTH  NERVE :  THE  VAGUS  OR  PNEUMOGASTRIC  NERVE. 

This,  like  the  ninth  nerve,  contains  both  sensory  and  motor  fibres. 
Its  sensory  branches  come  from  two  ganglia  on  its  trunk,  of  which 
the  upper  ganglion  is  large,  oval,  and  resembles  the  posterior  spinal 
ganglia,  and  the  other  is  long  and  irregular,  the  cells  being  scattered 
among  the  fibres  of  the  nerve.  (Plate  XXVIII.)  These  sensory  fibres 
enter  the  side  of  the  medulla  outside  of  the  olivary  bodies  and  pass  in 
several  directions.  Some  go  inward  and  backward  to  terminate  in  a 
mass  of  gray  matter  which  lies  on  the  floor  of  the  ventricle  laterad  of 
the  twelfth  nerve  nucleus  in  the  medulla  and  dorsad  of  it  in  the  upper 


PLATE   XXVIII 


GLOSSOPHARYNGEAL 


XI    NERVE- 


PNEUMOGASTRIC 

lOOR  15  BUNDLES 


PARS  ACCESSORIA 

4  OR  5  BUNDLES 


AURICULAR   BRANCH 


.  PARS  SPINALIS 


RIGHT  VAGUS 
BEHIND  — 


LEFT  VAGUS 

TO  HEPATIC 

PLEXUS 


RIGHT  VAGUS 

TO  CCELIAC, 

SPLENIC  AND  LEFT 

RENAL  PLEXUSES 


Distribution  of  Right  Pneumogastric  Nerve.     (W.  Keiller.) 


TEE  TENTH  NEEVE :  VAGUS  OB  PNEUMOGASTBIC  NEBVE.     669 

cord,  a  mass  which  projects  upon  the  floor  of  the  tourth  ventricle  form- 
ing the  ala  cinerea.  This  is  the  respiratory  and  cardiac  centre.  Others 
join  the  ninth  nerve  fibres  and  enter  the  solitary  bundle,  descending 
in  it  and  ending  in  the  substantia  gelatinosa  which  borders  it  on  its 
inner  side.  From  these  nuclei  new  fibres  arise  which  enter  the  for- 
matio  reticularis  and  ascend  to  the  brain,  but  their  course  and  termi- 
nation is  yet  undetermined. 

The  motor  branches  of  the  vagus  arise  from  the  cells  of  the  nucleus 
ambiguus,  turn  inward,  curve  about  in  the  formatio  reticularis,  and 
then  turn  outward  and  make  their  exit  on  the  side  of  the  medulla, 
where  they  form  the  great  trunk  of  the  vagus  nerve. 

The  destination  of  some  of  these  sensory  and  motor  fibres  has 
already  been  shown  in  the  table,  page  668. 

There  are  also  fibres  which  pass  to  the  meningeal  branches  of  the 
fifth,  to  the  auricular  branches  of  the  seventh,  and  to  the  carotid  and 
abdominal  plexuses  of  the  sympathetic  system. 

While  it  might  be  supposed  from  the  anatomical  distribution  that  a 
lesion  of  the  glossopharyngeal  nerve  would  cause  anaesthesia  and 
paralysis  of  the  pharynx  and  larynx  and  difficulty  in  swallowing,  there 
are  no  cases  on  record  of  lesion  of  this  nerve  trunk. 

In  bulbar  paralysis  the  nuclei  of  the  nerve  are  affected,  and  paralysis 
of  the  oesophagus  and  of  the  pharynx  and  larynx  are  the  result ;  but 
here,  too,  it  is  not  possible  to  distinguish  sharply  between  symptoms 
due  to  lesion  of  the  ninth  and  tenth  nerves. 

The  great  length  of  the  vagus  nerve  exposes  it  to  many  injuries  and 
to  compression  by  tumors  at  any  point  in  its  course.  Acute  neuritis 
of  the  vagus  from  cold  or  rheumatism  is  not  known  to  occur.  Certain 
poisons,  especially  that  of  diphtheria,  and  alcohol  and  certain  drugs, 
atropine,  veratrum,  aconite,  and  digitalis,  etc.,  appear  to  have  a  special 
selective  action  upon  the  nerve  and  may  produce  symptoms  referable 
to  it.  While  the  distribution  of  the  nerve  to  the  heart,  lungs,  and 
viscera  is  a  very  wide  one,  we  know  but  little  regarding  the  symptoms 
actually  produced  by  its  lesion.  The  nerve  is  a  bilateral  one  in  its 
action,  and  in  many  cases  where  it  has  been  divided  by  stab  wounds, 
or  in  the  course  of  surgical  operations  upon  the  neck,  very  few  symp- 
toms have  ensued,  though  experiments  upon  animals  would  indicate 
that  a  division  of  the  left  nerve  should  be  attended  by  a  very  rapid 
heart  action  and  by  a  slowing  of  respiration.  This  has  occasionally 
occurred  when  surgeons  have  included  the  vagus  in  tying  the  left 
carotid  artery.  The  branches  of  the  nerve  which  pass  to  the  larynx 
and  supply  all  the  muscles  of  the  larynx  are  most  important,  and  their 
injury  gives  rise  uniformly  to  very  marked  symptoms.  These  symptoms 
have  been  carefully  studied  by  Gowers,  whose  table  is  so  complete 
that  it  is  here  reproduced. 

It  will  be  remembered  that  the  recurrent  laryngeal  nerve  in  its 
long  course  around  the  aorta  is  frequently  compressed  by  aneurisms, 
and  the  result  of  such  compression  is  usually  jxiralysis  of  the  vocal 
cord  on  one   side,  with  whispering  voice  and  difficulty  of  respiration. 


670 


TEE   CBANIAL  NERVES  AND    THEIB  DISEASES. 


The  abductors  of  the  glottis  being  paralyzed,  the  opening  of  the  glottis 
is  not  sufficient  during  the  act  of  respiration,  and  hence  a  whistling 
sound  or  wheeze  and  a  sense  of  suffocation  results.  In  a  few  cases  of 
injury  of  the  vagus  in  this  part  of  the  neck  some  anaesthesia  of  the 
throat  back  of  the  palate  has  been  observed,  together  with  disturbance 
in  the  act  of  swallowing.  The  reflex  act  of  swallowing  from  irritation 
of  the  pharynx  is  also  suspended  in  these  cases. 

An  occasional  paralysis  of  the  vagus  nerve  occurs  as  a  complication 
of  locomotor  ataxia,  and  spasm  of  the  larynx  with  suffocation  may 
also  occur  in  this  disease. 


Sym.pioms. 

No  voice  ;  no  cough  ;  stridor  on 
deep  inspiration  only. 

Voice  low-pitched  and  hoarse  ; 
no  cough  ;  stridor  absent  or 
slight  on  deep  breathing. 


Voice  little  changed ;  cough 
normal  ;  inspiration  difficult 
and  long,  with  loud  stridor. 


Symptoms  inconclusive  ;   little 
affection  of  voice  or  cough. 


No  voice,  perfect  cough ;    no 
stridor  or  dyspnoea. 


Signs. 

Both  cords  moderately  abducted 
and  motionless. 

One  cord  moderately  abducted 
and  motionless ;  the  other 
normal  or  even  beyond  the 
middle  line  in  phonation. 

Both  cords  near  together,  and 
during  inspiration  not  sepa- 
rated but  even  drawn  nearer 
together. 

One  cord  near  the  middle  line, 
not  moving  during  inspira- 
tion ;  the  other  normal. 

Cords  normal  in  position,  and 
moving  normally  in  respira- 
tion, but  not  brought  together 
on  an  attempt  at  phonation. 


Lesions. 

Total  bilateral 
palsy. 

Total    unilateral 
palsy. 


Total    abductor 
-     palsy. 


Unilateral    abduc- 
tor palsy. 

Adductor  palsy. 


The  diagnosis  of  vagus  paralysis  can  be  made  when  the  recurrent 
branches  of  the  nerve  are  affected  and  disturbances  in  respiration  and 
in  the  voice  develop,  or  when  there  is  a  distinct  anaesthesia  of  the 
pharynx,  with  difficulty  of  swallowing,  not  due  to  paralysis  of  the 
palate  or  to  paralysis  of  the  tongue.  Rapidity  of  the  heart  is  not  a 
sufficient  sign  to  warrant  a  diagnosis,  and  there  are  no  lung,  gastric, 
or  visceral  symptoms  that  are  characteristic  of  the  disease,  the  majority 
of  the  statements  made  in  the  books  being  purely  theoretical  and  not 
based  upon  clinical  facts  or  pathological  findings. 

In  the  course  of  bulbar  paralysis  the  nuclei  of  the  ninth  and  tenth 
nerves  become  involved  and  undergo  degeneration.  This  is  indicated 
clinically  by  the  development  of  respiratory  and  laryngeal  palsy  and 
by  a  very  rapid  heart  action.  These  may  lead  to  a  fatal  result.  The 
occurrence  of  hemorrhage  or  of  embolism  in  the  medulla  involving  the 
vagus  nuclei  is  the  cause  of  sudden  death  in  some  cases  of  apoplexy. 
The  vagus  nucleus  when  disturbed  in  its  function  can  no  longer  secure 
normal  breathing  or  heart  action,  hence  Cheyne-Stokes  respiration  and 
intermittent  or  very  rapid  heart  action  are  supposed  to  be  symptoms 
referable  to  its  functional  disturbance.  Vomiting  is  also  supposed  to 
be  due  to  its  irritation.     This  form  of  vomiting  is  sudden,  explosive, 


THE  ELEVENTH  NERVE:  THE  SPINAL  ACCESSORY  NERVE.     671 

not  preceded  by  nausea  and  is  very  violent,  and  is  attended  oy  great 
prostration^  It  occurs  occasionally  in  tumors  or  abscess  of  the  brain 
in  any  location  and  also  in  meningitis  of  the  base. 

The  vagus  nerve  is  often  involved  in  the  course  of  multiple  neuritis 
due  to  any  of  the  numerous  causes  which  produce  that  disease.  The 
signs  of  its  implication  are  rapid  pulse,  general  vasomotor  paralysis, 
shown  by  cyanosis,  oedema,  profuse  sweating,  and  respiratory  paralysis, 
causing  irregular,  labored  breathing.  Under  these  conditions,  which 
are  of  a  very  unfavorable  prognostic  importance,  stimulation  by  alcohol, 
strychnine,  and  digitalis,  and  by  nitroglycerin  are  imperative.  (See 
page  209.) 

Spasm  of  the  oesophagus  is  not  to  be  considered  as  an  evidence  of 
affection  of  the  ninth  nerve,  but  is  a  functional  nervous  disease  usually 
hysterical  in  origin  or  due  to  organic  disease  of  the  oesophagus. 

THE  ELEVENTH  NERVE :  THE  SPINAL  ACCESSORY  NERVE. 

Paralysis  of  the  Accessorius.  —  The  eleventh  nerve  arises  from  a 
long  column  of  cells  which  lie  in  the  lateral  part  of  the  anterior  horn  in 
the  upper  five  cervical  segments  of  the  spinal  cord.  The  roots  issue 
from  the  lateral  surface  of  the  cord  and  ascend  to  the  brain,  where  they 
receive  further  fibres  from  the  lateral  portion  of  the  medulla,  the  acces- 
sory portion,  which  really  is  independent  of  the  nerve  and  belongs  to 
the  vagus,  and  then  make  their  exit  from  the  skull  through  the  jugular 
foramen.  It  is  rarely  injured  by  disease  within  the  spinal  canal  or 
skull,  though  fractures  and  injuries  may  affect  it.  The  usual  cause  of 
paralysis  of  the  spinal  accessory  is  cold,  injury,  or  pressure  by  tumors, 
swollen  glands,  or  abscess  in  the  neck.  I  have  seen  the  condition  in  a 
young  girl  due  to  accidental  division  of  the  nerve  when  deep-lying 
glands  were  removed  from  the  neck. 

Symptoms.  —  The  symptoms  of  paralysis  of  the  spinal  accessory  nerve 
are  paralysis  of  the  sterno-cleido-mastoid  muscle  and  of  the  trapezius 
muscle.  The  sterno-cleido-mastoid  turns  the  head  and  elevates  the 
chin,  so  that  when  the  right  muscle  contracts  the  patient  looks  over  his 
left  shoulder.  The  trapezius  turns  the  head  backward  and  over  toward 
the  shoulder  and  also  elevates  the  shoulder.  Other  muscles  act  in  asso- 
ciation with  these  two  muscles,  and  hence  a  lesion  of  the  spinal  acces- 
sory nerve  causing  a  paralysis  of  these  muscles  is  not  apparent  until  the 
patient  is  examined ;  then  it  is  found  that  the  motions  which  they  ac- 
complish are  not  done  with  the  usual  amount  of  power,  and  a  slight 
amount  of  resistance  renders  the  patient  incapable  of  turning  the  head 
or  of  elevating  the  shoulder.  The  greatest  defect  lies  in  the  elevation 
of  the  arm  above  the  horizontal  line  ;  the  trapezius  counteracts  the 
action  of  the  serratus,  and  if  it  is  paralyzed  the  serratus  rotates  the 
scapula  in  such  a  manner  as  to  produce  a  slight  elevation  upon  the  side 
of  the  neck  by  the  projection  upward  of  its  inner  angle.  When  the 
patient  l^rings  his  arm  forward  and  outward  the  edge  of  the  shoulder- 
blade  is  no  longer  held  tightly  to  the  chest,  and  hence  projects  upon 
the  };uck. 


672  TEE   CBANIAL  NEBVES  AND    THEIE  DISEASES. 

The  diagnosis  is  made  by  the  discovery  of  the  weakness  of  the  mus- 
cles, of  the  imperfect  elevation  of  the  arm,  of  the  deformity,  and  of  the 
existence  of  reaction  of  degeneration  to  the  electrical  examination. 

The  prognosis  will  depend  upon  the  possibility  of  removal  of  the 
cause,  and  the  treatment  is  by  massage  and  electricity.  In  cases  which 
do  not  recover  it  may  be  possible  by  appropriate  apparatus  to  pro- 
duce a  fixation  of  the  shoulder-blade  in  its  proper  position,  and  thus 
aid  in  the  movement  of  the  arm. 

Torticollis:  Wryneck. —  Congenital  lesions  of  the  eleventh  nerve 
due  to  defects  of  development  or  injuries  at  the  time  of  birth  sometimes 
result  in  a  defective  development  of  the  sterno-cleido-mastoid  muscle, 
which  fails  to  grow  in  proportion  to  the  other  muscles,  and  hence  be- 
comes permanently  shortened.  In  these  cases  as  the  child  grows  the 
head  is  slowly  turned  away  from  the  affected  side,  the  chin  approaching 
the  shoulder,  and  it  cannot  be  turned  straight  or  to  the  opposite  side  on 
account  of  the  permanent  shortening  of  the  affected  muscle.  In  such 
a  condition  there  is  no  spasmodic  contraction  of  the  muscle  and  no 
twitching  of  the  head,  but  the  lesion  is  a  wryneck  fixed  and  permanent. 
This  condition  is  easily  remedied  by  a  division  of  the  tendons  of  the 
sternomastoid  muscle,  when  the  other  muscles  secure  a  proper  position 
and  proper  movement  of  the  head.  Spasmodic  torticollis  is  described 
on  page  772. 

THE  TWELFTH  NERVE:  THE  HYPOGLOSSAL  NERVE. 

Anatomy.  —  The  hypoglossal  nerve  arises  from  a  long  group  of  cells 
which  lies  on  the  floor  of  the  fourth  ventricle  in  its  lowermost  part, 
where  its  walls  approach  and  where  it  closes  into  the  central  canal  of 
the  spinal  cord.  (Fig.  77.)  The  nerve  cells  are  large  and  multipolar. 
They  send  their  axones  ventrally  through  the  medulla,  passing  at  the 
side  of  the  interolivary  tract  and  pyramid  and  issuing  between  the 
pyramid  and  the  olivary  body  on  the  ventral  surface  of  the  medulla ; 
thence  they  make  their  exit  from  the  skull  through  the  anterior  con- 
dyloid foramen  of  the  atlas  and  pass  to  the  muscles  of  the  tongue.  (See 
Fig.  52,  page  107,  and  Fig.  78,  page  145.) 

Paralysis  of  the  Hypoglossal  Nerve.  —  The  hypoglossal  nerve  is 
rarely  involved  in  lesions  upon  the  base  of  the  brain,  though  occasion- 
ally tuberculous  or  syphilitic  masses  have  implicated  it.  In  such  cases 
the  vagus  nerve,  which  lies  quite  near  it,  is  also  involved.  It  is  not 
affected  in  multiple  neuritis. 

A  few  cases  of  injury  of  the  hypoglossal  nerve  have  been  recorded 
where  this  nerve  has  been  divided  by  stab  wounds  or  by  gunshot  wounds 
in  the  neck.  Occasional  attempts  to  cut  the  throat  result  in  a  division 
of  the  hypoglossal  nerve.  Cameron  ^  described  such  a  case  with  great 
care.  The  autopsy  showed  that  the  right  hypoglossal  nerve  had  been 
divided  just  internal  to  the  bifurcation  of  the  carotid  artery.  The  cut 
ends  were  retracted,  the  central  end  was  bulbous,  and  the  distal  end 
was  found  in  a  state  of  advanced  degeneration.     In  this  patient  the 

^British  Medical  Journal,  1901. 


TEE  TWELFTH  NEBVE:  THE  HYPOGLOSSAL  NERVE. 


673 


Fig.  283. 


right  side  of  the  tongue  was  paralyzed,  had  a  flabby  appearance,  and 
presented 'ihree  transverse  wrinkles  due  to  the  atrophy  ;  but  the  tongue 
could  be  moved  in  all  directions,  though  imperfectly,  and  with  diffi- 
culty toward  the  left.  It  protruded  to  the  right  side  of  the  mouth  in 
being  put  out,  but  there  was  no  paralysis  of  taste. 

The  roots  of  the  hypoglossal  nerve  are  occasionally  affected  in  dis- 
ease located  within  the  medulla.  They  pass  close  to  the  pyramidal 
tract  and  to  the  interolivary  tract.  These  convey,  respectively,  motor 
and  sensory  impulses  to  and  from  the  opposite  side  of  the  body  ;  hence 
a  form  of  alternate  paralysis  or  alternate  anaesthesia  may  be  caused  by 
a  lesion  which  destroys  one  of  these  tracts  and  the  hypoglossal  root. 
Such  lesions  may  be  of  vascular  origin  or  may  be  new  growths. 

The  symptoms  of  paralysis  of  the 
twelfth  nerve  are  inability  to  move  the 
muscles  of  the  tongue  or  to  protrude 
this  organ  from  the  mouth.  The  re- 
sult is  that  the  tongue  lies  motionless 
within  the  mouth,  its  muscular  fibres 
very  often  are  in  a  state  of  fibrillary 
tremor  and  the  tongue  itself  becomes 
atrophied,  and  the  mucous  membrane 
is  thrown  into  folds  with  deep  grooves 
between  them  which  run  lengthwise. 
(Fig.  283.)  Inasmuch  as  the  mus- 
cles of  the  tongue  are  closely  interlaced 
upon  the  two  sides,  a  unilateral  hypo- 
glossus  paralysis  does  not  entirely 
paralyze  this  organ.  It  can  be  moved 
in  the  mouth,  and  though  the  pro- 
nunciation of  some  of  the  lingual  letters 
is  interfered  with,  yet  unless  the  paral- 
ysis is  bilateral  this  is  not  very  notice- 
able. In  the  act  of  protruding  the 
tongue  the  healthy  muscles  elevate  its 

posterior  portion,  pushing  it  outward  more  fully  on  the  nonparalyzed 
side ;  hence  the  tongue  deviates  toward  the  side  of  the  paralysis. 
Chewing  and  swallowing  are  not  interfered  with  by  unilateral  hypo- 
glossus  paralysis,  but  are  seriously  impaired  if  the  paralysis  is  bilateral. 
It  is  difficult  to  examine  the  tongue  electrically  on  account  of  its  great 
sensitiveness,  but  it  may  be  possible  to  demonstrate  a  reaction  of 
degeneration  in  its  muscles. 

Paralysis  of  the  twelfth  nerve  from  disease  of  its  nucleus  may  be 
produced  as  a  part  of  the  lesion  of  bulbar  paralysis.  ^  (See  p.  616.) 
When  the  paralysis  is  due  to  an  affection  of  the  nuclei  of  the  hypoglossal 
nerve  there  is  usually  some  paralysis  of  the  orbicularis  oris  asso- 
ciated with  the  paralysis  of  the  tongue  as  a  few  of  the  fibres  from 
that  nucleus  pass  to  this  muscle. 

43  'Deut.  Zeitsch.  f.  Nervenheilk. ,  Bd.  xiii.,  4. 


Eight  hemiatrophy  of  the  tongue. 
(Scripture.) 


674  TEE   CBANIAL  NEEFES  AND    TUEIB  DISEASES. 

The  cortical  centres  for  the  movements  of  the  tongue  lie  in  the 
lower  third  of  the  motor  area,  and  are  joined  to  the  nucleus  by  fibres 
which  pass  in  the  motor  tract  anterior  in  the  capsule  and  median  in 
the  cms  to  the  tracts  for  the  arm  and  leg.  These  fibres  decussate  in 
the  raphe  of  the  medulla. 

Partial  paralysis  of  the  tongue  with  deviation  toward  the  paralyzed 
side  is  usually  an  accompaniment  of  hemiplegia,  but  in  this  case  there 
is  no  fibrillary  twitching  and  no  atrophy  in  the  tongue. 

There  is  no  known  treatment   for  this    form  of  atrophic   paralysis. 

A  spasmodic  contraction  of  the  muscles  of  the  tongue  (aphthongia) 
has  been  described  by  Charcot  ^  and  must  have  been  known  in  the 
middle  ages,  as  the  best  examples  are  found  copied  in  stone  in  some 
of  the  gargoyles  of  the  Notre  Dame  in  Paris  and  other  cathedrals  in 
Europe.  In  this  aifection  the  muscles  of  the  tongue  are  contracted, 
and  as  a  result  the  tongue  is  forcibly  thrust  out  of  the  mouth,  either 
straight  out  if  the  spasm  is  bilateral  (the  common  form),  or  toward 
one  side  if  it  is  unilateral.  The  tongue  may  also  be  forcibly  rolled  in 
the  mouth  or  thrust  against  the  teeth.  The  spasm  occurs,  as  a  rule,  in 
attacks  lasting  a  short  time,  but  extremely  annoying  to  the  patient, 
and  is  not  usually  associated  with  any  disease  of  the  tongue,  such  as 
ulceration,  smoker's  tongue,  or  cancer.  It  has  been  regarded  as  a 
purely  functional,  possibly  hysterical  affection,  but  like  all  spasms  may 
be  reflex  from  some  peripheral  or  central  irritation. 

The  prognosis  is  good.  The  treatment  should  consist  of  nerve 
sedatives,  especially  bromides,  chloral,  valerian,  and  asafcetida. 

1  Icon,  de  la  Salp^triere,  vol.  i.,  p.  87. 


SECTION  IV. 

GENEEAl  DISEASES  OE  THE  NEEVOUS 

SYSTEM. 


CHAPTER   XXXVI. 

MULTIPLE  SCLEROSIS. 

Multiple,  or  insular,  or  disseminated  sclerosis  is  a  disease  which 
affects  both  brain  and  spinal  cord  together  in  varying  degree,  and  is 
characterized  by  the  production  of  small  plaques  or  islets  of  sclerotic 
tissue  scattered  irregularly  through  the  central  nervous  system. 

Pathology.  —  The  sclerotic  patches  vary  from  a  millimetre  to 
several  centimetres  in  diameter  and  are  of  irregular  shape.  There 
may  be  a  hundred  or  more  such  plaques.  They  are  rarely  located 
in  the  cortex  of  the  brain,  but  are  found  in  the  white  matter  beneath 
it,  in  the  centrum  ovale  and  in  the  corpus  callosum,  and  in  the  gray 
matter  of  the  basal  ganglia  ;  also  in  the  pons,  medulla,  and  spinal 

Fig.  284. 


Multiple  sclerosis.    Sclerotic  patches  are  seen  in  both  anterior,  one  lateral,  and  both  posterior  col- 
umns.   The  left  anterior  horn  is  reduced  in  size  and  is  sclerotic.     (Larkin.) 

cord,  and  with  less  frequency  in  the  cerebellum  both  in  its  white  and 
gray  matter.  Sometimes  the  nerves,  both  cranial  and  spinal,  appear 
to  be  invaded  for  a  short  distance  from  their  origin. 

The  lesion  differs  from  that  occurring  in  other  forms  of  isclerosis 

675 


676 


MULTIPLE   SCLEBOSIS. 


as  it  is  not  limited  to  definite  tracts  and  as  it  does  not  lead  to  second- 
ary degeneration  in  the  various  tracts  in  which  it  may  happen  to  lie. 
This  is  because  it  spares  the  axones,  though  involving  the  medullary 
sheath  of  the  neurones.  In  a  fresh  state  the  patches  are  pinkish-gray 
and  more  translucent  than  normal  brain  tissue.  Some  have  a  gelatin- 
ous appearance.  They  replace  the  brain  tissue  without  increasing  its 
volume,  hence  are  not  like  small  tumors.  On  section  each  plaque  is 
sharply  defined  from  the  normal  tissue  about  it,  though  the  transition 
from  abnormal  to  normal  fibres  is  less  sudden  when  a  single  fibre  is 
followed  along  its  course.  It  may  be  soft  or  hard  and  may  in  old 
cases  cut  like  cartilage.  There  are  no  long  columns  of  sclerosis,  as 
in  tabes  or  lateral  sclerosis,  but  each  islet  is  limited  in  extent. 

On  microscopic  examination  the  sclerotic  patch  is  seen  to  consist  of 
very  fine  neuroglia  fibres  and  glia  cells,  forming  a  feltwork  of  interlac- 

FiG.  285. 


Multiple  sclerosis.    A  sclerotic  area  implicates  a  large  portion  of  the  medulla  oblongata.     (Spiller.) 

ing  fibrils  with  dense  infiltration  with  nuclei,  and  there  may  also  be  an 
increase  of  connective  tissue.  The  fibres  seem  finer  than  ordinary 
neuroglia  fibres,  and  are  more  abundant  in  the  older  cases,  the  early 
stage  being  characterized  by  the  presence  of  small  cells.     In  the  older 


PATHOLOGY. 


677 


cases  larg^  spider  cells  are  often  found.  There  is  usually  some  thick- 
ening of  the  bloodvessels.  The  neurones  themselves  are  singularly 
free  from  degeneration,  even  in  the  midst  of  sclerotic  patches  ;  but  oc- 
casionally cell  bodies  are  found  in  various  stages  of  degeneration  when 
the  plaque  invades  the  gray  matter,  and  fatty  globules  and  granular 
corpuscles,  the  result  of  this  degeneration,  are  then  scattered  through 
the  plaque.  Even  by  Nissl's  method  of  staining  there  are  found  few 
or  no  evidences  of  degeneration  in  the  neurones.  In  some  regions  a 
simple  atrophy  of  cells  has  been  seen.  The  nerve  fibres  passing 
through  the  islet  of  sclerosis  appear  to  be  squeezed,  their  calibre  being 
reduced  but  their  continuity  preserved.  The  medullary  sheath  is  re- 
duced materially,  and  here  and  there  an  axone  is  broken  and  degen- 
erated. The  result  of  the  degeneration  of  the  myelin  is  seen  in  the 
presence  of  fatty  globules  and  waxy  granules  in  the  nerve  patches.  But, 
as  a  rule,  the  axones  are  preserved  and  lie  naked,  adjacent  to  one 
another,  through  the  plaque,  and  appear  as  if  the  medullary  sheath  had 

Fig.  286. 


Multiple  sclerosis.    Longitudinal  section  through  a  plaque  in  the  spinal  cord  ;  g,  thickened  neur- 
oglia ;  I,  empty  spaces  in  the  plaque  ;  z,  large  glia  cells  ;  a,  axis  cylinder.     (Schmaus-Sacki.) 

been  dissolved  off  and  absorbed.  This  peculiar  appearance,  first  noticed 
by  Charcot,  has  been  confirmed  by  all  subsequent  observers  and  is  char- 
acteristic of  insular  sclerosis.  The  preservation  of  the  axone  explains 
the  absence  of  secondary  degenerations  in  the  various  tracts.  In  a  few 
recently  reported  cases,  probably  of  very  long  duration,  reliable  ob- 
servers have  reported  secondary  degenerations  in  both  sensory  and 
motor  tracts,  but  those  are  certainly  the  exception. 

The  essential  feature  of  the  disease  is  therefore  the  production  of  a 


678 


MULTIPLE  SCLEROSIS. 


peculiar  neuroglia  structure  which  is  associated  with  a  disappearance 
of  the  myelin  sheaths  of  nerve  fibres.  There  are  many  theories  in 
regard  to  this  process,  no  one  of  which  is  generally  accepted.  Striimpell 
believes  that  it  is  a  congeuitah  affection  of  the  nature  of  gliosis,  not 
unlike  syringomyelia  {q.  v.),  and  that  the  nerve  fibres  are  affected 
secondarily.  He  urges  as  proof  the  fact  that  it  occurs  in  childhood. 
Schmaus  suggests  that  instead  of  a  process  of  degeneration  of  the 
myelin  sheath  there  has  been  a  defective  development  of  myelin  origi- 
nally, and  that  the  naked  fibres  have  never  developed  a  myelin  cover- 
ing, a  possibility  which  is  rendered  more  likely  because  of  the  fact  that 
in  the  development  of  the  nerve  fibres  the  myelin  appears  later  than 


the  axone.  Under  these  conditions  it  would  be  expected  that  the 
symptoms  would  appear  in  infancy  instead  of  developing  after  the  age 
of  ten  years  or  later. 

Rindfleisch  and  others,  Ribbert,  Fiirstner,  Marie,  Bartsch,  and 
Schmaus  believe  that  the  process  is  vascular  in  origin,  a  toxic  agent 
ia  the  blood  affecting  first  the  vessels,  then  the  medullary  sheath  of 


ETIOLOGY.  679 

the  nefves,  and  then  leading  to  a  secondary  sclerosis.  They  affirm 
that  the  j^laques  lie  about  the  vessels,  which  others  deny.  Others 
affirm  that  a  lymph  stasis  in  the  lymph  spaces  about  the  bloodvessels 
is  the  primary  cause ;  that  these  spaces  are  crowded  with  epithelioid 
cells  which  distend  them,  hinder  the  flow  of  lymph,  and  thus  lead  to 
swelling  and  degeneration  of  the  glia  cells  and  nerve  elements,  and 
finally  to  a  sclerosis.  But  this  theory  has  met  with  little  favor,  as  the 
majority  of  observers  regard  this  lymph  stasis  as  a  result  and  not  as  a 
cause,  and  when,  under  other  conditions,  such  lymph  stasis  occurs  it 
leads  to  a  degeneration  of  the  axis  cylinder  and  not  to  one  of  the  myelin 
sheath  only.  Charcot,  Erb,  Gowers,  Leyden,  Wernicke,  and  Thoma  ^ 
hold  that  the  disease  is  an  interstitial  or  glia  inflammation  and  that  the 
myelin  sheaths  are  destroyed  secondarily.  Leyden,^  after  a  critical 
review,  concludes  that  multiple  sclerosis  is  the  termination  of  a  chronic 
interstitial  inflammation.  Redlich  holds  that  it  is  a  parenchymatous 
disease  in  the  myelin,  of  toxic  origin,  and  that  the  glia  formation  is 
secondary.  Andre  Tliomas  ^  affirms  that  it  begins  in  the  axis  cylinder 
and  affects  some  but  not  all  of  the  fibrils  of  which  it  is  made  up.  Its 
toxic  origin  is  admitted  by  all,  but  the  exact  manner  in  which  the 
toxic  agent  acts  and  the  reason  for  the  limitation  of  the  lesion  and  the 
escape  of  the  distal  part  of  the  axones  is  as  yet  a  matter  of  mystery. 
Buzzard*  who  has  called  attention  particularly  to  the  fact  that  the 
symptoms  of  the  disease  may  be  intermittent,  recoveries  and  relapses 
being  not  uncommon  in  his  experience,  refuses  to  admit  any  congenital 
defect  and  inclines  to  the  toxic  theory  of  the  disease. 

Etiology.  —  Multiple  sclerosis  is  a  disease  of  youth,  the  majority  of 
cases  developing  between  the  tenth  and  thirty-first  year.  No  age,  how- 
ever, is  exempt.  Unger  found  nineteen  cases  in  infants.  The  disease 
is  rarely  inherited,  but  two  cases  being  on  record  in  which  a  woman 
with  the  disease  had  a  child  who  showed  symptoms.  It  has  no  rela- 
tion to  syphilis.  Cold  and  trauma  were  formerly  supposed  to  be  the 
active  causes,  and  many  cases  have  been  recorded  which  render  this 
probable,  recent  cases  of  Gaupp  ^  and  Blencke  ^  being  undoubtedly  trau- 
matic. Of  late,  owing  to  the  investigations  of  Marie,  more  importance 
has  been  assigned  to  infectious  and  toxic  agents,  and  now  it  is  gener- 
ally believed  that  the  disease  is  usually  a  sequal  of  some  acute  infec- 
tious disease,  typhoid,  variola,  malaria,  scarlet  fever,  measles,  whoop- 
ing-cough, influenza,  pneumonia,  acute  rheumatism,  dysentery,  cholera, 
or  diphtheria.  It  has  been  known  to  develop  in  women  after  child- 
birth. I  have  seen  such  a  case,  and  Balint'^  has  reported  a  case  in 
which  after  each  of  four  successive  confinements  a  rapid  increase  in  the 
symptoms  of  the  disease  followed.     Oppenheim  has  seen  cases  due  to 

'ZurPath.  hist,  der  Mult.  Sclerose,  Deut.  Zeitschr.  f.  Nervenlieilk. ,  1900,  Bd.  xvii., 
p.  265. 

^Nothnagel's  Spec.  Path.  u.  Therap.,  Bd.  x.,  p.  459. 

^Rcv.  NouroI(^f,nque,  1900,  vol.  viii.,  p.  490. 

*  The  Lancet,  .Tuly  16,  1904. 

■'' Zur  Aetiologie  der  Mult.  Sclerose,  Centralbl.  f.  Nervenheilk.,  June,  1900. 

«Monatsschr.  f.  TJnfallsheilk.,  December,  1900. 

■'Deut.  Zeitsclir.  f.  Nervenheilk,,  Bd.  xvi.,  p.  437. 


6^0  MULTIPLE  SCLEttO^m. 

poisoning  by  metallic  poisons,  especially  lead,  zinc,  tin,  and  mercury, 
and  Etienne  ^  has  also  reported  one  by  carbonic  oxide  gas.  Striimpell, 
who  believes  the  disease  to  be  of  congenital  origin  and  due  to  mal- 
development,' ascribes  little  importance  to  these  causes  and  holds  that 
they  are  merely  exciting  causes  when  the  defective  nervous  system  is 
present.  Hoffman  was  not  able  to  find  any  cause  in  more  than  one- 
half  of  100  cases  studied.^ 

Symptoms.  —  It  has  been  already  stated  that  the  lesions  of  multiple 
sclerosis  affect  the  brain,  brain  axis,  and  spinal  cord  in  different  degrees 
in  various  cases,  and  therefore  it  is  not  surprising  that  three  types  of 
the  disease  have  been  described  by  writers  according  as  symptoms  ref- 
erable to  one  of  these  parts  predominate  ;  but  since  in  all  cases  which 
continue  for  a  long  time  the  terminal  result  is  about  the  same,  it  is 
only  necessary  to  mention  these  types  as  variations  in  the  mode  of  on- 
set. It  must  also  be  stated  at  the  outset  that  great  irregularity  in  the 
development  and  course  of  cases  has  been  observed,  and  therefore  Buz- 
zard is  quite  right  in  holding  that  no  typical  picture  of  multiple  sclerosis 
can  be  drawn.  In  fact,  in  many  cases  it  is  only  by  a  process  of  exclu- 
sion of  other  forms  of  cerebral  and  spinal  disease,  and  of  hysteria  in 
particular,  that  the  diagnosis  can  be  reached.  It  is  also  necessary  to 
premise  any  statement  of  symptoms  by  calling  attention  to  a  fact  ad- 
mitted by  all  authors  that  all  cases  are  characterized  by  occasional 
complete  intermissions  in  the  symptoms,  one  or  many  disappearing 
entirely  for  months,  but  returning  later. 

The  ordinary  type  of  case,  first  described  by  Charcot,  is  of  very 
gradual  development.  The  patient  has  some  numbness  and  weakness 
in  the  legs,  which  slowly  increases  until  he  has  a  spastic  or  ataxic  or 
even  cerebellar  gait,  and  stiffness  and  paralysis  in  the  legs,  with  in- 
creased knee-jerks  and  ankle  clonus  and  Babinski's  symptom.  There 
is  usually  a  slight  difficulty  in  the  control  of  the  sphincters  which  ap- 
pears early.  The  skin  reflexes  are  often  lost.  The  gait  is  usually  like 
that  of  lateral  sclerosis,  the  feet  being  drawn  along  the  floor  and  over- 
lapping and  the  legs  stiff  and  adducted.  It  may  be  a  staggering  gait 
like  that  of  a  drunken  man,  with  irregular,  uncertain  steps.  Some- 
times it  is  a  combination  of  these  two  gaits.  The  entire  body  sways 
in  walking,  which  is  due  to  an  irregular  contraction  of  the  muscles  of 
the  trunk,  producing  what  Oppenheim  has  termed  "  vascillation."  At 
the  same  time  a  tremor,  or  rather  a  jerky,  irregular  action  of  the  hands, 
appears,  which  is  increased  by  effort  both  to  hold  them  still  and  to 
perform  any  fine  motion.  The  rate  of  the  tremor  is  from  five  to  seven 
per  second.  This  is  termed  by  the  Germans  an  "intentional  tremor." 
It  does  not  occur  when  the  hands  are  at  rest,  but  gets  worse  and  worse 
as  they  are  used,  the  motions  being  more  and  more  extensive,  until 
finally  the  patient  makes  irregular,  jerky  motions  and  cannot  attain 
his  end.  When  he  ceases  to  try  the  tremor  stops.  Thus  he  spills  all 
the  water  from  a  glass  in  attempting  to  drink,  and  is  unable  to  adjust 

^Kev.  Neurologique,  1900,  vol.  viii.,  p.  825, 

^DqxxL  Zeitschr.  f.  Nervenheilk.,  1902,  Bd.  xxi.,  p.  1. 


SYMPTOMS.  681 

his  clothing  or  to  write.  The  entire  arm  and  forearm  share  in  this 
tremor,  ©ccasionally  this  tremor  is  confined  to  one  side.  The  arms 
may  become  weak  and  stiff,  but  this  is  rare. 

At  the  same  time  a  marked  defect  in  speech  develops,  known  as 
scanning  speech.  It  is  a  slow,  jerky  utterance  of  words,  with  pauses 
between  words  or  between  syllables,  and  a  forced  effort  to  pronounce, 
which  leads  to  unusual  accentuation  and  possibly  to  a  clipping  of  words, 
so  that  they  are  not  fully  uttered.  There  may  be  also  a  tremor  of  the 
face  and  of  the  tongue,  and  tremor  of  the  vocal  cords  has  been  seen  by 
the  laryngoscope.  Sometimes  there  is  a  tremor  or  queer,  jerky  move- 
ment of  the  head  which  can  be  produced  if  the  patient  looks  up  or 
turns  his  head.  This  may  occur,  like  a  senile  nodding,  when  the 
patient  is  at  rest,  the  mere  act  of  supporting  the  head  being  enough  of 
a  voluntary  act  to  start  the  tremor. 

Nystagmus  on  lateral  or  upward  movement  of  the  eyeballs  appears 
early,  and  later  may  occur  when  no  motion  is  made.  This  is  found  in 
75  per  cent,  of  the  cases.  •  The  pupils  are  often  unequal  and  may  be 
contracted,  but  always  respond  to  light.  Strabismus,  due  to  a  weak- 
ness of  one  or  more  ocular  muscles,  usually  the  externi,  is  an  occasional 
symptom.  This  may  only  become  evident  on  conjugate  movements  of 
the  eyes  to  one  side.  Temporary  attacks  of  blindness  may  occur, 
which  are  often  followed  by  a  permanent  condition  due  to  optic  atrophy ; 
in  fact,  optic  atrophy  may  be,  as  in  tabes,  the  earliest  symptom,  as 
shown  by  cases  of  Bruns  and  Stolling,^  and  is  found  in  a  large  number 
of  cases  (52  per  cent.,  Utthoff).  It  is  usually  a  partial  atrophy  and 
causes  a  narrowing  of  the  visual  field  or  scotoma  rather  than  total 
blindness.  The  outer  or  the  inner  halves  of  the  disk  are  pale,  or  the 
entire  disk  may  be  white  and  sharply  defined.  There  is  also  a  change 
in  color  vision,  red  and  green  being  lost  before  blue  and  yellow,  which 
is  the  reverse  of  the  order  observed  in  hysteria.  It  is  a  peculiarity  of 
the  defects  of  vision  in  multiple  sclerosis  that  they  vary  from  day  to 
day,  at  times  disappearing  entirely. 

Vertigo  is  a  common  symptom,  deafness  is  rare.  Mental  disturb- 
ance is  frequently  observed.  There  is  a  sense  of  well-being  and  a 
boastfulness  without  delusions  which  suggests  paresis.  There  is  a  lack 
of  control  of  the  emotions.  At  times  there  is  involuntary  laughing, 
which  is  causeless  and  which  annoys  the  patient,  but  cannot  be  con- 
trolled. There  is  imperfect  memory  and  a  manifest  weakening  in  the 
power  of  reasoning  and  of  judgment.  In  some  cases  (25  per  cent.) 
attacks  of  epilepsy  or  of  apoplexy  with  subsequent  aphasia  or  hemi- 
plegia occur.  Parsesthesia  is  occasionally  complained*  of,  but  anaes- 
thesia is  rarely  observed.     Pains  sometimes  give  the  patients  distress. 

In  some  cases  the  spastic  paraplegia  with  atrophy  of  the  muscles, 
loss  of  control  of  the  sphincters,  ataxia,  trophic  disturbances  in  the 
joints  and  skin,  and  bed-sores  make  it  evident  that  the  spinal  cord  is 
the  part  chiefly  involved,  and  in  these  cases  the  other  symptoms  of  bul- 
bar and  cerebral  nature  may  be  slow  in  developing,  or  may  not  occur 
before  the  patient  dies. 

^Monatsschr.  f.  Psych,  u.  Neurologic,  1900,  Bd.  vii.,  p.  89. 


682  MULTIPLE  SCLEUOSIS. 

In  some  cases  the  cerebral  symptoms  are  the  first  to  appear,  espe- 
cially scanning  speech  and  hemiplegic  attacks  of  temporary  duration. 
It  is  in  these  patients  that  the  diagnosis  may  Ije  made  of  paresis  or  of 
cerebral  softening,  and  only  after  months  or  years,  when  the  bulbar 
and  spinal  symptoms  develop,  is  the  right  diagnosis  reached.  The  fact 
that  other  symptoms  of  paresis  do  not  aj^pear  within  a  year  of  the 
onset  and  that  the  physical  signs  of  paresis  are  absent  may  aid  in  the 
differentiation  between  the  two  diseases. 

In  a  third  type  of  cases  the  bulbar  symptoms  precede  all  others,  and 
difficulty  of  swallowing,  paralysis  of  the  vocal  cords,  atrophy  of  the 
tongue,  polyuria,  and  glycosuria,  and  attacks  of  suffocation  may  occur. 

The  course  of  the  disease  varies  greatly.  In  some  cases  there  is  a 
slow  but  continuous  progress  in  the  symptoms,  and  after  some  years 
the  patient  becomes  helpless,  and  finally  dies  of  some  intercurrent  dis- 
ease. In  these  cases  the  typical  symptoms  are  so  evident  that  the 
diagnosis  is  simple.  In  other  cases  there  is  a  slow  onset  of  the  spinal 
symptoms  which  increase  in  intensity,  and  there  are  sudden  attacks  of 
an  epileptic  or  apoplectic  type,  or  sudden  attacks  of  blindness  which 
may  subside.  In  still  other  cases  there  are  remissions  in  all  the  symp- 
toms which  may  be  so  complete  as  to  lead  to  the  hope  of  recovery,  and 
recurrence  takes  place  only  after  some  months  or  even  years.  Buzzard  ^ 
who  has  recently  called  attention  to  the  frequency  of  an  intermittent 
course  of  the  symptoms  reports  a  case  in  which  the  symptoms  appeared 
five  times  in  the  same  patient,  each  time  following  a  pregnancy  and 
each  time  subsiding  entirely  after  several  months'  duration.  In  other 
cases  the  onset  is  rapid,  many  symptoms  appearing  in  the  course  of  a 
few  weeks,  subsiding  and  recurring  in  a  series  of  attacks.  And  lastly, 
there  are  cases  which  are  said  to  go  on  to  complete  recovery  by  gradual 
subsidence  of  all  the  symptoms.  Patients  have  died  within  two  years 
of  the  onset.  Others  have  lived  for  twenty  years  or  more.  It  is  thus 
evident  that  the  multiplicity  of  the  symptoms  and  their  very  irregular 
course  makes  any  attempt  to  draw  a  picture  of  multiple  sclerosis  quite 
hazardous.  It  is  also  evident  that  a  diagnosis  can  only  be  reached  in 
the  obscure  cases  by  careful  and  long  observation  and  by  an  attempt 
to  eliminate  all  other  organic  and  also  some  functional  diseases. 

The  interest  which  unusual  cases  of  disease  excites  has  led  to  the 
report  of  many  cases  of  multiple  sclerosis  in  literature.  As  a  fact,  it 
is  a  very  rare  afiection.  In  my  clinic  but  109  cases  have  been  seen 
among  31,502  patients,  and  in  my  private  practice  the  ratio  is  about 
the  same.^  Spiller  has  confirmed  its  rarity  in  this  country  from  a 
study  of  autopsy  records  in  Philadelphia  as  well  as  from  his  large 
clinical  observation.  It  is  as  common  as  tabes  in  Germany  and  in 
England.  Hence  too  great  importance  should  not  be  attached  to  the 
space  occupied  by  the  disease  in  foreign  literature. 

In  one  patient,  a  male  aged  thirty-five  years,  no  cause  could  be  as- 
certained. The  first  symptom  noticed  was  a  staggering  gait  quite  like 
that  of  a  drunken  man,  which  was  not  increased  by  closing  the  eyes 

iThe  Lancet,  July  16,  1904. 

2  JellifFe,  Journal  of  Nervous  and  Mental  Disease,  July,  1904. 


SYMPTOMS.  e^a 

and  was  not  accompanied  by  any  change  in  the  knee-jerks.  After  this 
had  been  present  for  some  months  the  man's  friends  noticed  a  change 
in  his  speech  which  soon  became  typically  scanning  in  character.  Then 
it  was  noticed  that  there  was  a  change  in  his  manner,  so  that  he  gave 
the  impression  of  being  weak-minded  and  silly,  laughed  at  everything 
which  was  said  to  him,  and  did  not  appear  to  appreciate  his  condition. 
Examination  showed  lateral  nystagmus  but  no  optic  atrophy  or  ocular 
palsy  and  no  actual  paralysis.  These  symptoms  had  been  coming  on 
for  a  year  at  the  time  I  saw  him,  and  had  not  been  intermittent,  and 
were  not  affected  by  treatment. 

In  a  second  patient,  male,  aged  twenty -four  years,  no  cause  could 
be  found.  The  first  symptom  was  vertigo,  which  was  increased  by 
walking,  and  caused  an  irregular  ataxic  gait.  Soon  after  a  tremor 
began  in  the  left  hand  which  soon  extended  to  the  left  leg  and  increased 
his  ataxic  gait,  so  that  by  the  end  of  six  months  he  staggered  constantly 
and  could  hardly  dress  himself.  This  tremor  was  increased  by  any 
mental  effort,  by  emotion,  or  by  any  active  movement.  Ten  months 
after  the  onset  he  noticed  a  disturbance  of  vision  which  was  not  due  to 
optic  atrophy,  but  was  produced  by  a  lateral  nystagmus,  which  was 
constant  in  looking  at  any  object  or  on  turning  his  eyes.  About  the 
same  time  typical  scanning  speech  was  noticed  by  his  wife.  This 
patient  had  no  sensory  disturbance,  no  paralysis,  and  no  mental  symp- 
toms, but  all  his  symptoms  were  rapidly  increasing  in  intensity. 

A  third  patient,  a  woman  aged  twenty-five  years,  had  suffered  for 
three  years  when  first  seen.  Her  symptoms  began  with  tremor,  first 
noticed  in  her  head  and  neck,  then  in  her  hands,  and  finally  in  her 
body  and  legs,  which  was  followed  by  the  slow  development  of  a 
spastic  gait  attended  by  increased  knee-jerks.  During  the  third  year 
lateral  nystagmus  and  scanning  speech  appeared.  Her  pupils  and  op- 
tic nerves  were  normal,  and  she  had  no  mental  symptoms,  and  her 
condition  had  remained  stationary  for  some  months  when  last  seen. 

A  fourth  patient,  a  woman  aged  thirty  years,  had  suffered  for  four 
years.  She  had  always  been  delicate,  though  she  came  of  strong  stock 
and  had  no  illness  prior  to  this  one.  Her  symptoms  began  with  stiff- 
ness and  weakness  of  the  legs,  which  in  six  months  had  developed  into 
a  spastic  paraplegia  with  extreme  contractures  and  loss  of  control  of 
the  sphincters.  Then  severe  vomiting  and  indigestion  began,  which 
weakened  her  greatly,  and  for  some  time  she  had  to  be  nourished  by 
enemata.  Then  for  three  months  she  lay  in  a  state  of  semiconscious- 
ness, being  fed  by  rectum,  her  bowels  and  bladder  being  evacuated 
artificially.  Of  this  period  she  had  no  recollection,  and  it  was  with 
difficulty  that  she  was  kept  alive.  This  condition  and  the  paralysis 
were  supposed  to  be  hysterical,  and  an  attempt  was  made  by  orthopedic 
apparatus  to  extend  the  contractured  legs  and  to  get  her  to  walk. 
During  this  treatment,  which  lasted  six  months,  her  hands  began  to 
tremble,  her  speech  l)ecame  scanning,  her  eyesight  became  poor,  and 
she  saw  double.  Attempts  to  stand  caused  great  shaking  of  the  entire 
body  and  head,  but  became  more  and  more  successful,  so  that  she  was 
able  to  walk  with  the  snj)|)ort  of  two  persons,  and  recover/sd  control 


6S4  MULTIPLE  SCLEROSIS. 

of  her  sphincters.  After  three  months  of  a  fair  degree  of  comfort  her 
memory  began  to  fail  and  her  control  over  her  emotions  was  much 
weakened.  Then  the  intentional  tremor  increased  rapidly,  so  that  no 
voluntary  movement  was  possible.  There  was  no  paralysis  of  the 
limbs  when  I  saw  her.  There  was  a  loss  of  knee-jerks,  nystagmus, 
paralysis  of  the  left  external  rectus,  no  loss  of  pupil  reflex,  vision  was 
perfect,  there  was  marked  tremor  of  the  intention  type,  and  typical 
scanning  speech.     Her  manner  was  childish  and  hysterical. 

Diagnosis. — The  diagnosis  from  locomotor  ataxia,  spastic  para- 
plegia ataxic  paraplegia,  and  amyotrophic  lateral  sclerosis  is  to  be 
made  (1)  by  the  appearance  of  bulbar  symptoms  and  cerebral  symp- 
toms, which  do  not  occur  in  these  diseases  —  e.  g.,  scanning  speech, 
nystagmus,  and  lack  of  mental  activity,  and  an  emotional  state ;  (2) 
by  the  lack  of  many  symptoms  which  are  characteristic  of  these 
diseases  ;  (3)  by  the  course  of  the  case  with  remissions  which  do  not 
occur  in  these  affections.  The  diagnosis  from  cerebral  disease,  from 
paresis,  multiple  softening  due  to  atheroma  and  brain  tumor  is  to  be 
made  by  the  presence  of  intentional  tremor  and  a  scanning  speech  which 
does  not  resemble  the  trembling,  indistinct  pronunciation  of  the 
paretic ;  the  absence  of  severe  dementia ;  the  absence  of  delusions ; 
the  absence  of  headache,  vomiting,  and  choked  disks ;  the  preserva- 
tion of  pupil  action ;  the  intermittence  of  the  symptoms,  and  the 
irregular  course  of  the  case.  Toxic  tremors  and  paralysis  agitans 
never  resemble  intentional  tremor  and  lack  all  other  symptoms  of 
multiple  sclerosis.  The  diagnosis  from  hysteria  may  be  at  all  times 
difficult,  especially  as  hysterical  manifestations  frequently  occur  in  the 
course  of  multiple  sclerosis.  Both  diseases  occur  in  young  persons, 
though  hysteria  is  more  common  in  women  and  multiple  sclerosis  in 
men.  Both  diseases  may  follow  an  emotional  shock,  or  a  trauma,  or 
an  infectious  disease.  Both  present  a  multitude  of  incongruous  symp- 
toms which  come  and  go  suddenly  without  apparent  reason,  and 
manifest  great  changes  in  character  and  course.  Scanning  speech  and 
intentional  tremor  have  been  seen  in  hysteria.  Nystagmus  is  never 
an  hysterical  symptom  ;  optic  atrophy  is  never  hysterical ;  the  peculiar 
oscillation  of  the  head  and  body  is  not  seen  in  hysteria,  and  loss  of 
control  of  the  sphincters  is  never  hysterical.  In  the  majority  of  cases 
a  study  of  the  individual,  her  heredity,  nature,  character,  and  her 
surroundings,  and  the  discovery  of  the  stigmata  of  hysteria  will  enable 
a  diagnosis  to  be  reached,  though  Buzzard,  Oppenheim,  and  every 
specialist  has  been  obliged  to  confess  to  occasional  mistakes  in  diagnosis 
which  were  apparently  inevitable.  The  knowledge  of  the  possibility 
of  such  mistakes  should  intensify  the  effort  to  obtain  objective  proof 
of  the  existence  of  multiple  sclerosis.  So  long  as  subjective  symp- 
toms only  are  present  or  symptoms  which  can  be  voluntarily  simulated 
no  one  can  be  certain  of  a  diagnosis. 

There  is  a  condition  described  by  Westphal  as  "  pseudo-sclerosis  " 
which  presents  symptoms  almost  identical  with  multiple  sclerosis,  yet 
in  which  no  lesions  have  been  found.  In  this  condition  the  mental 
symptoms,  delirium  and  an   apathetic    state  going   on   to  moderate 


TREATMENT.  685 

dementia  appear  early  and  are  more  marked  than  in  multiple  sclerosis ; 
the  tremor  is  a  slow  one,  the  movements  being  but  two  or  three  to  the 
second,  and  may  occur  during  rest ;  all  movements,  even  speech,  are 
unusually  deliberate ;  nystagmus  and  optic  atrophy  do  not  develop. 
Marie  considers  this  disease  of  Westphal  as  a  form  of  hysteria,  but 
Striimpell,  who  has  studied  two  cases,  supports  Westphal's  contention 
that  it  is  a  neurosis,  not  hysterical  in  origin.  It  is  not  improbable  that 
new  histological  methods  may  reveal  a  lesion  as  yet  not  discovered  and 
as  widespread  as  is  multiple  sclerosis. 

The  diagnosis  of  multiple  sclerosis  from  Marie's  form  of  hereditary 
cerebellar  ataxia  requires  consideration,  since  both  diseases  develop  in 
youth,  both  are  attended  by  ataxia,  nystagmus  and  tremor  of  the  head, 
body,  and  limbs,  and  by  mental  failure.  In  multiple  sclerosis  some 
spastic  paraplegia  usually  precedes  the  ataxia;  this  is  not  often  of 
cerebellar  type,  and  is  attended  by  exaggerated  reflexes.  In  multiple 
sclerosis  optic  atrophy  is  common,  but  it  has  not  been  observed  in 
Marie's  disease.  The  course  of  the  disease  is  steadily  progressive  in 
Marie's  disease,  while  in  multiple  sclerosis  intermissions  and  remissions 
are  the  rule. 

The  diagnosis  of  multiple  sclerosis  from  cerebro-spinal  syphilis  is 
discussed  in  the  next  chapter  on  page  696. 

Prognosis.  — The  prognosis  in  multiple  sclerosis  is  unfavorable  for 
recovery,  but  the  condition  does  not  make  rapid  progress,  and  as 
remissions  in  the  symptoms  occur  spontaneously  some  relief  from  time 
to  time  may  be  promised.  In  a  few  cases  the  symptoms  have  subsided 
and  have  not  returned  for  several  years.  The  cases  which  have  been 
reported  as  cured  belong  to  this  form.  The  earlier  its  development  in 
life  the  better  is  the  chance  of  its  arrest.  Even  blindness  has  been 
known  to  subside,  and,  as  the  optic  atrophy  is  never  total,  some 
degree  of  vision  may  be  assured.  The  appearance  of  bulbar  symp- 
toms should  always  give  rise  to  apprehension,  as  some  cases  of  rapidly 
fatal  termination  from  paralysis  of  deglutition  or  from  respiratory 
failure  have  been  recorded. 

Treatment.  —  It  is  essential  that  patients  should  avoid  physical 
exertion,  and  a  rest  cure  often  helps  greatly  to  arrest  the  disease. 
Even  voluntary  movements  of  the  hands  and  too  much  talking  may 
be  avoided.  The  nutrition  must  be  kept  at  the  best  possible  point, 
fresh  air,  good  food,  tepid  or  alcohol  sponge  baths,  massage,  and  all 
kinds  of  tonics  being  employed  to  increase  the  store  of  energy.  As 
a  rule,  very  hot  baths  are  to  be  avoided,  also  cold  shocks.  A  daily 
bath  at  90°  F.,  followed  by  rubbing,  and  salt  baths  at  95°  F.  are  of 
much  service.  These  patients  do  well  at  Nauheim  and  in  water-cure 
establishments.  General  galvanization  has  been  thought  to  be  of  use. 
In  regard  to  the  use  of  drugs,  I  have  seen  apparent  benefit  follow  the 
use  of  arsenic,  quinine,  and  salicin,  and  would  urge  these  remedies, 
which  may  be  used  in  succession  two  weeks  at  a  time.  Cod-liver  oil 
is  also  of  service.  There  is  little  effect  to  be  expected  from  the  use 
of  mercury  or  of  iodide  of  potassium.  Nitrate  of  silver,  once  highly 
praised,  has  been  abandoned. 


CHAPTER  XXXyil. 

SYPHILIS   OF   THE   NERVOUS   SYSTEM. 

While  it  must  be  admitted  that  syphilis  is  the  cause  of  many  forms 
of  nervous  disease ;  neuritis,  cerebral  and  spinal  affections,  and  func- 
tional disturbances,  it  is  also  to  be  born6  in  mind  that  there  is  nothing 
pathognomonic  about  syphilitic  nervous  diseases.  If  therefore  one  at- 
tempted to  describe  such  diseases  as  a  separate  entity,  one  would  be 
obliged  to  restate  many  facts  elsewhere  presented  in  this  work,  view- 
ing them  therefore  from  one  side  only.  In  the  consideration  of  all 
the  organic  diseases  of  the  nervous  system  already  studied  attention 
has  been  directed  in  the  sections  on  Etiology  to  syphilis  as  a  cause  of 
the  disease  in  question.  And  in  the  section  upon  symptoms  the 
variations  in  these  symptoms  due  to  the  syphilitic  element  of  the 
disease  has  been  emphasized.  The  question  of  the  treatment  of  the 
syphilitic  element  has  also  been  fidly  discussed  in  connection  with  each 
disease.  On  the  other  hand,  there  are  many  interesting  facts  in  con- 
nection with  the  syphilitic  element  in  these  diseases  which  need  to  be 
mentioned.  In  this  chapter  some  of  these  facts  are  considered,  refer- 
ence however  being  constantly  made  to  other  sections  of  this  book  in 
which  the  symptomatology  and  diagnosis  of  the  different  diseases  are 
more  fully  set  forth. 

Nervous  manifestations  develop  in  about  ten  per  cent,  of  cases  of 
syphilis,  as  shown  by  the  study  of  statistics  from  large  clinics  and 
hospitals.  These  manifestations  may  appear  within  a  few  months  of 
the  infection  or  they  may  not  develop  until  many  years  have  elapsed. 
In  some  cases  the  nervous  disease  is  a  result  of  inherited  syphilis. 

It  has  been  noticed  by  many  authorities  that  nervous  symptoms 
appear  more  commonly  in  those  who  have  suffered  least  at  the  onset, 
and  a  mild  attack  of  syphilis,  or  a  thorough  course  of  treatment  in 
the  first  and  second  stages,  does  not  exempt  the  victim  from  serious 
nervous  diseases,  as  a  tertiary  manifestation.  In  fact  in  some  cases 
these  nervous  diseases  develop  even  during  a  course  of  antisyphilitic 
treatment. 

Pathology.  —  The  pathological  changes  which  are  found  in  syphilis 
of  the  nervous  system  are  of  three  kinds  : 

I.  Syphilitic  endarteritis  with  consequent  malnutrition  and  later 
with  thrombosis  and  consequent  softening ; 

II.  Syphilitic  exudations  about  the  bloodvessels  in  the  meninges 
with  the  production  of  gummy  tumors,  often  multiple  ;  and 

III.  Syphilitic  chronic  hyperplastic  inflammation  with  cellular  infil-. 
tration  of  the  membranes  and  of  the  brain,  spinal  cord  or  nerves, 

686 


PATHOLOGY. 


687 


These  three  conditions  are  very  frequently  combined.  In  fact  it  is 
the  excepti(/n  to  find  at  an  autopsy  one  of  these  alone,  and  hence  it  is 
rarely  possible  to  draw  a  conclusion  as  to  the  pathological  condition 
present  from  a  study  of  the  symptoms.  In  the  majority  of  cases  which 
are  examined  after  death  an  extensive  endarteritis  is  present,  infiltra- 
tions and  exudations  about  the  vessels  and  in  the  nervous  substance  are 
found  and  gummata  exist. 

In  addition  to  these  active  manifestations  of  syphilis  in  the  nervous 
system  characterized  by  objective  lesions,  we  also  have  to  admit  the 
existence  of  a  number  of  postsyphilitic  or  parasyphilitic  diseases  of  a 
most  serious  type,  without  any  characteristic  pathology.     These  are 

Fig.  288. 


Sections  through  a  group  of  cerebral  arteries  showing  endarteritis  syphilitica  in  various 
stages.    Thrombi  are  seen  in  two  of  the  vessels. 

paresis,  tabes,  and  spastic  paraplegia,  all  of  which  can  in  the  large 
majority  of  instances  be  ascribed  to  the  disease.  They  are  due  to  de- 
generative processes  in  the  neurons,  whether  as  the  result  of  the  direct 
action  of  the  microorganism,  or  of  the  toxin,  or  of  the  antibodies,  or  of 
some  byproducts  of  the  micro5rganism,  or  of  some  antitoxin  produced 
in  the  body  in  its  effort  to  destroy  the  spirochseta  pallida  we  cannot 
yet  affirm. 

The  determination  of  the  syphilitic  element  as  a  factor  in  any  of 
these  various  forms  of  nervous  disease  can  now  be  made  by  the  dis- 
covery of  lymphocytes  or  of  an  increase  of  globulin^  in  the  cerebro-spinal 


688  SYPHILIS   OF   THE   NEBVOUS   SYSTEM. 

fluid  obtained  by  lumbar  puncture,  and  also  by  the  Wassermann  test 
of  the  blood.  And  as  therapeutic  measures  must  be  guided  by  this 
determiuation,  as  well  as  the  prognosis  modified  in  any  one  case,  the 
importance  of  these  tests  cannot  be  overestimated. 

Noguchi  has  made  an  interesting  comparative  study  of  the  Wasser- 
mann  reaction,  the  butyric  acid  test  and  the  cytological  formula  as 
applied  to  the  cerebro-spinal  fluid  in  various  forms  of  syphilis  of  the 
nervous  system. 

Wassennann        Butyric  Cytological 

Reaction.  Acid  Eeaction.  Formula. 

Hereditary  syphilis 80%                90%  .   .    . 

Cerebro-spinal  syphilis 50%              100%  ... 

General  paresis 73%                90%  91% 

Tabes 52%               100%  100% 

Fig.  289. 


Vein  in  pia  mater  with  swollen  and  infiltrated  wall.    Eosin-methylene-blue  stain. 
Magnification  175.     (Barrett.) 

In  tuberculous  cases  search  must  be  made  for  the  bacillus  and  the 
Wasserraann  test  applied  to  both  the  spinal  fluid  and  the  blood  serum. 

These  are,  in  brief,  the  means  by  which  we  make  our  diagnosis  in 
syphilis. 

I.  Syphilitic  Endarteritis  and  its  Results  on  the  Nervous  System.  — 
Syphilitic  endarteritis  produces  a  progressive  diminution  in  the  calibre 
of  the  bloodvessels,  both  in  the  arteries,  as  Huebner  has  shown,  and  in 
the  veins,  as  Reeder  has  shown.  This  has  already  been  described  on 
page  469.  The  decreased  supply  of  blood  consequent  upon  the  ob- 
struction to  its  flow  causes  a  state  of  malnutrition  of  the  part  of  the 
nervous  system  supplied  by  the  vessels  affected,  and  eventually  leads 


PATEOLOGT.  689 

to  thrombosis  in  those  vessels,  followed  by  a  localized  softening  in  the 
area  cut  off*  from  its  blood  supply.  The  pathology  of  a  syphilitic  soft- 
ening differs  in  no  way  from  that  produced  by  embolism  or  thrombosis 
of  non-syphilitic  origin  (see  page  476  and  Figs.  221  to  223). 

The  symptoms  of  this  condition  are,  in  the  early  stage,  those  of 
imperfect  function  of  the  brain  or  of  the  spinal  cord.  The  patients 
present  at  first  symptoms  of  neurasthenia,  viz.,  headache,  vertigo,  in- 
somnia, inability  to  work  or  to  concentrate  the  attention,  defects  of 
memory  and  irritability  of  temper.  Later  they  have  slight  temporary 
suspensions  of  function,  such  as  partial  aphasia,  numbness  in  one  limb 
or  in  one  half  of  the  body,  double  vision,  or  staggering  gait.  Stimu- 
lants to  the  heart  relieve  these  symptoms  temporarily,  but  after  a 
series  of  such  prodromata  an  apoplectic  attack  occurs.  The  various  types 
of  apoplectic  attack  have  been  already  described  on  page  483.  An  hemi- 
plegic  or  aphasic  attack  in  a  person  between  twenty  and  thirty-five  is 
usually  due  to  syphilitic  endarteritis  with  thrombosis.  It  may  be  less 
severe  than  an  ordinary  attack  of  apoplexy  and  there  is  usually  no  loss 
of  consciousness.  It  is  not  infrequently  attended  by  a  general  convul- 
sion. Sometimes  a  state  of  somnolence  may  last  for  several  days  and  the 
mind  be  much  disturbed  and  bewildered.  The  subsequent  course  of  the 
disease  resembles  that  in  an  ordinary  apoplexy,  and  the  fact  that  the 
origin  of  the  attack  is  a  specific  endarteritis  does  not  improve  the  prog- 
nosis. Antisyphilitic  treatment  will  not  benefit  the  condition,  although 
it  may  be  indicated  to  prevent  other  attacks. 

There  are  other  cases  which  do  not  present  any  apoplectic  attacks, 
but  which  show  all  the  evidences  of  small  localized  areas  of  softening 
in  the  brain  already  described  in  the  section  on  arterial  sclerosis  (page 
468).  A  condition  quite  similar  to  that  seen  in  senile  dementia 
(page  470)  may  develop  in  these  cases,  with  a  chronic  course. 

If  the  endarteritis  affects  the  spinal  bloodvessels  there  occurs  a  pro- 
dromal period  of  indefinite  spinal  pain,  sensations  of  numbness  in  the 
legs  and  about  the  trunk,  occasional  difficulty  in  the  control  of  the 
sphincters,  and  a  delay  or  loss  of  the  sensation  of  pain  in  the  legs. 
Then  a  sudden  attack  of  paraplegia  ensues  with  all  the  symptoms  of  an 
acute  myelitis  (see  page  403}.  The  course  of  such  a  case  is  slow  and 
the  prospect  of  recovery  is  bad.  Here  again  antisyphilitic  treatment 
has  no  effect  in  restoring  the  function  of  degenerated  nervous  tissues. 
For  more  complete  details  of  the  effects  of  syphilitic  endarteritis,  the 
reader  is  referred  to  the  chapter  on  vascular  diseases  of  the  brain  (page 
468)  and  upon  myelomalacia  (page  400). 

II.  Syphilitic  Exudations.  —  Syphilitic  exudations  about  the  blood- 
vessels and  in  the  meninges  of  the  brain  and  spinal  cord  are  very 
common  as  a  sequel  of  syphilitic  disease,  especially  as  an  early  mani- 
festation, cases  having  been  observed  within  six  months  of  the  initial 
lesion.  They  may  occur  as  a  diffuse  glue-like  mass  over  a  large  area 
of  the  surface  or  base  of  the  brain  or  cord  as  shown  in  Figs.  290  and 
291,  or  they  may  be  more  distinctly  encapsulated,  forming  a  definite 
gummy  tumor,  and  such  gummy  tumors  are  often  multiple.  The  glue- 
44 


690 


SYPHILIS   OF   THE   NEBVOUS  SYSTEM. 


like  substance  is  deposited  rapidly  and  extensively  through  the  meninges 
and  produces  pressure  upon  the  subjacent  brain,  or  cranial  nerves, 
or  spinal  cord  and  nerves,  and  upon  the  bloodvessels.  It  therefore 
causes  a  suspension  of  function  both  of  the  tissues  compressed  directly 
and  of  regions  at  a  distance  when  their  blood  supply  is  cut  off. 

The  usual  history  of  a  case  of  brain  syphilis  is  as  follows  :  After  a 
period  of  several  weeks  of  indefinite  nervousness  with  inability  to  work, 

Fig.  290. 


Multiple  gummata  upon  the  base  of  the  brain  and  about  the  cerebellum.     (Siemerling  ) 


discomfort  and  restlessness  in  the  late  afternoon,  with  dull  headache 
worse  at  night,  and  sleeplessness,  the  patient  is  suddenly  seized  with 
very  intense  headache,  is  dizzy  and  sees  double.  He  may  have  numb- 
ness in  one  side  of  the  body  and  a  feeling  of  incapacity  for  all  thought 
or  eflPort.  On  examination  a  third  nerve  palsy  may  be  discovered,  or 
a  beginning  optic  neuritis  and  possibly  a  slight  hemiplegia  or  hemi- 
ansesthesia.     An  epileptiform  attack  occasionally  precedes  the  onset  or 


PATHOLOGY. 


691 


may  occur  after  other  symptoms  have  appeared.  The  condition  if 
promptly  treated  soon  begins  to  improve,  the  ptosis  and  strabismus 
subside,  and  in  four  or  six  weeks  the  patient  has  no  objective  signs  of 
the  attack  and  is  free  from  symptoms.  If  it  is  not  treated  the  symptoms 
of  brain  tumor  upon  the  base  ensue.  This  is  the  usual  history  of  an 
exudation  of  gummy  nature  upon  the  base  of  the  brain  about  the  crura, 
its  point  of  most  common  occurrence. 

The  frequency  of  oculo-motor  palsy  as  an  evidence  of  cerebral 
syphilis  has  been  noted  by  all  writers.  Graefe  stated  that  more  than 
a  half  of  all  oculo-motor  palsies  are  of  syphilitic  nature.  The  nerves 
supplying  the  eyeballs  have  a  long  course  upon  the  base  of  the  brain 
and  any  exudation  there  is  liable  to  affect  them.  Irregularity  of  the 
pupils,  a  loss  of  reaction  to  light,  ptosis,  strabismus  from  partial 
paralysis  of  single  eye  muscles,  or  all  the  symptoms  of  a  third  nerve 
or  sixth  nerve  paralysis  may  develop.  In  259  cases  of  brain  syphilis 
Uhthoff  found  the  oculo-motor  nerve  affected  in  96  cases.     Not  infre- 

FiG.  291. 


•X* 
t 


Gumma  lying  on  the  surface  of  the  cerebrum.    From  an  exudation  on  the  under  surface  of  the 

dura.     (Larkin.) 


quently  both  third  nerves  are  affected.  Double  sixth  nerve  palsy  is 
very  rare.  Such  palsies  usually  subside  rapidly  under  treatment  and 
leave  no  permanent  effects. 

Or  a  patient  may  suffer  from  gradually  increasing  localized  headaches 
or  diffuse  pain  over  the  entire  head,  which  may  last  for  weeks  without 
further  manifestation  ;  or  may  be  followed  by  a  gradually  increasing 
mental  dullness  going  on  even  to  dementia.  Changes  in  the  pupils, 
inequality,  or  sluggish  action   or  failure  to  contract  to  light  are  often 


692 


SYPHILIS   OF   THE  NEEFOUS  SYSTEM. 


found,  and  sometimes  an  optic  neuritis  appears.  Occasionally  epileptic 
convulsions  occur,  either  localized  Jacksonian  attacks  or  general  con- 
vulsions. These  may  be  the  only  symptoms  of  a  general  syphilitic 
meningitis  of  the  convexity.  If  to  this  are  added  gummy  exudations, 
the  local  signs  of  brain  tumor  may  appear. 

The  pressure  of  syphilitic  exudations  upon  the  bloodvessels  of  the 
brain  adds  to  the  complexity  of  the  symptoms,  often  causing  pseudo- 
apoplectic  attacks. 

Fig.  292. 


Syphilitic  meningitis  of  spinal  cord.  Section  from  tlie  midthoracic  region  of  the  spinal  cord,  show- 
ing the  thickening  of  the  membranes  and  their  adhesion  to  the  cord,  a,  a,  a,  a,  a,  degeneration  of  the 
peripheral  parts  of  the  cord.     (Spiller.) 

In  syphilitic  exudations  within  the  Sylvian  fissure  the  pressure  may 
produce  a  stasis  in  the  Sylvian  artery,  causing  a  sudden  hemiplegic  and 
aphasic  attack  and  convulsions.  If  the  basilar  artery  is  compressed, 
very  irregular  symptoms  of  bulbar  palsy  appear  and  a  fatal  termination 
may  follow.  Yet  even  in  these  cases  recovery  is  possible  if  under 
brisk  treatment  the  exudation  is  absorbed  and  the  pressure  removed. 
I  have  seen  one  case  of  total  aphasia  and  hemiplegia  recover  entirely, 
although  the  pressure  symptoms  remained  a  week.  I  have  seen  a  case 
presenting  all  the  signs  of  acute  bulbar  paralysis  with  a  state  of  coma 
lasting  ten  days  followed  by  complete  recovery.  I  have  seen  a  case 
with  all  the  symptoms  of  brain  tumor  attended  by  unilateral  and  gen- 
eral convulsions,  reaching  the  number  of  eighty  attacks  in  one  day 
which  entirely  recovered.  Such  facts  make  it  evident  that  a  brisk 
antisyphilitic  treatment  may  cure  even  in  desperate  cases. 


PATHOLOGY.  693 

The  usual  history  of  a  case  of  syphilis  of  the  cord  is  as  follows : 
After  a  period  of  severe  backache  the  patient  notices  a  difficulty  in 
passing  water  and  an  occasional  leakage  of  urine ;  a  loss  of  sensation 
to  cold,  and  an  inability  to  distinguish  diifereuces  of  temperature  in  the 
legs  and  body.  Soon  he  feels  a  girdle  sensation  and  has  sharp  shooting 
pains  in  the  limbs.  Then  his  movements  become  stiif  and  weak, 
motions  of  the  spine  increase  both  the  backache  and  the  sharp  pains, 
an  increase  of  reflexes  is  discovered,  and  some  general  anaesthesia  in 
the  legs,  a  loss  of  vibratory  sensation  or  of  sensation  of  cold,  and  impo- 
tence. In  the  course  of  a  week  or  two  partial  paraplegia  develops, 
usually  with  considerable  pain  in  the  back,  especially  in  the  dorsal 
region,  the  arms  being  exempt.  This  history  indicates  the  develop- 
ment of  an  acute  meningo-myelitis  with  gummy  exudations  about  the 
dorsal  region  of  the  cord,  and  a  cell  infiltration  about  the  periphery  of 
the  cord,  causing  degeneration  in  various  columns.  At  this  point  by 
active  treatment  the  progress  may  be  arrested  and  a  progressive  im- 
provement results.  Complete  recovery  may  ensue,  but  is  rare,  and 
patients  usually  remain  stiif  and  weak  in  their  legs  and  back,  and  sub- 
ject to  parsesthesise  and  often  unable  to  control  the  bladder  and  rectum 
completely.     Their  gait  is  spastic. 

Sometimes  the  lesion  is  limited  to  the  meninges  and  does  not  afiect 
the  cord ;  then  pain  in  the  back  and  pain  referred  to  the  limbs  are 
prominent  symptoms,  and  rigidity  of  the  spine  develops,  giving  rise  to 
a  picture  similar  to  that  seen  in  cervical  or  lumbar  pachymeningitis 
(see  page  450).  Many  chronic  backaches  are  due  to  this  condition. 
When  the  exudation  is  about  the  cauda  equina  the  pains  are  felt  in  the 
legs  or  feet  and  there  may  be  inability  to  control  the  sphincters. 

A  special  type  of  spinal  syphilis  has  been  described  by  Erb.  This 
is  probably  due  to  an  endarteritis  of  those  spinal  vessels  which  enter 
the  cord  around  the  periphery,  and  as  these  vessels  are  more  numerous 
on  the  lateral  surfaces  the  eifect  of  the  obstruction  of  circulation  is  seen 
in  the  degeneration  of  the  fibres  and  the  development  of  a  secondary 
sclerosis  in  these  lateral  columns.  In  other  cases  it  is  a  chronic 
thickening  of  the  meninges  about  the  dorsal  region  which  leads  to 
pressure  on  the  cord  and  to  secondary  lateral  sclerosis.  In  still  other 
cases  Erb's  spastic  paraplegia  must  be  ascribed  to  a  primary  degenera- 
tion in  the  fibres  of  the  lateral  columns  often  combined  with  some 
sclerosis  in  the  posterior  columns.^  In  all  these  pathological  forms 
the  symptoms  are  the  same,  viz.,  a  slowly  advancing  spastic  paraplegia 
(see  page  839).  In  still  other  cases  the  exact  localization  of  the  spinal 
symptoms  makes  it  evident  that  a  single  gummy  tumor  is  compressing 
the  cord  (see  page  444).  There  is  nothing  particularly  characteristic 
of  gumma  as  distinct  from  other  tumors  of  the  brain  or  cord  ;  and  from 
a  study  of  the  general  and  local  symptoms  it  is  not  possible  to  determine 
that  the  tumor  is  specific.  But  in  any  case  of  tumor  it  is  wise  to  give 
the  patient  the  benefit  of  the  doubt,  and  if  under  antisyphilitic  treat- 
ment the  symptoms  sul^side  rapidly,  the  diagnosis  is  clear. 

'  For  the  pathological  bawiH  of  Erb's  cases  see  Nonne,  Deut,  Zeitsch.  f.  Nerven.,  Oct. 
1907.    .Also  Kenner,  Deut.  Zeitsch.  f.  Nerven.,  June,  1908. 


694 


SYPHILIS   OF   THE   NERVOUS   SYSTEM. 


III.  Syphilitic  Hyperplastic  Inflammation.  —  Syphilitic  hyperplastic 
inflammation  with  an  infiltration  of  the  membranes  and  of  the  brain  or 
cord  by  small  cells,  is  the  usual  accompaniment  of  exudative  processes, 
but  may  occur  without  the  production  of  gumma.  It  may  cause  chronic 
meningitis,  or  it  may  produce  chronic  indurative  or  sclerotic  patches 
through  the  nervous  tissues.     If  the  meninges  are  chiefly  involved  the 


Fig.  293. 


--^iS^ 


^i-^v.V. 


'':¥  '■ 


Area  of  encephalitis.  ^-1,  infiltrated  pia  mater.  5,  focus  of  granulation  tissue.  C,  zones  in  which 
glia  and  non-nervous  elements  are  proliferating.  D,  layer  of  epithelioid  cells  lying  along  the  edge  of 
(£)  white  substance.    Nissl  stain.    Magnification  15.     (Barrett.) 


symptoms  will  be  those  of  chronic  meningitis  (see  pages  457  and  726). 
If  the  brain  is  aifected  the  symptoms  will  be  very  similar  to  those  of 
general  paresis  (see  page  540),  for  localized  patches  of  encephalitis  with 
degeneration  of  the  neurons  may  occur  (Fig.  293).  I  have  seen  a 
number  of  cases  in  which  it  was  impossible  to  determine  whether  the 
patient  had  a  disseminated  syphilitic  induration  of  the  cortex  or 
paresis,  as  all  the  mental  and  physical  symptoms  of  paresis  were 
present,  but  in  which  antisyphilitic  treatment  was  followed  by  com- 
plete recovery,  now  permanent  for  fifteen  years.  This  fact  indicates 
the  propriety  of  trying  such  treatment  in  cases  supposedly  paretic. 

If  the  spots  of  induration  are  widely  scattered  through  the  brain, 
cerebral  axis  and  spinal  cord,  the  symptoms  will  be  identical  with  those 
of  multiple  sclerosis.  The  diagnosis  may  then  be  difficult,  as  Sachs ' 
and  Spiller  ^  have  indicated.     Ocular  palsies,  optic  atrophy,  difficulty  of 

1  Sachs.     Phil.  Med.  Jour.,  1898. 

2  Spiller.     Amer.  Jour.  Med.  Sci.,  June,  1907. 


DIAGNOSIS.  695 

speech,  meutal  changes,  attacks  of  an  apoplectic  nature  and  a  spastic  gait 
with  increased  reflexes  are  common  to  both  diseases.  The  development 
of  an  Argyll  Robertson  pupil,  of  rotary  nystagmus,  of  a  tremor  which 
is  distinctly  increased  by  voluntary  motions,  of  speech  which  is  typically 
scanning  rather  than  merely  hesitating,  the  lack  of  complete  remissions 
in  the  course  of  the  case,  its  steady  increase  of  symptoms  and  its  im- 
provement under  specific  treatment  are  all  in  favor  of  a  disseminated 
syphilitic  process  rather  than  of  multiple  sclerosis.  In  any  case  where 
the  diagnosis  is  impossible  a  test  of  the  spinal  fluid  or  of  the  blood 
should  be  made,  as  the  treatment  and  prognosis  are  very  different  in  the 
two  diseases. 

Occasionally  the  peripheral  nerves  are  involved,  causing  symptoms 
of  neuritis. 

IV.  Parasyphilitic  Diseases.  —  The  parasyphilitic  diseases  are  evi- 
dence of  degenerative  changes  in  the  neurons.  It  is  still  uncertain 
whether  they  are  produced  directly  by  the  toxic  action  of  the  syphilitic 
poison,  or  by  the  antitoxins  produced  in  the  blood  as  the  result  of  the 
syphilitic  infection,  or  in  some  other  unknown  way.  That  syphilis  is 
the  most  important  factor  in  the  production  of  tabes,  lateral  sclerosis 
and  paresis,  is  admitted  by  all,  while  some  go  so  far  as  to  say  that  it 
is  the  only  factor.  This  question  has  been  discussed  in  the  chapters 
devoted  to  these  three  diseases  and  requires  no  further  mention. 

V.  Hereditary  Syphilis.  —  Hereditary  syphilis  manifests  itself  in 
some  cases  by  the  production  of  nervous  diseases.  It  may  show  itself 
in  any  or  all  of  the  lesions  described  in  this  chapter,  the  infiltration  of 
the  tissues  and  meninges  being  most  common,  though  a  combination  of 
endarteritis  and  meningitis  is  sometimes  found.  ^  It  occasionally  causes 
cerebral  atrophy  in  infants  with  its  symptoms  (see  Chapter  XXYIII). 
Many  anomalies  of  development  of  the  brain  and  some  cases  of  hydro- 
cephalus are  traceable  to  hereditary  syphilis.  Some  cases  of  epilepsy 
are  due  to  it  and  in  every  such  case  developing  in  childhood  this  factor 
should  be  eliminated.  In  one  of  my  cases  of  epilepsy  in  which  all 
such  inheritance  was  denied,  the  appearance  of  Hutchinson  ian  teeth 
gave  a  clue  and  the  epilepsy  gradually  ceased  under  specific  treatment 
when  bromides  had  failed.  Cases  of  juvenile  paresis,  of  juvenile  tabes, 
and  of  juvenile  spastic  paraplegia  have  all  been  traced  to  inherited 
syphilis.  The  Wassermann  test  will  in  any  case  determine  the  exist- 
ence of  hereditary  syphilis. 

Diagnosis.  —  In  any  doubtful  case,  where  the  patient  denies  syphilis 
but  where  the  combination  of  symptoms  leads  to  the  suspicion  of  its 
existence,  it  is  the  duty  of  the  physician  to  have  the  Wassermann  test 
of  the  blood  made.  The  Wassermann  reaction,  briefly  outlined,  is  as 
follows:  Two  tubes  are  prepared  with  the  following  contents.  Tube  1. 
Extract  of  the  liver  and  spleen  of  a  syphilitic  fetus,  representing  the 
syphilitic  organism  (or  antigen),  plus  serum  to  be  tested.  Tube  2. 
Red  blood  cells  of  sheep  or  other  animal,  suspended  in  normal  saline 
solution,  plus  serum  of  a  rabbit  which  had  been  immunized  to  the  par- 
ticular red  blood  cells  used.      Complement  (a  sul)stance  necessary  to 

'  Rfinke.  Ubor  Uoliirnveranderungen  beiangeborenen  Syphilis.  Neurol.  Centralbl., 
Nov.,  1«J08.  - 


696  SYPHILIS   OF   THE   NERVOUS   SYSTEM. 

all  immune  reactions,  which  exists  in  tlie  fresh  serum  of  all  animals), 
in  the  shape  of  fresh  normal  guinea-pig  serum,  is  now  added  to  Tube 
1  and  allowed  to  stand.  At  the  end  of  a  half  hour  the  contents  of 
Tube  1  are  added  to  that  of  Tube  2.  If  the  serum  in  Tube  1  has  come 
from  a  syphilitic  individual,  its  relation  to  the  extract  of  antigen  is  a 
specific  one,  and  complement  will  be  absorbed  thereby  so  that,  when 
the  contents  of  Tubes  1  and  2  are  joined,  no  hemolysis  of  the  red  blood 
cells  in  Tube  2  will  take  place.  If  the  serum  is  not  from  a  syphilitic, 
complement  will  not  be  absorbed,  but  will  remain  active  and  cause 
hemolysis  in  Tube  2.  Hemolysis  is  observed  in  the  test  tube  as  a 
tingeing  of  the  whole  field  with  a  transparent  red  color.  The  red 
corpuscles  have  been  disintegrated  and  the  hemoglobin  liberated.  In 
the  absence  of  hemolysis  the  solution  of  red  cells  remains  turbulent 
and  opaque.  The  test,  of  course,  mvolves  numerous  controls  and  a 
careful  standardizing  of  the  hemolytic  serum  and  of  the  syphilitic 
extract. 

In  many  cases  of  neurasthenia  and  of  psychasthenia  with  marked 
fears  of  syphilis  it  is  wise  to  subject  the  patient  to  this  test :  if  his  fear 
is  confirmed  a  clue  to  treatment  is  obtained,  if  his  fear  has  no  basis  he 
will  be  helped  by  the  knowledge. 

A  reaction  of  the  spinal-cord  fluid  recently  discovered  by  Dr.  Noguchi 
at  the  Rockefeller  Institute  is  an  equally  positive  proof  of  syphilis. 
This  is  described  by  him  as  follows :  0. 1  c.c.  of  the  spinal  fluid  to  be 
tested  is  placed  in  a  test  tube  whose  diameter  is  1  cm.  or  less.  To  this 
is  added  0.5  c.c.  of  a  10  per  cent,  solution  of  butyric  acid.  The  tube 
is  now  heated  until  the  fluid  is  bubbling,  and  while  still  hot  0.1  c.c.  of 
a  normal  (4  per  cent.)  solution  of  sodium  hydrate  is  added.  In  nearly 
all  spinal  fluids  an  opalescence  or  cloud  occurs,  but  in  the  spinal  fluid 
of  a  syphilitic  person  the  cloud  soon  separates  into  a  definite  floccu- 
lence  which  is  characteristic.  The  flocculence  usually  appears  in  a  few 
moments  —  rarely  requiring  more  than  20  minutes.  The  reaction 
shown  by  Dr.  Noguchi  has  the  advantage  of  being  positive  in  a  quan- 
tity of  0.1.  c.c.  or  less,  and  of  being  a  qualitative  as  well  as  a  quanti- 
tative test.  What  the  flocculence  may  mean  is  not  known,  but  it 
may  consist  of  some  globulin  fraction  which  is  found  only  in  syph- 
ilitic or  metasyphilitic  disorders  of  the  nervous  system.  That  the  re- 
action is  not  entirely  quantitative  is  shown  by  the  fact  that  many 
fluids  from  non-paralytic  cases  give  much  richer  clouds  than  those  from 
paresis,  but  in  such  cases  the  clouds  do  not  flocculate. 

Nonne  has  elaborated  further  tests  for  the  cerebro-spinal  fluid.  The 
reader  is  referred  to  his  recent  work  on  syphilis  and  the  nervous  system 
for  their  descri])tion.^ 

Treatment.  —  The  treatment  of  syphilis  of  the  nervous  system  may 
be  by  the  use  of  salvarsan  or  by  the  use  of  mercury  and  the  iodides. 
In  all  cases  included  in  sections  I,  II,  and  III  salvarsan  should  be 
used.  In  cases  described  in  sections  IV  and  V  it  is  useless.  The 
effects  appear  to  be  better  when  the  remedy  is  injected  into  the  veins 
than  when  it  is   given  into  the  muscles.     At  least  three  injections 

*  M.  Nonne,  Syphilis  und  Nervensystem.     M.  Karger,  Berlin,  1911. 


"TREATMENT.  697 

should  be  made  at  intervals  of  two  weeks.     There  does  not  seem  to  be 
any  danger  of  optic  neuritis  such  as  was  at  first  feared/ 

For  the  details  of  the  use  of  salvarsan  the  reader  is  referred  to  recent 
literature.^  If  salvarsan  cannot  be  used  mercury  may  be  employed. 
Mercury  is  to  be  used  by  inunction,  one  drachm  of  the  ointment  being 
rubbed  into  the  skin  daily  until  a  slight  physiological  effect  is  pro- 
duced. It  is  well  to  mix  the  officinal  blue  ointment  with  an  equal 
part  of  simple  cerate  or  lanolin,  as  absorption  is  then  more  complete. 
The  inunction  may  be  made  upon  the  back  or  upon  various  parts  of 
the  body  and  extremities,  a  new  part  being  chosen  daily.  It  is  better 
absorbed  if  the  part  selected  be  thoroughly  washed  with  warm  water 
and  soap  and  then  with  alcohol  or  ether  before  the  application  ;  and 
after  the  application  —  which  should  be  thorough  and  therefore  should 
take  half  an  hour  —  a  tight  flannel  bandage  should  be  applied  over 
the  part  rubbed  ;  thus  what  is  left  on  the  surface  is  gradually  absorbed. 
It  aids  materially  in  the  effect  if  the  patient  takes  a  warm  bath  (98°  F.) 
daily  for  twenty  minutes.  The  production  of  salivation  is  to  be 
avoided.  If  inunctions  cannot  be  employed  the  mercury  may  be  given 
by  the  stomach,  the  protiodide,  in  dose  of  from  1  grain  to  ^  grain  three 
times  daily,  being  the  best  preparation.  The  amount  may  be  increased 
gradually  until  a  slight  diarrhoea  is  produced,  and  then  a  dose  a  little 
less  than  that  which  caused  the  diarrhoea  may  be  kept  up  for  some 
time.  I  prefer  the  French  pills  of  Garnier  and  Lamoureux,  as  of 
uniform  strength  and  obtainable  everywhere,  and  I  use  from  3  to  12 
of  these  pills  daily.  Corrosive  sublimate  may  be  employed  —  from 
-^Q  grain  to  gL  grain  three  times  daily  —  in  the  same  manner.  The 
advantage  of  the  method  by  inunction  is  that  it  does  not  affect  the 
digestion  unfavorably.  The  same  advantage  is  afforded  by  hypoder- 
mic injections  of  mercury  which  some  prefer.  Iodide  of  potassium  is 
to  be  given  in  connection  with  the  mercury  in  gradually  increasing 
doses,  from  15  to  100  grains  three  times  daily.  It  should  be  admin- 
istered, very  largely  diluted,  in  an  alkaline  water  after  meals,  or  else  in  a 
bitter  tincture  before  meals.  It  is  well  to  reduce  the  large  dose  of 
the  iodide  after  a  month,  and  then  to  continue  it  in  25-grain  doses 
three  times  daily  for  two  or  three  months  longer.  If  free  elimination 
of  iodide  is  secured  by  the  use  of  laxatives,  of  diuretics  (of  which  water 
is  best),  and  of  warm  baths  daily,  it  can  be  taken  for  a  long  period. 

The  use  of  salvarsan  and  of  mercury  in  the  parasyphilitic  diseases 
is  not  recommended.  They  have  no  effect  upon  the  course  of  tabes 
or  paresis.  In  tabes  JSTonne  and  Sachs  affirm  that  the  symptoms  are 
occasionally  relieved  temporarily  by  salvarsan  ;  but  not  to  any  degree 
greater  than  by  the  use  of  mercury.  I  have  not  seen  any  benefit 
resulting  from  its  use.  In  paresis  it  does  not  arrest  the  disease  and 
frequently  excites  attacks  of  violent  headache  and  mental  irritability. 
In  the  course  of  the  parasyphilitic  diseases  it  is  rare  to  observe  anv 
active  syphilitic  process  and  it  is  only  when  such  a  process  appears 
that  I  would  use  either  salvarsan  or  mercury. 

'See  Sachs,  Medical  Kecord,  Feb.  3,  1912. 

=>  Amer.  Jour.  Med.  Science,  1910,  p.  910  ;  1911,  p.  751. 


CHAPTER  XXXVIII. 

PELLAGRA. 

Pellagra  (pelle,  skin,  agra,  rough)  is  an  acute  disease  character- 
ized by  gastro-iutestinal,  skin,  and  nervous  symptoms,  progressive  in 
character,  subject  to  remissions,  but  of  a  chronic  nature. 

History. — The  disease  has  been  known  in  Italy  for  150  years  and 
has  been  steadily  increasing  there,  as  it  is  said  that  over  100,000  cases 
occur  annually.  It  has  been  recognized  in  the  United  States  since 
1863,  and  it  is  now  believed  to  have  been  the  cause  of  many  deaths  in 
the  prisons  and  camps  in  the  South  during  our  Civil  War.  Within 
the  past  two  years  it  has  been  increasing  rapidly  so  that  last  year  about 
10,000  cases  were  reported  in  various  parts  of  the  Southern  and 
Western  States. 

Etiology.  —  It  occurs  chiefly  in  the  spring  and  summer.  Sixty- 
five  per  cent,  of  the  cases  are  women.  While  it  may  occur  at  any  age, 
the  majority  of  cases  occur  between  the  ages  of  20  and  40.  An  alco- 
holic habit  appears  to  predispose  to  the  disease. 

It  has  been  generally  supposed  that  the  disease  occurs  in  consequence 
of  eating  corn  or  corn-meal,  called  polenta  in  Italy,  which  is  musty  or 
contains  some  mould  or  bacterium.  The  reason  for  this  belief  is  that 
the  disease  appears  only  where  corn  is  grown  and  used  as  a  chief  article 
of  food,  but  only  under  conditions  where  the  corn  is  not  free  from 
mould.  It  ceases  when  a  corn  diet  is  stopped.  It  does  not  prevail 
where  corn  is  not  used  as  a  food.  The  poison  producing  it  is  unknown 
though  several  forms  of  bacteria  and  mould  have  been  isolated  from 
spoiled  corn  and  certain  chemical  poisons  are  known  to  be  present  in 
spoiled  corn  which  have  been  supposed  to  have  been  a  cause.  One 
author,  however,  Sambon,  believes  it  to  be  due  to  the  sting  of  the  sand- 
fly. He  claims  that  it  is  more  common  in  moist  places  near  the  water 
and  among  field  laborers  who  are  exposed  to  the  sting  of  the  fly.  He 
believes  that  the  fly  conveys  a  micro-organism  to  man,  just  as  the  mos- 
quito conveys  the  malarial  organism  and  that  the  disease  is  due  to  this 
organism  in  the  blood.  After  a  careful  review  of  these  theories  Xiles ' 
inclines  to  the  first  hypothesis. 

Symptoms.  —  The  symptoms  of  the  disease  are  classified  under 
three  divisions,  gastric,  skin  and  nervous  symptoms.  In  the  majority 
of  instances  they  appear  in  the  order  named,  but  anyone  of  the  three 
may  usher  in  the  affection.     When  it  is  fully  developed  all  are  present. 

The  gastric  symptoms  consist  of  a  loss  of  appetite,  burning  pains  m 
the  stomach,  considerable  flatulence,  acid  eructations  and  occasional 
vomiting,  and  a  coated  tongue,  the  edges  of  which  are  smooth  and 

1  Pellagra,  G.  M.  Mies,  M.D.,  W.  B.  Saunders  and  Co.,  1912. 
698 


SYMPTOMS.  699 

occasionally,  sore.  The  mucous  membrane  of  the  mouth  is  red  and 
the  corners  of  the  mouth  become  ulcerated.  There  is  an  extra  flow  of 
saliva.  There  are  pains  in  the  abdomen,  considerable  gas  in  the  in- 
testines and  a  watery  diarrhoea,  attended  by  a  peculiar  characteristic 
odor,  and  by  difficulty  in  the  control  of  the  rectum. 

The  skin  symptoms  consist  of  an  erythema  with  considerable  swell- 
ing of  the  skin,  especially  upon  the  hands  and  feet  and  on  the  extensor 
surface  of  the  arms  and  legs  which  appears  symmetrically,  and  is 
sharply  marked  from  the  normal  skin  by  lines  of  division.  This 
eruption  as  it  goes  on  leads  to  a  thickening  and  rough  appearance  of 
the  skin  which  becomes  dark  and  even  black,  is  thrown  into  folds  and 
is  very  hard.  It  burns  but  it  does  not  itch.  It  extends  to  the  face 
and  to  the  trunk  in  patches.  When  this  eruption  fades,  the  skin 
desquamates,  occasionally  leaving  deep  scars,  occasionally  healing  till 
no  trace  remains  of  the  eruption. 

The  nervous  symptoms  are  first  burning  pains  in  the  extremities  with 
formication,  tremor  of  the  hands,  gradually  increasing  weakness  and 
difficulty  of  movement  and  contracture  in  the  muscles,  occasional  anaes- 
thesia in  the  affected  skin,  roughening  and  thickening  of  the  nails,  an 
increase  of  reflexes,  but  no  change  in  the  electrical  reaction  of  the 
muscles.  These  indicate  an  invol  vent  of  the  peripheral  nerves.  Symp- 
toms referable  to  the  central  nervous  system  are  also  present.  Headache, 
general  malaise,  sense  of  indefinite  distress  and  vertigo  are  frequent 
complaints.  Spasms  in  the  legs,  arms  and  back  occasionally  occur. 
Disturbance  of  speech  is  frequently  complained  of,  not  a  true  aphasia 
but  a  thickness  of  speech  and  a  weakness  of  the  voice.  Pupils  are 
usually  normal  and  react  to  light.  There  is  no  strabismus  or  nystag- 
mus. The  perception  through  the  senses  is  rather  dull  but  this  is  prob- 
ably owing  to  the  mental  condition  rather  than  to  any  defect  of  the 
senses.  Slight  ataxia  in  walking  and  swaying  with  the  eyes  closed  has 
been  occasionally  recorded. 

The  mental  symptoms  are  as  noticeable  as  the  nervous  symptoms. 
Depression  of  spirits,  irritability  of  temper  and  inability  to  fix  the  at- 
tention, unusual  antipathies,  occasional  morbid  fears  and  suicidal  im- 
pulses are  complained  of  in  the  majority  of  cases.  The  memory  is  im- 
paired and  the  power  of  attention  is  imperfect.  Delusions  and  halluci- 
nations occur  in  about  a  fifth  of  the  cases.  The  hallucinations  are  of 
sight  and  hearing.  The  delusions  as  a  rule  are  of  a  depressed  nature, 
of  danger  and  of  persecution.  The  prevailing  mental  state  is  one  of 
apprehension,  anxiety  and  depression.  There  is  an  undue  emotional 
irritability  and  the  patients  are  prone  to  outbursts  of  temper  and  to 
excessive  weeping.  The  facial  expression  is  one  of  melancholia,  but 
the  patients  do  not  reproach  themselves  as  a  true  melancholic  does,  and 
the  danger  of  suicide  is  much  less  than  in  melancholia.  Many  of  these 
patients  drift  into  the  lunatic  asylums  in  the  last  stage  of  the  disease 
and  their  cases  are  recognized  as  differing  from  those  of  the  typical 
insanities.' 

'  See  B.  E.  Tucker,  Amer.  Jour,  of  Med.  Sciences,  March  12,  1912. 


700  FtiLLAGUA. 

Course.  —  The  disease  begins  gradually  with  symptoms  chiefly  of 
malaise,  indefinite  pains  and  indigestion  which  form  a  prodromal  stage 
lasting  several  months.  This  is  usually  followed  by  the  stage  of 
erythema  when  the  eruption  appears  and  rapidly  develops,  and  in  this 
stage  the  gastro-intestinal  symptoms  become  more  acute.  This  stage 
may  last  for  several  weeks  or  months  and  is  followed  by  the  stage  of 
depression  with  the  development  of  the  nervous  and  mental  symptoms 
which  seem  to  be  the  culmination  of  the  poison.  A  fever  is  not  present. 
When  the  condition  is  fully  developed  it  may  remain  stationary  for 
several  months  and  then  under  appropriate  diet  and  treatment  begin 
to  subside.  Almost  all  the  symptoms  disappear  gradually  and  the 
patient  may  remain  well  for  some  time  but  a  recurrence  of  the  symptoms 
is  always  sure  to  occur,  usually  in  the  spring  or  summer  time.  After 
two  or  three  periods  of  remission  the  condition  becomes  chronic  and 
the  mental  change  permanent. 

Prognosis.  —  About  13  per  cent,  of  the  patients  die  in  the  first 
attack  of  the  disease,  about  10  per  cent,  recover  permanently  after  a 
light  attack.  The  remainder  go  on  to  a  series  of  attacks  with  remis- 
sions and  finally  become  chronic  invalids,  many  of  them  drifting  into 
insane  asylums. 

Treatment.  —  The  most  important  measures  to  combat  the  disease 
are  the  removal  of  the  patient  to  a  cold  climate  where  he  is  to  take  up 
his  permanent  residence ;  and  a  change  of  diet  in  such  a  way  as  to 
eliminate  all  preparations  of  corn.  To  increase  the  nutrition  in  every 
possible  way  by  carefully  regulated  diet  and  by  aids  to  digestion,  is 
part  of  the  duty  of  the  physician.  Arsenic  and  chloride  of  sodium  are 
to  be  used  freely  and  persistently.  Recently  some  favorable  effects 
have  been  noticed  from  the  use  of  atoxyl  given  hypodermically  every 
second  day  in  ^  grain  dose  gradually  increased  to  three  grains.  Uro- 
tropin  has  been  used  in  doses  of  ten  grains  four  times  a  day  with  some 
success.  The  irritation  of  the  stomach  and  intestines  can  be  mitigated 
by  large  doses  of  bismuth  and  oxalate  of  cerium.  The  burning  sensa- 
tions of  the  skin  can  be  benefited  by  powders  and  by  oxide  of  zinc 
ointment,  but  the  course  of  the  skin  disease  does  not  appear  to  be 
influenced  by  local  treatment.  The  nervous  symptoms  may  be  miti- 
gated by  the  use  of  bromides  and  coal  tar  products. 


CHAPTER  XXXIX. 


CAISSON  DISEASE. 

Etiology.  —  Persons  who  have  worked  in  caissons  or  in  deep  mines 
under  high  atmospheric  pressure,  or  under  the  sea  in  divers'  garments 
under  high  atmospheric  pressure,  are  occasionally  seized  with  paralysis 
on  coming  out  into  the  ordinary  air.  This  paralysis  has,  therefore, 
been  ascribed  to  the  sudden  change  of  atmospheric  pressure,  it  being 
supposed  that  the  system  accustoms  itself  to  a  very  much  increased 
pressure  —  say  of  four  to  six  atmospheres  —  without  difficulty,  but  that 
the  sudden  removal  of  this  is  the  cause  of  the  paralysis.  Hence,  at  the 
present  time,  where  individuals  have  to  work  subjected  to  such  pres- 

FiG.  294. 


Caisson  Disease.    Air  bubbles  in  the  veins  of  tlie  brain.     (Larkin.) 

sures  provision  is  usually  made  to  remove  this  pressure  very  slowly. 
Thus  modern  mines  and  caissons  have  several  intermediate  chambers 
in  which  the  workmen  are  advised  to  remain  for  one  hour  or  more, 
thus  accustoming  themselves  gradually  to  the  ordinary  atmosphere, 
and  if  this  precaution  is  observed  no  ill  results  follow. 

It  has  been  found  that  ordinary  healthy  men  of  good  habits  are  very 
much  less  liable  to  the  disease  than  men  who  indulge  in  alcohol  freely,  or 
who  have  some  form  of  heart  or  kidney  disease,  or  who  are  very  fleshy. 
Workmen  gradually  become  accustomed  to  endure  pressure,  and  hence 
there  is  a  greater  danger  for  new  hands  than  for  the  older  workmen, 

701 


702  CAISSON  DISEASE. 

Pathology.  —  A  remarkable  condition  of  air  embolism  in  the  veins 
and  capillaries  of  the  brain  and  spinal  cord  has  been  found  as  the 
primary  lesion  in  these  cases.  The  sudden  relief  from  the  extra- 
ordinary atmospheric  pressure  has  the  same  effect  on  the  blood  that  it 
has  in  champagne  when  uncorked ;  gas  is  evolved  in  the  fluid.  The 
gas  emboli  act  as  other  emboli  act  in  obstructing  the  circulation  and 
producing  secondary  infarctions  and  consequent  softening.  The  extent 
of  this  softening  may  be  large  or  small  depending  upon  the  size  of  the 

Fig.  295. 


Lesion  of  the  cord  in  caisson  disease.    A  large  cavity  is  seen  tilled  with  necrotic  debris  of  the 
cord  coagulated  by  the  preservative.     (Larkin. ) 

vessel  blocked  (Fig.  294).  In  the  majority  of  cases  the  emboli  are  of 
microscopic  size  and  located  in  the  capillaries,  hence  the  areas  of 
infarction  are  minute,  and  as  the  anastomosis  is  sufficient  to  reestab- 
lish the  circulation  and  nutrition  there  is  a  rapid  restoration  of  function 
and  a  progressive  recovery.  In  other  cases  where  the  infarctions  are 
large  and  where  softening  of  the  brain  or  cord  follow,  there  is  a  rap- 
idly fatal  termination  or  a  chronic  state  of  paralysis  remains.  The 
lesions  in  the  cord  resemble  those  already  described  in  the  section  upon 
myelomalacia  and  are  shown  in  Fig.  295.  There  are  hemorrhages  in 
the  cord  diffusely  scattered  and  surrounded  by  small  regions  of  acute 
myelitis.  There  is  an  infiltration  of  the  cord  by  small  cells  and  by 
lymphocytes,  and  the  development  of  fatty  degeneration  both  in  the 
cells  and  in  the  fibres  of  the  cord.  Vacuoles  form  on  the  cord.  Air 
emboli  have  been  found  on  the  meninges  of  the  cord  in  cases  which 
were  rapidly  fatal.  In  the  cases  which  survive  a  few  days  these  air 
emboli  are  not  found,  but  the  infarctions  and  the  degenerative  changes 
of  myelomalacia  are  everywhere  seen.  Occasionally  the  lesion  in  the 
cord  is  a  transverse  one  only  ;  usually  it  is  disseminated  widely. 

Symptoms.  —  The  symptoms  of  the  disease  develop  very  rapidly 
within  a  short  time  after  the  exit  of  the  individual  from  the  caisson 
into  the  ordinary  air.  These  symptoms  are  very  numerous  and  are 
mentioned  in  the  order  of  frequency  of  their  occurrence.  Pain  is  the 
chief  symptom  and  is  usually  felt  in  the  back  and  in  the  limbs,  so  that 


SYMPTOMS.  703 

the  patient  falls  to  the  ground  and  is  unable  to  move  freely  on  account 
of  the  discomfort.  This  pain  apj)ears  to  be  located  in  the  muscles, 
being  a  true  myalgia.  It  is  usually  general  but  occasionally  is  limited 
to  the  muscles  of  the  back,  sometimes  to  the  muscles  of  a  single  limb. 
It  is  attended  by  considerable  tenderness  of  the  muscles  to  pressure 
and  any  attempt  at  movement  increases  the  pain. 

Pain  in  the  joints  is  also  very  commonly  felt  and  may  give  rise  to 
considerable  disability.  This  is  next  in  frequency  to  pain  in  the 
muscles.  It  may  be  general,  all  the  joints  being  affected,  but  it  is 
usually  confined  to  the  knees,  the  hips,  the  shoulders,  and  the  elbows. 
It  is  not  attended  by  any  swelling  or  redness,  or  heat,  and  therefore 
the  disease  can  be  distinguished  from  ordinary  rheumatism. 

The  symptoms  next  in  frequency  to  these  pains  are  symptoms 
referable  to  the  ear.  These  are  severe  earache,  tinnitus  aurium,  deaf- 
ness, and  intense  vertigo,  together  with  the  sense  of  prostration  and 
vomiting  which  are  not  infrequent  accompaniments  of  disturbed  circu- 
lation in  the  ear.  Rupture  of  the  drum  has  been  frequently  observed 
and  hemorrhages  from  the  ear  are  not  uncommon.  The  hearing  may 
be  temporarily  impaired,  but  usually  in  the  course  of  a  few  days  these 
symptoms  subside  and  the  patient  recovers  his  hearing  completely. 

Another  set  of  symptoms  are  those  referable  to  the  nervous  system. 
They  are  both  cerebral  and  spinal.  The  cerebral  symptoms  consist  of 
headache,  prostration,  feelings  of  faintness,  nausea,  vomiting  and  ver- 
tigo, double  vision,  sometimes  sudden  blindness  or  deafness,  and  diffi- 
culties of  speech  and  of  breathing.  Many  cases  have  been  observed  in 
which  a  monoplegia  or  hemiplegia  has  ensued,  and  many  patients  have 
suffered  from  attacks  of  aphasia,  either  motor  or  sensory.  Sometimes 
the  general  symptoms  accompanying  these  cerebral  symptoms  are  such 
as  to  indicate  a  great  increase  of  intracranial  pressure,  the  pulse  tension 
rising  to  200  or  over,  the  pulse  being  slow,  respirations  sterterous  or 
Cheyne-Stokes  in  character.  Several  cases  that  have  presented  such 
symptoms  have  been  operated  upon,  a  decompression  operation  for  the 
relief  of  intracranial  pressure  having  been  done,  and  in  these  cases  the 
dura  has  bulged  and  on  opening  the  dura  it  has  been  evident  that  an 
effusion  of  fluid  into  the  ventricles  has  produced  this  great  pressure. 
In  the  majority  of  instances  such  decompressive  operations  have  not 
been  successful  •  in  saving  the  life  of  the  patient.  Yet  when  such 
symptoms  occur  it  seems  necessary  to  attempt  such  relief,  and  tapping 
the  ventricles  is  to  be  attempted  if  lumbar  puncture  has  failed  to  relieve 
the  symptoms.  Many  patients  pass  into  a  state  of  coma  after  these 
cerebral  symptoms  and  die,  but  in  some  cases  a  gradual  recovery 
ensues  and  the  patients  may  recover  entirely  or  may  be  left  with  only 
a  slight  hemiplegia  or  monoplegia,  or  a  mild  aphasia. 

The  spinal  symptoms  are  as  common  as  the  cerebral  symptoms. 
Soon  after  exit  from  the  caisson  the  patient  complains  of  severe  pains, 
wliich  are  usually  felt  in  tlie  legs  and  in  the  trunk,  occasionally  in  the 
arms  and  ba(;k,  and  th(!se  pains  persist  for  many  days  through  the  ill- 
ness. The  pains  resemble  in  tlieir  intensity  those  of  tabes.  They  are 
attended  by  numbness  and  tingling  or  sensations  of  cold  or  heat.    Soon 


704  CAISSON   DISEASE. 

after  the  onset  of  pain  a  feeling  of  weakness  develops  in  the  legs,  which 
goes  on  rapidly  to  a  condition  of  paralysis,  both  legs  being  totally  par- 
alyzed, with  loss  of  control  of  the  sphincters.  Anaesthesia  accompanies 
the  paralysis  and  extends  a  variable  height  upon  the  trunk.  In  the 
majority  of  instances  the  paralysis  does  not  extend  to  the  arms,  but 
occasionally  these  also  are  affected  and  then  the  patient  is  totally  par- 
alyzed and  ansesthetic  even  up  to  the  neck.  In  one  case  observed  by 
me  all  the  muscles  below  the  deltoid  were  completely  paralyzed  for 
four  days,  but  the  ansesthesia  did  not  extend  above  the  eighth  rib.  In 
this  case  a  gradual  recovery  ensued  and  after  two  months  the  man  had 
entirely  recovered.  Variations  in  degree  in  all  these  symptoms  have 
been  observed.  The  majority  of  the  patients  develop  the  most  com- 
plete paralysis  within  a  few  hours.  In  some  cases  the  symptoms  sub- 
side as  rapidly  as  they  have  developed,  and  within  a  week  the  patient 
is  up  and  about.  In  other  cases,  however,  many  spinal  symptoms 
remain  and  after  a  month  some  of  these  patients  present  all  the  symp- 
toms of  tabes.  In  other  cases  the  symptoms  are  those  of  lateral  scler- 
osis only,  and  in  still  other  cases  the  symptoms  are  those  of  a  general- 
ized myelitis.  In  fatal  cases  the  lesions  of  all  these  three  spinal  dis- 
eases have  been  found  at  the  autopsy.  The  prognosis,  however,  in  the 
cases  of  spinal-cord  affection  developing  after  caisson  disease  is  dis- 
tinctly better  than  when  such  symptoms  develop  under  other  circum- 
stances, and  if  the  patients  are  promptly  treated  many  recover  who, 
under  other  circumstances,  would  have  gone  on  to  death  or  to  a  state 
of  chronic  invalidism.^ 

The  causes  of  death,  as  in  acute  myelitis,  are  usually  cystitis,  bed- 
sores, or  pneumonia. 

Diagnosis.  —  The  diagnosis  of  the  disease  rests  upon  ascertaining 
whether  the  patient  afflicted  has  been  subjected  to  high  atmospheric 
pressure  and  then  has  come  back  into  the  air  very  suddenly. 

Treatment.  —  A  knowledge  of  the  cause  of  this  affection  should 
lead  employers  to  warn  their  workmen  against  coming  immediately 
from  a  high  to  a  low  atmospheric  pressure  and  should  make  it  incum- 
bent in  all  works  to  have  a  graduated  series  of  chambers  through  which 
the  laborers  should  be  made  to  pass  from  a  higher  to  a  lower  pressure. 
When  the  symptoms  have  developed  it  has  been  recommended  to  imme- 
diately return  the  patient  to  the  caisson  or  to  place  him  in  a  pneumatic 
cabinet  where  a  high  atmospheric  pressure  can  be  immediately  pro- 
duced, and  then,  by  a  gradual  reduction  of  the  pressure,  to  accustom 
him  to  the  ordinary  atmosphere.  Such  a  pneumatic  cabinet  should 
always  be  accessible  in  works  or  mines  where  laborers  are  subject  to 
this  disease.  When  this  is  not  feasible  it  has  been  recommended  by 
those  who  have  experience  in  this  affection  that  large  doses  of  ergot, 
one  drachm  every  hour  or  every  two  hours,  should  be  given.  Bandag- 
ing the  limbs  tightly  with  an  Esmarch  bandage,  and  thus  confining  the 
blood  to  the  larger  cavities,  has  been  employed  with  good  effect.  Later 
on  the  treatment  should  be  that  for  acute  myelitis. 

'See  "  Luftdruck  Erkrankungen,"  by  E.  Heller,  W.  Mager,  and  H.  von  Schrotten. 
—  A.  Holder,  Vienna,  1900, 


.       CHAPTER    XL. 

PACHYMENINGITIS   AND   MENINGEAL   HEMORRHAGE. 

Pachymeningitis  or  inflammation  of  the  dura  mater,  may  be  lim- 
ited to  the  external  or  to  the  internal  surface  of  the  dura,  or  may  in- 
volve both  surfaces  at  once. 

PACHYMENINGITIS  EXTERNA. 

Pachymeningitis  externa  occurs  as  a  result  of  fractures  of  the  skull, 
or  follows  any  disease  of  the  bones  of  the  cranium,  such  as  caries, 
especially  after  ear  disease,  syphilitic  exostoses,  tumors  invading  the 
bones,  and  erysipelas  of  the  face. 

In  septic  cases  it  may  lead  to  the  formation  of  an  abscess  between 
the  dura  and  the  bone.  The  only  symptoms  produced  are  those  of  the 
original  causative  disease,  and  local  pain,  which  may  be  increased  by 
pressure  and  by  percussion.  In  these  cases  trephining  will  lead  to  the 
evacuation  of  the  pus. 

It  may  take  the  form  of  a  connective-tissue  proliferation,  causing  a 
thickening  which  is  permanent. 

In  a  few  cases  a  firm  adhesion  between  the  dura  and  the  cranial 
bones,  with  the  production  of  bony  deposits  and  a  solidification  of  the 
diploe,  has  been  found.  This  may  occur  in  early  life  and  lead  to  the 
development  of  chronic  headache  with  many  neurasthenic  and  hys- 
terical symptoms  which  resist  all  forms  of  treatment. 

PACHYMENINGITIS  INTERNA. 

Etiology.  —  This  disease  may  result  from  the  same  causes  as  pachy- 
meningitis externa,  especially  from  trauma,  or  may  be  a  sequel  of  it. 
It  is  never  possible  in  life  to  distinguish  sharply  between  the  two  con- 
ditions. It  may  be  a  sequel  of  sunstroke.  It  has  been  found  in  many 
cases  of  chronic  insanity,  especially  in  paretic  and  senile  dementia.  It 
has  been  ascribed  to  chronic  alcoholism.  It  has  been  found  in  cases 
of  purpura  and  in  persons  dying  of  various  infectious  and  wasting  dis- 
eases ;  a  causal  connection,  however,  has  not  been  established.  It  may 
be  due  to  syphilis. 

Pathology.  —  The  result  of  an  inflammation  of  the  inner  layer  of 
the  dura  is  the  production  of  a  thin  vascular  membrane  covering  it 
like  a  veil.  The  vessels  in  this  are  extremely  delicate  and  rupture 
easily.  As  a  result  a  hsematoma  forms,  flat  and  thin,  but  of  sufficient 
size  to  compress  the  brain  beneath  and  to  cause  symptoms.  Some 
pathologists  believe  that  in  cases  of  hsematoma  the  hemorrhage  from 
45  705 


706      PACHYMENINGITIS  AND  PACHYMENINGEAL  HEMORRHAGE. 

congested  vessels  occurs  first,  then  the  clot  becomes  organized  and 
adherent  to  the  dura,  and  then  new  vessels  form  in  it.  Bevan  Lewis 
believes  this  to  be  the  invariable  case  in  the  chronic  insane.  The  new 
membrane  may  be  limited  in  extent  or  quite  large.  It  may  lie  on  any 
part  of  the  dura.  If  the  condition  goes  on  for  some  time  several  layers  of 
new  tissue  may  form  until  the  dura  is  several  times  its  ordinary  thickness. 
In  such  a  thickened  series  of  layers  flat  hsematomata  are  often  found. 
The  age  of  the  various  layers  can  be  determined  by  their  degree  of 
hardness  and  vascularity.  In  the  older,  deeper  layers  there  may  be 
thin  deposits  of  bone.  In  the  superficial  layers  there  is  occasionally 
an  adhesion  to  the  pia.  This  is  the  condition  found  in  chronic  alco- 
holism and  in  terminal  dementia. 

Symptoms.  —  The  disease  may  be  present  for  years  as  a  chronic 
condition,  causing  dull  headaches  only  or  slight  difficulty  in  thinking, 
with  impaired  memory,  and  may  not  be  diagnosticated.  It  is  by  the 
occurrence  of  the  hemorrhages  which  produce  symptoms  of  a  temporary 
apoplexy  that  the  suspicion  may  be  awakened  of  a  pachymeningitis 
hemorrhagica.  Thus  an  alcoholic  subject  who  had  had  a  sunstroke  and 
had  at  various  times  suffered  from  blows  on  the  head  complained  for 
some  weeks  of  severe  headaches,  and  suddenly  had  an  attack  of  left 
hemiplegia  attended  by  vomiting  and  a  short  general  convulsion.  In 
the  course  of  two  weeks  all  signs  of  the  hemiplegia  subsided  and  it  was 
evident  that  no  destruction  of  brain  tissue  had  occurred.  His  head- 
aches continued  and  four  months  after  the  first  attack  a  second  with 
right  hemiplegia  and  aphasia  occurred  which  proved  fatal.  The  au- 
topsy showed  an  extensive  pachymeningitis  interna  with  a  large  recent 
hsematoma  on  the  left  side  and  evidences  of  an  old  one  on  the  right 
side.  In  any  case  where  apoplectic  symptoms  occur  and  subside 
rapidly  the  possibility  of  hsematoma  must  be  considered.  During  the 
attack  and  immediately  after  it  the  diagnosis  of  apoplexy  is  the  only 
one  possible.  The  pupil  is  dilated  on  the  side  of  lesion,  the  head 
and  eyes  may  turn  toward  the  lesion,  and  a  rigid  state  of  the  limbs  may 
exist  for  some  hours  after  the  attack. 

The  course  of  a  case  in  pachymeningitis  is  sometimes  very  slow. 
The  patient  may  suffer  for  several  years  from  occasional  attacks  of 
severe  headache  lasting  several  days  and  then  gradually  subsiding,  but 
leaving  him  with  disagreeable  cerebral  sensations  and  a  sense  of  inability 
to  do  his  work.  He  may  be  aware  of  difficulty  in  fixing  his  attention 
upon  business  or  matters  of  importance,  and  may  suffer  from  difficulty 
in  recollecting  recent  events.  He  may  manifest  all  the  symptoms  of 
cerebral  neurasthenia  with  occasional  attacks  of  vertigo  and  prostra- 
tion. As  time  goes  on  he  may  have  sudden  attacks  of  unconsciousness, 
with  or  without  twitching  of  a  general  character  of  the  limbs,  or  with 
a  true  Jacksonian  epileptic  attack,  and  after  such  an  attack  he  may  be 
much  prostrated  for  ten  days  or  two  weeks,  being  confused  mentally, 
or  almost  stuperous,  or  being  very  irritable  and  restless.  Then  all  these 
symptoms  may  subside  and  he  may  return  to  a  state  of  apparent  health 
and  even  be  able  to  go  back  to  business.     But  even  when  apparently 


PACHYMENINGITIS  INTERNA.  707 

well  he  will  be  known  by  those  who  are  most  intimately  associated 
with  him  to  show  some  changes  in  disposition  and  character  which  are 
unnatural.  After  a  period  varying  from  six  months  to  a  year  the 
headaches  may  reappear,  convulsions  may  be  repeated,  and  inability 
to  think  and  remember  may  again  become  noticeable.  Attacks  of 
hemiplegia,  or  of  hemianopsia,  or  of  aphasia  may  follow,  and  the 
patient  may  again  be  reduced  to  a  state  of  invalidism.  It  is  seldom 
that  two  remissions  occur,  but  they  have  been  observed.  Finally,  all 
the  symptoms  become  intensified,  the  headache  becomes  continuous, 
the  mental  dulness  becomes  more  marked,  and  the  patient  is  obliged 
to  go  to  bed,  where  he  lies  in  a  state  of  semi-stupor  for  weeks  or  even 
months.  In  this  condition  he  can  be  aroused,  will  answer  simple 
questions,  but  shows  no  interest  in  his  surroundings  or  in  his  family, 
and  is  indifferent  to  his  own  condition.  He  will  take  food  when  it  is 
offered,  but  seems  to  live  in  a  dazed  condition,  and  is  very  likely  to 
neglect  ordinary  cleanliness.  He  sleeps  heavily  in  the  daytime  and  is 
wakeful  at  night,  and  very  often  has  a  muttering  delirium  during  the 
night ;  at  times  starting  up  in  a  state  of  alarm ;  at  times  crying  out  as  if 
in  pain,  and  at  times  being  restless  and  desirous  of  getting  up  and  going 
out.  A  general  condition  of  motor  weakness  gradually  develops,  even 
when  the  patient  does  not  show  the  traces  of  apoplectic  attacks.  His 
movements  become  uncertain  and  ataxic,  with  considerable  tremor  of 
the  extremities  and  even  of  the  entire  body.  In  getting  up  out  of 
bed  he  will  do  so  in  a  feeble  manner,  with  much  trembling  and  shaking 
of  the  trunk,  and  it  seems  difficult  for  him  to  retain  his  balance  or  to 
stand  alone,  oscillating  movements  of  the  body  and  of  the  head  being 
constant.  When  he  is  up  he  seems  unable  to  decide  what  to  do, 
whether  to  remain  standing  or  to  sit  down,  and  acts  in  a  dazed  manner. 
There  is  rarely  any  loss  of  sensibility  to  touch,  temperature,  oi*  pain, 
but  sometimes  the  patient  appears  to  be  deaf,  and  in  some  cases  the 
eyesight  is  affected. 

The  patient  may  remain  in  this  condition  for  several  weeks,  grad- 
ually losing  strength  and  weight,  and  finally  dying  of  some  compli- 
cation, such  as  obstinate  constipation,  cystitis,  or  bed-sores.  A  pro- 
gressive emaciation  and  a  condition  of  extreme  anaemia  or  of  nephritis 
are  not  uncommonly  observed  in  the  course  of  the  disease. 

Optic  neuritis  has  been  occasionally  found.  In  these  cases  brain 
tumor  may  be  suspected  and  cannot  be  excluded. 

Treatment.  —  It  is  so  seldom  that  one  can  make  a  diagnosis  of 
pachymeningitis  interna  hemorrhagica  that  it  is  almost  futile  to  speak 
of  treatment.  Ice  to  the  head,  or,  better,  applications  of  the  actual 
cautery,  may  relieve  the  headache.  Purgatives  of  a  drastic  kind  may 
also  be  used.  When  an  attack  of  an  apoplectic  nature  occurs  it  is  to 
be  treated  as  a  cerebral  hemorrhage.  It  is  rarely  possible  to  operate 
for  the  removal  of  such  a  hemorrhage  at  the  time  of  its  occurrence. 
There  may  be  indications  which  justify  an  operation  later,  as  in  the 
following  case  : 

A  child,  aged  nine  years,  had  a  severe  fall  on  the  head,  that  was  fol- 


708     PACHYMENINGITIS  AND  PAGHYMENINGEAL  HEMORRHAGE. 

lowed  by  coma  lasting  several  hours,  after  which  a  left  hemiplegia  was 
discovered.  This  subsided  entirely  in  the  course  of  two  weeks,  but 
he  continued  to  have  severe  headaches,  and  after  two  months  attacks  of 
Jacksonian  epilepsy  on  the  left  side,  beginning  in  the  hand,  com- 
menced. These  kept  up  with  increasing  frequency  for  six  months,  not 
being  controlled  by  bromides.  He  was  then  operated  upon  by  Mc- 
Cosh,  a  large  bony  flap  being  made  and  free  access  to  the  brain 
being  had.  The  inner  surface  of  the  dura  over  the  motor  area  of  the 
brain  was  covered  by  a  tliin,  velvet-like  layer  of  tissue  which  bled 
freely  on  being  touched.  This  was  adherent  to  the  pia  over  the  mid- 
dle third  of  the  posterior  central  convolution,  but  was  easily  stripped 
off.  It  was  removed  by  a  curette,  the  hemorrhage,  which  was  trifling, 
being  controlled  by  pressure,  and  the  dura  was  united  and  the  wound 
closed.  The  child  recovered  from  the  operation  and  had  had  no  return 
of  the  Jacksonion  fits  at  the  end  of  two  years. 

The  operation  was  undertaken  on  the  belief  that  a  cortical  traumatic 
hemorrhage  had  left  a  cyst  which  was  causing  the  epilepsy  and  which 
might  be  removed.  The  discovery  of  the  condition  of  pachymenin- 
gitis interna  hemorrhagica  was  a  surprise.  The  ease  with  which  the 
hsematoma  was  stripped  off  led  to  the  idea  that  it  was  merely  the 
remains  of  a  flat  clot,  but  microscopic  examination  showed  it  to  be  an 
organized  membrane  with  capillaries  identical  with  that  found  in 
pachymeningitis  interna. 

MENINGEAL  HEMORRHAGE. 

Hemorrhage  from  the  middle  meningeal  artery  occurs  either  with  or 
without  fracture  of  the  skull  as  the  result  of  trauma.  In  Fig.  296, 
the  sites  of  the  most  common  varieties  of  meningeal  hemorrhage  are 
shown.  These  hemorrhages  are  between  the  bone  and  the  dura.  The 
symptoms  of  such  hemorrhage  are  general  and  local.  After  a  blow  or 
fall  on  the  head,  with  or  without  apparent  fracture,  for  fracture  of  the 
internal  table  may  not  be  evident,  the  patient  becomes  comatose  and  is 
found  to  be  paralyzed  on  one  side,  with  the  pupil  on  the  side  of  the 
injury  very  widely  dilated  and  not  responsive  to  light.  The  coma  may 
not  be  deep,  the  patient  being  rather  in  a  deep  stupor,  and  this  may 
continue  with  varying  degree  until  the  pressure  is  relieved  by  trephin- 
ing. Or  consciousness  may  be  regained  slowly,  and  then  either  (1) 
aphasia,  or  (2)  hemiplegia,  or  both  together,  or  (3)  hemiansesthesia,  or 
(4)  hemianopsia  are  discovered  according  to  the  position  of  the  hemor- 
rhage which  causes  a  diffuse  compression  of  one  hemisphere.  Such 
cases  should  be  trephined  as  soon  as  the  diagnosis  is  made  and  the  torn 
vessel  tied  and  the  clot  removed. 

Hemorrhage  inside  of  the  dura,  either  from  its  vessels  or  from  sonie 
vessel  in  the  pia  mater  is  also  caused  by  trauma  with  or  without  frac- 
ture of  the  skull.  Hemorrhages  which  occur  in  infants  during  labor, 
from  delayed  or  instrumental  delivery,  are  usually  inside  the  dura  and 
result  in  a  large  clot  spread  out  on  the  surface  of  the  brain.     In  some 


MENINGEAL  HEMORJRHAOE. 


709 


(iases  the  vessel  torn  is  a  large  one  and  the  clot  is  rapidly  formed,  pro- 
duces greftt  pressure,  and  death  follows. 

In  other  cases  the  vessel  is  a  small  one  and  the  clot  extends  slowly ; 
the  symptoms  then  gradually  increase  during  several  hours  or  even 
days  until  the  bleeding  ceases,  and  then  subside  more  slowly  still  and 
only  in  part.  In  infants  the  existence  of  such  a  pressure  by  a  large 
clot  may  prevent  the  process  of  development  of  the  brain  and  lead 


Fig.  296, 


The  meningeal  artery  and  its  relation  to  the  fissures  of  Rolando  and  Sylvius.  I.,  area  of  a  hemor- 
rhage from  the  main  artery,  causing  hemiplegia  and  aphasia ;  II.,  area  of  a  hemorrhage  from  the 
anterior  branch,  causing  mental  dulness  and  aphasia ;  III.,  area  of  a  hemorrhage  from  the  posterior 
branches,  causing  sensory  aphasia  and  hemianopsia. 

to  atrophy  and  sclerosis.  (See  Chapter  XXVIII.)  The  general  symp- 
toms of  pressure  are  diminishing  consciousness  to  the  degree  of  stupor, 
or  even  coma,  headache,  a  slow  pulse,  vomiting,  and  possibly  difficulty 
of  respiration,  and  a  rise  of  temperature.  The  local  symptoms  are 
those  of  compression  of  some  part  of  the  hemisphere.  This  is  usually 
on  the  side  of  the  injury,  but  occasionally  on  the  opposite  side.  A 
dilated  pupil  on  the  side  of  the  lesion,  deviation  of  head  and  eyes  to 
that  side,  and  aphasia,  symptoms  of  paralysis,  or  loss  of  sensation  or 
of  sight  on  the  opposite  side,  are  the  local  symptoms,  and  their  value 
is  to  be  estimated  as  in  cases  of  cerebral  hemorrhage.  When  these 
symptoms  persist  for  more  than  two  weeks  there  is  evidence  of  con- 
tinued pressure,  and  it  is  not  likely  tliat  a  clot  large  enough  to  cause 
them  will  be  absorbed,  hence  it  must  be  removed.     Neisser  has  made 


710    PACHYMENINGITIS  AND  PACHYMENINGEAL  HEMORRHAGE. 

the  diagnosis  in  many  cases  by  puncture  of  the  skull  and  aspiration  — 
the  technique  of  which  is  more  fully  described  on  page  599.  In  one 
case  he  removed  180  c.c.  of  blood  from  the  posterior  fossa  and  saved 
the  patient.  In  many  cases  this  is  merely  a  diagnostic  procedure  and 
is  to  be  followed  by  trephining  for  the  removal  of  the  clot.  The  treat- 
ment, therefore,  should  be  by  trephining.  The  following  case  is  a  good 
illustration  of  this  condition  : 

A  physician,  aged  forty  years,  was  thrown  from  his  carriage  on 
i^ugust  17,  1889.  He  was  slightly  stunned  by  the  fall,  but  had  no 
wound,  and  was  able  to  help  his  wife,  who  appeared  to  be  injured,  to 
reach  home.  For  several  hours  he  seemed  to  be  suffering  merely  from 
bruises,  and  was  able  during  the  afternoon  to  converse  with  a  patient 
and  to  administer  a  hypodermic  injection.  In  the  course  of  the  even- 
ing, however,  he  became  delirious,  then  stupid,  and  for  the  following 
three  days  he  lay  in  a  semi-comatose  condition.  On  the  morning  after 
the  injury  he  was  found  to  be  completely  hemiplegic  on  the  right  side, 
and  aphasic.  When,  after  a  week,  his  consciousness  had  fully  returned, 
it  appeared  that  the  aphasia  was  purely  motor,  as  he  could  understand 
what  was  said  to  him,  and  could  read.  The  hemiplegia  was  attended 
by  a  partial  anaesthesia,  the  paralyzed  limbs  felt  numb,  were  less  sen- 
sitive to  touch  and  to  pain,  but  acutely  sensitive  to  cold  and  to  heat. 
In  this  condition  he  remained  until  December,  when  he  was  brought 
to  New  York  and  admitted  to  Roosevelt  Hospital. 

On  examination,  the  patient,  a  large  man,  was  unable  to  say  any- 
thing, even  "  yes  "  or  "  no,"  the  vowel  sounds  being  the  only  sounds 
produced  by  effort.  He  evidently  understood  questions,  and  attempted 
to  reply  by  gestures  and  by  trying  to  write  with  his  left  hand.  It  was 
noticed,  however,  that  he  was  mentally  dull,  so  that  the  result  of  tests 
of  muscular  sense  were  not  satisfactory,  though  he  could  be  tested  for 
tactile  and  pain  senses,  which  were  found  to  be  impaired  on  the  para- 
lyzed side.  He  was  emotionally  unstable,  laughing  too  readily,  and  at 
times  appearing  to  be  much  depressed.  Ophthalmoscopic  appearances 
were  normal.  Sight  and  hearing  were  normal.  The  right  hemiplegia 
was  partial.  He  could  turn  his  eyes  in  all  directions,  but  could  not 
turn  his  head  to  the  right.  His  left  pupil  was  one-third  larger  than 
the  right  pupil,  but  both  reacted  normally.  His  face  was  slightly  flat 
but  not  paralyzed,  and  his  tongue  protruded  straight.  His  arm  was 
almost  totally  paralyzed,  the  only  motion  possible  being  a  slight  abduc- 
tion at  the  shoulder.  ,  His  leg  could  be  moved  a  little  at  the  hip  and 
knee,  and  when  held  up  by  two  persons  he  could  drag  the  leg  forward 
a  little,  but  could  not  stand  alone.  The  hand  was  flexed  and  pronated, 
the  leg  was  extended ;  both  were  very  rigid,  and  all  the  deep  reflexes 
were  greatly  exaggerated,  so  that  wrist  and  finger  clonus,  as  well  as 
patella  and  ankle  clonus,  were  easily  produced.  He  controlled  his 
sphincters  perfectly.     There  were  no  scars  upon  the  head. 

It  seemed  probable  that,  as  a  result  of  the  fall,  there  had  been  a 
rupture  of  a  small  vessel  from  which  very  slow  hemorrhage  had  taken 
place ;  the  vessel  was  thought  to  be  a  vein  rather  than  an  artery, 


MENINGEAL  HEMORRHAGE. 


711 


because  of  the  very  slow  development  of  the  symptoms.  The  situa- 
tion of  the 'clot  was  thought  to  be  upon  the  surface  and  not  within  the 
left  hemisphere,  and  it  was  located  upon  the  posterior  part  of  the  third 
frontal  convolution  and  over  the  anterior  central  convolution  in  its 
middle  third,  as  shown  in  the  diagram.  A  flat  clot  in  such  a  situation 
might  fail  to  compress  the  face  area  and  could  produce  an  absolutely 
total  motor  aphasia,  while  a  subcortical  or  capsular  clot  could  hardly 
produce  total  permanent  motor  aphasia  and  paralysis  of  the  arm  with- 
out producing  paralysis  of  the  face  and  tongue.  On  the  strength  of 
this  diagnosis  it  was  thought  best  to  operate. 


Meningeal  hemorrhage.    The  situation  of  the  clots  causing  aphasia  and  right  hemiplegia. 
Removal  resulted  in  recovery.     (Starr  and  McBurney.) 

On  December  13,  1889,  McBurney  trephined  the  skull.  The  tre- 
phine was  applied  at  a  point  one  and  seven-eighths  inches  behind,  and 
seven-eighths  of  an  inch  above  the  external  angular  process  of  the 
frontal  bone,  and  the  opening  was  then  enlarged  by  the  rongeur 
forceps  upward  and  backward,  the  dura  being  laid  bare  over  an  oval 
area  three  by  two  inches.  The  dura  did  not  pulsate.  On  opening  the 
dura  the  pia  was  found  to  be  very  oedematous  and  discolored,  and  the 
surface  of  the  brain  was  separated  from  the  dura  by  a  space  half  an 
inch  in  depth,  and  did  not  pulsate.  The  clot  was  seen  lying  beneath 
the  pia  upon  the  posterior  part  of  the  third  frontal  convolution  and 
extending  over  the  anterior  central  convolution  in  a  thin  layer  (marked 
in  lines  on  the  diagram)  into  the  fissure  of  Rolando,  which  was  filled 
with  a  larger  clot  lying  in  the  situation  shown  in  the  diagram  and 
extending  downward  so  as  to  fill  up  and  distend  greatly  the  cul-de-sac 
at  the  lower  end  of  the  fissure.  The  clot  had  not  covered  the  lower 
third  of  the  anterior  central  convolution  and  had  not  reached  the 
upper  quarter  of  the  fissure  of  Rolando.  The  brain,  at  a  distance  of 
an  inch  about  it,  appeared  to  be  healthy  and  pulsated,  but  the  parts  of 


712    PACHYMENINGITIS  AND  PACHYMENINGEAL  HEMORRHAGE. 

the  cortex  on  which  the  clot  lay  were  pulseless  and  stained  a  yellowish- 
red.  After  the  pia  had  been  incised  the  clot  was  removed  little  by 
little  by  fine  sponges,  at  least  a  drachm  of  partly  organized  clot  being 
taken  out  of  the  fissure  of  Rolando.  The  retraction  of  the  brain 
from  the  skull  was  even  more  evident  when  the  operation  was  com- 
plete. The  operation  was  done  under  strict  aseptic  precautions,  and 
was  not  followed  by  any  rise  of  temperature.  The  wound  was  dressed 
in  the  open  method,  being  packed  with  gauze  and  drainage  tubes  being 
inserted.  Ou  renewing  the  dressings  three  days  subsequently  to  the 
operation  it  was  found  that  the  entire  surface  of  the  brain  was  pulsat- 
ing normally  and  that  the  brain  surface  presented  a  normal  color  and 
appearance.  After  a  week  the  drainage  tubes  were  removed,  and 
after  three  weeks  the  wound  had  entirely  healed,  the  level  of  the  scalp 
at  the  bottom  of  the  cavity  being  an  inch  below  the  normal  level. 

On  the  evening  of  the  day  of  operation  the  patient  said  "  yes  "  and 
"  no  "  for  the  first  time  since  the  injury,  and  after  that  time  his  recov- 
ery of  speech  and  of  power  was  progressive.  After  a  few  days  it  was 
noticed  that  he  was  rhuch  more  intelligent  and  no  longer  emotional. 
Power  in  his  leg  began  to  increase  very  soon,  and  two  months  after  the 
operation  he  was  able  to  walk  with  a  cane.  The  return  of  speech  was 
slow  but  continuous,  and  it  appeared  as  if  he  were  learning  a  new 
language.  He  repeated  words  after  another  until  he  had  learned 
them.  He  talked  in  monosyllables  for  the  first  three  mouths,  then  he 
began  to  put  two  words  together,  and  then  used  short  sentences  of 
three  or  four  words.  Fifteen  months  after  the  operation  he  was  able 
to  practise  medicine  in  his  native  town,  was  able  to  walk  without  a 
cane,  could  name  at  once  any  object  shown  to  him,  but  in  talking 
used  only  a  few  words  at  a  time,  not  speaking  fluently,  wrote  with  his 
left  hand,  and  had  it  not  been  for  the  loss  of  power  in  the  right  hand, 
might  have  been  considered  fairly  well.  The  paralysis  of  the  right 
hand  remained,  and  with  it  a  marked  diminution  of  tactile  and  pain 
senses,  two  points  being  felt  as  one  when  two  centimetres  apart  on  the 
tips  of  the  fingers.  The  muscular  and  temperature  senses  were  perfect. 
He  could  move  the  arm  and  forearm  in  all  directions  with  much  force, 
and  could  supinate  the  hand,  but  movements  below  the  wrist  were  very 
slight,  the  fingers  being  flexed  and  rigid.  The  reflexes  were  much  less 
exaggerated  than  before  the  operation,  clonus  not  being  elicited  except- 
ing at  the  ankle ;  the  head  could  be  turned  in  any  direction,  and  the 
pupils  were  equal.  In  this  condition  he  remained  for  ten  years,  until 
he  died  of  pneumonia. 

This  subject  being  chiefly  surgical  the  reader  is  referred  for  further 
details  and  for  the  technique  of  operation  to  Harvey  Cushing's  article 
on  Brain  Surgery  in  Keen's  System  of  Surgery. 


CHAPTER   XLI. 

CEEEBRAL  MENINGITIS. 

Epidemic  Cerebro-spinal  Meningitis.     Endemic  Meningitis.    Hydrocephalus.     Septic 
Meningitis.     Secondary  Meningitis.     Tuberculous  Meningitis.     Syphilitic  Meningitis. 

Meningitis  is  an  inflammation  of  the  pia  mater.  It  is  an  acute 
disease  in  the  majority  of  cases,  and  is  then  of  bacterial  origin.  There 
are  several  classes  of  cases  : 

1.  Epidemic  cerebro-spinal  meningitis,  or  spotted  fever,  which  is 
due  to  the  invasion  of  the  pia  by  the  diplococcus  intracellularis. 

2.  Endemic  meningitis  of  infants  due  to  infection  by  various  micro- 
organisms. 

3.  Septic  meningitis,  which  occurs  with  wounds,  fractures,  and 
operations,  or  after  otitis  media,  and  is  due  to  the  invasion  of  the  pia 
by  streptococcus  or  staphylococcus ;  and  secondary  meningitis  which 
occurs  as  a  complication  of  pneumonia,  ulcerative  endocarditis,  em- 
pyema, typhoid  and  typhus  fever,  influenza,  the  eruptive  fevers  of 
childhood,  erysipelas,  and  in  fact  any  form  of  disease  due  to  a  micro- 
organism. In  these  cases  the  germ  of  the  disease  attacks  the  pia  mater, 
and  has  been  found  in  it.  The  list  of  bacteria  which  have  been 
detected  in  the  pia  is  increasing  daily,  a  large  variety  having  thus  far 
been  found.  There  is  no  special  bacillus  of  meningitis,  though  various 
investigators  have  isolated  various  forms  and  made  a  claim  to  its  ex- 
clusive causative  action.  Thus  Still  has  found  a  diplococcus  allied  to 
that  of  cerebro-spinal  meningitis,  Weichselbaum  has  described  a  men- 
ingococcus intracellularis,  and  Bibes  a  micrococcus  lanceolatus. 

4.  Tuberculous  meningitis,  due  to  the  direct  invasion  of  the  pia  by 
the  tubercle  bacillus. 

5.  Syphilitic  meningitis,  due  to  an  exudation  of  gummy  material 
into  the  pia  mater. 

The  entire  pia  of  the  convexity  and  of  the  base  may  be  involved  in 
the  inflammation,  but  certain  forms  of  meningitis  appear  to  select  cer- 
tain localities.  Thus  in  the  epidemic  form  the  entire  pia,  both  of  the 
brain  and  of  the  spinal  cord,  is  invaded.  In  the  septic  and  secondary 
forms  the  pia  of  the  convexity  is  particularly  affected,  and  that  of  the 
base  may  escape.  In  these  cases  the  process  may  be  localized  in  a 
small  area.  This  occurs  especially  after  wounds,  after  otitis  media, 
and  nasal  disease.  In  meningitis  of  infants  the  base  is  particularly 
affected  and  hydrocephalus  is  a  frequent  result.  In  tubercular  menin- 
gitis the  pia  on  the  base  alone  may  be  the  portion  inflamed,  hence  this 
form  has  been  named  basilar  meningitis.     In  syphilitic  meningitis  the 

713 


714 


CEBEBRAL  MENINGITIS. 


pia  on  the  base  near  the  posterior  perforated  space  and  about  the  crus 
cerebri  or  at  the  sides  of  the  medulla  and  pons  is  usually  aifected.  It 
is  evident  that  this  difference  in  location  will  cause  a  great  difference 
in  the  symptoms  of  the  various  types. 

Pathology. — After  a  short  period  of  hypersemia  the  congested  pia 
becomes  covered  with  an  exudation  of  serum,  lymph,  fibrin,  and  pus. 
The  serum  infiltrates  its  meshes  and  collects  between  the  pia  and  the 
brain,  making  the  latter  oedematous.  It  also  collects  below  the  arach- 
noid, lifting  this  so  that  it  appears  to  contain  cysts.  The  fibrin  is  de- 
posited in  flakes  on  and  in  the  pia,  rendering  it  less  transparent  than 
normal  and  covering  it  and  the  brain  with  a  thick  opaque  lymph.  The 
pus  is  infiltrated  through  the  meshes  of  the  pia,  and  as  it  increases  in 

Fig.  298. 


Acute  meningitis  of  the  convexity  of  the  brain.    The  thickening  of  the  pia,  its  infiltration  with  the 
products  of  inflammation,  and  the  invasion  of  the  cortex  by  cells  is  seen. 

amount  fills  up  the  space  between  the  convolutions  along  the  lines 
where  the  pia  dips  down,  then  it  collects  in  the  sulci,  and  finally  may 
form  masses  of  a  yellowish-green  color,  covering  the  surface  and  filling 
up  its  irregular  spaces.  The  cortex  of  the  brain  on  which  this  serous 
and  purulent  exudation  lies  soon  becomes  affected;  in  fact,  in  every 
case  one  has  to  deal  rather  with  a  meningo-encephalitis  than  with  a 
simple  meningitis.  The  cortex  is  greatly  congested.  Wherever  the 
pial  vessels  dip  into  the  cortex,  serum,  lymph,  and  pus  are  exuded, 
and  small  hemorrhages  and  little  collections  of  pus  are  found  every- 
where in  the  cortical  layers.  Occasionally  large  areas  of  softening  or 
little  abscesses  are  found  in  the  brain.  The  pia  covering  the  cerebel- 
lum and  the  cranial  nerves  is  similarly  affected,  and  in  cases  where 
the  process  extends  downward  the  pia  along  the  entire  length  of  the 
spinal  cord  may  be  inflamed  and  covered  with  lymph  and  pus. 


PATHOLOGY  OF  CEREBRAL  MENINGITIS.  715 

The  pia  extends  into  the  ventricles,  and  hence  a  similar  process  goes 
on  there,  i^^sulting  in  an  eifiisiou  and  consequent  distention  of  the 
ventricles  with  serum,  which  soon  contains  flocculi  of  fibrin  and  pus. 
The  cerebro-spinal  fluid  contains  similar  flocculi,  cells,  and  free  bac- 
teria, and  hence  by  lumbar  puncture  and  examination  of  the  fluid 
obtained  the  diagnosis  of  meningitis  can  always  be  made. 

In  cases  in  infants  which  last  for  more  than  a  week  a  distention  of 
the  skull  commences,  due  to  this  accumulation  of  fluid  in  the  ventricles, 
and  this  results  in  a  separation  of  the  sutures,  which  goes  on  until  a 
hydrocephalic  shape  of  the  head  and  a  great  increase  in  its  size  is 
produced. 

The  collection  of  fluid  in  the  lateral  ventricles  only  occurs  when  an 
adhesion  has  formed  in  the  membranes,  closing  the  foramina  and  ob- 
structing the  free  communication  between  the  ventricles  and  the  sub- 
arachnoid space.  The  choroid  plexuses  of  the  ventricles  and  the  epi- 
thelium covering  them  secrete  the  cerebro-spinal  fluid,  which  is  not  a 
mere  transudation  from  the  blood.  Being  constantly  secreted  it  must 
be  drained  away  and  absorbed.  This  drainage  occurs  through  the 
cere"bro-spinal  foramen  and  the  two  lateral  forarhina  of  the  fourth 
ventricle  which  establish  a  communication  between  the  ventricles  and 
the  general  subarachnoid  space.  The  lateral  ventricles  communicate 
by  the  foramen  of  Monroe  with  the  third  ventricle,  and  this  empties  into 
the  fourth  ventricle  by  way  of  the  aqueduct  of  Sylvius,  the  minute 
size  of  which  secures  a  slow  drainage.  If  any  obstruction  occurs  to 
such  a  flow,  either  by  compression  of  the  aqueduct  or  by  closure  of 
these  foramina,  an  accumulation  of  fluid  will  occur.  In  meningitis, 
particularly  of  the  base,  there  occurs  an  adhesion  of  the  membranes 
or  an  effusion  of  lymph  which  closes  these  foramina.  Drainage  being 
impossible  fluid  accumulates  in  the  ventricles  and  causes  hydrocephalus. 
Barlow^  believes  that  in  many  cases  of  congenital  hydrocephalus  and 
of  chronic  hydrocephalus  developing  slowly  without  symptoms  of 
meningitis,  a  slight  attack  or  a  low  grade  of  meningitis  has  occurred 
at  the  outset  which  has  not  been  noticed  or  has  recovered.  In  the 
majority  of  cases  of  meningitis  in  infants,  if  death  does  not  take  place 
before  the  third  week,  hydrocephalus  appears.     (See  page  522.) 

In  tuberculous  meningitis  there  is  rarely  an  exudation  of  pus. 
There  is  a  deposit  of  miliary  tubercles  through  the  pia,  both  diffuse 
and  in  conglomerate  masses.  These  are  small  white  egg-shaped  bodies, 
usually  first  appearing  along  the  bloodvessels  and  always  found  on  their 
walls.  An  exudation  of  serum  lymph,  and  fibrin  occurs,  and  these 
form  a  thick  mucilaginous  material  in  which  the  miliary  tubercles  are 
imbedded.  This  process  is  always  much  more  marked  about  the  base 
of  the  brain,  where  the  many  irregular  spaces  are  filled  with  the  new 
material.  It  may  extend  to  the  convexity,  however,  and  then  the 
exudation  is  more  evident  along  the  sulci  and  fissures,  especially  in  the 
fissure  of  Sylvius.  The  miliary  tubercles  may  be  found  scattered 
widely  over  the  entire  extent  of  the  pia  and  in  the  ventricles.     The 

^  Allbutt's  System  of  Medicine,  vol.  vii.,  p.  493. 


716  CEREBRAL  MEmNGlTlS. 

ventricles  are  always  distended  with  cloudy  serum  in  which  fibriu  and 
blood  are  found.  This  distention  may  be  extreme  and  may  produce 
hydrocephalus.  The  exudation  surrounds  the  nerves  upon  the  base 
and  causes  a  neuritis.  A  diifuse  encephalitis  of  the  cortex  or  a  hemor- 
rhagic encephalitis,  either  localized  or  general,  may  accompany  tuber- 
cular meningitis.  Masses  of  tuberculous  material  in  a  state  of  cheesy 
degeneration  are  also  found  in  the  membranes  or  in  the  brain  in  some 
cases.     (Fig.  243,  page  573.) 

Syphilitic  meningitis  is  characterized  by  a  serous  and  fibrinous  exu- 
dation in  the  membranes  attended  by  a  deposit  of  mucilaginous  material 
of  a  gummy  nature  which  is  semi-translucent,  thick,  and  viscid,  and 
which  surrounds  the  nerves  and  fills  up  the  spaces  on  the  base  or  in 
the  sulci  of  the  convexity.  It  begins  to  appear  along  the  bloodvessels, 
but  soon  becomes  extensive.  It  is  not  attended  by  the  production  of 
pus,  and  there  is  not  often  any  exudation  of  serum  into  the  ventricles. 
The  exudation  invades  the  cranial  nerves,  especially  the  optic  and  ocu- 
lomotor, and  causes  a  degenerative  neuritis.  The  pia  is  thickened  by  a 
production  of  connective  tissue  which  causes  its  firm  adhesion  to  the  dura 
and  to  the  brain.  Areas  of  softening  on  the  cortex  are  commonly  found. 
(See  Fig.  ^98,  page  714.)  The  characteristic  syphilitic  endarteritis  is 
always  present  in  the  arteries  of  the  base.     (See  Fig.  288,  page  687.) 

Lumbar  puncture,  first  done  by  Quincke  in  1891,  is  a  valuable  aid 
to  diagnosis  in  all  forms  of  meningitis.  The  patient  is  placed  on  the 
left  side,  the  legs  being  drawn  up  and  the  body  bent  far  forward.  A 
line  is  drawn  between  the  posterior-superior  edges  of  the  ilia,  which 
will  pass  across  the  space  between  the  third  and  fourth  lumbar  ver- 
tebrae. The  lower  edge  of  the  spinous  process  of  the  third  lumbar  ver- 
tebra is  found,  and  puncture  is  made  by  a  long  hypodermic  needle  just 
below  this  in  children,  and  1  cm.  to  one  side  of  it  in  adults.  The 
hypodermic  needle  is  thrust  in  between  the  laminae,  and  should  be  pointed 
a  little  upward  and  inward.  It  can  be  felt  to  penetrate  the  membrane, 
and  then  its  point  can  be  moved  more  freely  than  in  the  solid  tissue. 
The  syringe  is  then  unscrewed  from  the  needle  and  the  fluid  allowed 
to  run  out.  It  must  never  be  exhausted  by  suction.  A  small  trocar 
may  be  employed  in  place  of  a  hypodermic  needle.  A  few  cubic  cen- 
timetres only  of  the  fluid  is  to  be  taken  in  a  test  tube.  The  pressure 
under  which  it  flows  is  to  be  noticed,  and  if  it  spurts  out  of  the  needle 
the  internal  pressure  is  abnormally  high.  This  occurs  in  meningitis. 
As  it  will  not  run  the  patient's  head  and  body  may  be  elevated,  and 
thus  a  few  drops  may  be  obtained.  It  is  usually  easy  to  obtain  10 
c.cm.  Normal  cerebro-spinal  fluid  is  clear,  straw-colored  and  contains 
a  few  cells  and  not  more  than  0.2  per  cent,  of  albumin.  In  menin- 
gitis it  is  cloudy,  contains  a  little  blood,  floccculi  of  fibrin,  polynuclear 
leucocytes,  pus,  and  micro-organisms.  It  usually  contains  more  albumin 
than  normal.  On  standing  the  cerebro-spinal  fluid  coagulates,  and  the 
coagulum  forms  more  quickly  in  cases  of  meningitis  and  is  firmer  than 
in  health.  It  is  the  existence  of  pus,  leucocytes,  and  bacteria  which 
are  the  most  important  diagnostic  signs  of  meningitis.     Cultures  should 


SYMPTOMS.  717 

always  be  imde  of  the  fluid.  The  finding  of  streptococcus,  staphylo- 
coccus, pneumococcus,  or  diplococcus  intracellularis,  or  of  tubercle 
bacilli,  makes  it  certain  that  a  meningitis  is  present.  In  tuberculous 
meningitis  mononuclear  lymphoid  cells  are  also  found,  while  in  the 
other  forms  poly  nuclear  cells  are  more  common.  When  the  diplo- 
coccus or  pneumococcus  is  not  found  in  the  cerebro-spinal  fluid  it  is 
occasionally  found  in  the  blood  and  in  such  cases  direct  injection  of 
10  c.c.  of  the  antiserum  into  a  vein  repeated  daily  has  resulted  in 
cures. 

Symptoms.  Epidemic  Cerebro-spinal  Meningitis.  —  The  disease, 
though  occasionally  occurring  in  epidemic  form,  particularly  in  winter 
and  spring,  both  in  cities  and  in  the  country,  is  more  commonly  seen 
as  a  sporadic  affection,  and  is  quite  rare.  Children  are  more  suscep- 
tible to  the  infection  than  adults.  Unwholesome,  dirty  surroundings, 
malnutrition,  and  overexertion,  either  mental  or  physical,  are  supposed 
to  make  one  more  susceptible.  Though  infectious,  the  disease  is  not 
contagious,  and  is  not  carried  in  clothing. 

The  period  of  incubation  is  not  determined. 

The  symptoms  appear  suddenly,  with  a  chill,  temperature  of  104°  F. 
or  higher,  full  rapid  pulse,  headache,  pains  in  the  back,  and  vomiting, 
which  is  projectile.  The  patients  feel  very  ill,  are  restless  and  irri- 
table, and  soon  complain  of  light  and  sound.  Stupor  and  coma  come 
on  rapidly.  Within  a  day  or  two  of  the  onset  the  headache  becomes 
very  intense,  and  stiifness  in  the  back  of  the  neck  appears,  which  is 
very  painful.  The  head  is  held  rigid  or  is  drawn  back,  the  back  also 
becomes  stiff,  so  that  children  can.  be  raised  from  the  bed  by  lifting  the 
back  of  the  head,  and  a  state  of  opisthotonus  develops,  attended  by 
great  pain  in  the  back  and  limbs  and  extreme  hyperaesthesia  to  all 
forms  of  sensation.  The  slightest  touch  causes  great  pain.  In  addition 
to  the  rigidity  of  the  muscles  there  are  occasional  spasms  of  a  clonic 
or  tonic  kind  in  the  extremities,  the  face,  too,  being  drawn,  and  tremor 
in  the  limbs  may  be  observed.  The  pupils  are  dilated,  and  sometimes 
unequal,  and  do  not  react  to  light.  Grinding  of  the  teeth  and  strabis- 
mus are  early  symptoms. 

Within  two  days  of  the  onset  delirium  of  an  active  character  begins, 
and  this  continues,  alternating  with  stupor  and  coma,  for  a  long  time. 
The  mind  is  always  clouded  and  filled  with  illusions,  and  the  patients 
have  no  memory  of  what  has  occurred  when  they  recover.  The 
delirium  may  at  times  be  wild,  and  is  always  worse  at  night  and  when 
the  temperature  rises.  After  the  third  day  an  eruption  appears  in 
the  majority  of  cases,  though  it  is  not  an  invariable  symptom.  It  is 
petechial,  diffused  over  the  entire  body,  but  deeper,  and  purple  in 
spots.  Herpes  appears  on  the  lips  early ;  erythema,  pemphigus,  and 
gangrene  of  the  skin  have  all  been  seen  in  various  cases  in  addition  to 
the  petechige.  Any  scratch  on  the  skin  leaves  a  dark  red  stripe,  the 
tdche  e^rehrale  of  Trousseau.  The  gastro-intestinal  functions  are 
affected  from  the  start,  nausea,  vomiting,  diarrhoea,  or  more  commonly, 
obstinate  constipation   being   present.     The  urine  contains  albumin, 


718  CEREBRAL  MENINGITIS. 

casts,  and  sometimes  blood.  It  is  sometimes  retained,  but  may  be 
passed  involuntarily,  as  are  also  the  feces.  The  spleen  is  enlarged. 
Contractures  soon  develop,  and  the  limbs  are  flexed  until  paralysis 
ensues  and  they  relax.     The  reflexes  are  increased. 

There  are  some  cases  of  a  malignant  type  which  end  within  the  first 
two  or  three  days.  Many  cases  prove  fatal  within  a  week.  If  the 
patients  do  not  die  of  fever  or  of  heart  failure  within  a  week  of  the 
onset  they  usually  have  a  remission  in  the  symptoms,  but  these  return 
again,  and  the  case  may  go  on  for  many  weeks  with  varying  intensity. 
There  is  no  typical  temperature  curve,  and  great  variations  are  pos- 
sible. In  one  patient  who  recovered  I  saw  a  rise  on  five  occasions 
during  two  months  to  106°  F.,  the  ordinary  variation  being  between 
101°  and  103°  F.,  with  occasional  falls  to  normal.  A  sudden  rise  or 
a  sudden  fall  is  unfavorable.  The  temperature  may  be  intermittent. 
The  pulse  may  be  rapid,  especially  in  children,  or  it  may  be  as  slow 
as  forty  per  minute.  It  is  frequently  intermittent.  The  respiration  is 
irregular,  at  times  of  the  Cheyne-Stokes  variety,  and  attended  by  sigh- 
ing and  yawning.  It  is  sometimes  difficult  to  feed  and  care  for  those 
patients,  as  they  resist  In  their  delirium  all  attempts  at  nursing,  and 
rapid  and  extreme  emaciation  results.  The  most  prominent  symptoms 
during  the  long  illness  are  headache,  delirium,  stupor,  insomnia,  rigidity 
of  the  muscles,  especially  of  the  neck  and  back,  hypersensitiveness  to 
touch,  to  sound,  and  to  light,  and  a  gradual  loss  of  voluntary  power. 

The  cranial  nerves  are  almost  always  seriously  affected.  Strabismus, 
which  develops  early,  persists.  It  is  often  attended  by  nystagmus. 
Vision  may  be  obscured  by  keratitis,  and  optic  neuritis  and  atrophy 
may  develop  and  finally  render  the  patient  blind,  though  in  two  patients 
I  have  seen  a  partial  recovery  of  sight,  sector-like  defects  in  the  visual 
field  remaining.  Deafness  is  a  common  symptom,  due  to  otitis  media 
or  to  an  affection  of  the  auditory  nerve,  and  may  be  permanent,  lead- 
ing to  deaf-mutism  in  children.  Facial  paralysis  is  very  common. 
Difficulty  in  swallowing  and  in  articulation  are  observed  in  many  cases. 
General  convulsions  may  occur  from  time  to  time  in  the  course  of  the 
case. 

Under  the  antiserum  treatment  of  Flexner  the  symptoms  rapidly 
subside  and  the  patients  regain  their  consciousness  and  in  the  course 
of  a  few  days  appear  to  be  well.  I  have  seen  a  child  sitting  up  in  her 
crib  and  playing  naturally  with  her  doll,  two  days  after  she  had  been 
in  a  state  of  coma  with  strabismus,  opisthotonus,  and  occasional  con- 
vulsions. If  relapses  occur  when  the  injections  are  stopped,  they  may 
be  resumed. 

The  most  permanent  of  all  the  symptoms  is  a  state  of  mental  apathy 
and  indifference.  Even  after  the  delirium  and  stupor  have  passed  oft' 
the  patients  may  show  no  mental  activity,  may  fail  to  control  the 
sphincters,  may  not  recognize  their  parents,  may  have  no  interest  in 
persons  or  events,  and  may  talk  with  great  difficulty.  I  have  seen  these 
symptoms  remain  for  several  weeks  after  the  temperature  had  become 
normal.     In  the  patients  who  do  not  succumb  to  general  exhaustion 


SYMPTOMS.  719 

or  die  of  a  complicating  pneumonia,  arthritis,  or  cystitis,  or  bed-sores, 
there  is  a  very  slow  return  to  health,  and  sometimes  it  is  six  months 
or  a  year  before  the  mental  and  physical  symptoms  have  entirely  dis- 
appeared. The  severity  of  the  disease  depends  somewhat  upon  the 
character  of  the  epidemic,  some  epidemics  being  very  fatal,  in  some 
the  mortality  being  75  per  cent.,  in  others  being  as  low  as  20  per  cent. 
In  some  epidemics  the  tendency  to  complications  seems  to  be  great. 

In  sporadic  cases  the  prognosis  depends  on  the  intensity  of  the  symp- 
toms, but  it  is  never  well  to  give  up  hope,  as  very  bad  cases  may  recover. 

The  treatment  consists  in  supporting  the  strength  by  fluid  food  and 
alcoholic  stimulants,  given  often  and  freely ;  in  the  application  of  ice 
to  the  head  and  spine  in  bags,  or  of  ice-water  in  coils ;  in  cool  bathing 
when  the  temperature  exceeds  102°  F.;  and  in  the  free  use  of  sedatives, 
of  which  bromides,  phenacetin  or  acetanilid,  sulphonal,  and  trional, 
and  in  many  cases  codeia  or  morphine,  in  small  dos6  frequently  re- 
peated, are  the  best.  Strychnine  should  never  be  given.  Some  authors 
recommend  ergot  in  large  doses.  It  is  questionable  whether  mercury 
or  iodide  of  potassium  are  useful,  though  some  writers  urge  their  use. 
The  discovery  by  Flexuer  ^  of  an  antiserum  for  cerebro-spinal  menin- 
gitis has  been  followed  by  its  successful  use  all  over  the  world.  The 
method  of  its  use  is  as  follows  :  By  lumbar  puncture  15  or  30  c.c.  of 
cerebro-spinal  fluid  is  withdrawn,  and  the  same  amount  of  antiserum  is 
injected  directly  into  the  spinal  canal,  slowly  and  carefully,  to  avoid 
the  production  of  symptoms  due  to  increased  pressure.  The  injection 
is  to  be  repeated  every  day  for  three  or  four  days.  The  earlier  the 
injection  in  the  course  of  the  disease  the  better  the  results.  Its  direct 
effect  can  be  observed  by  testing  the  cerebro-spinal  fluid  daily  for 
diplococci,  which  rapidly  disappear  under  its  use.  By  the  use  of  the 
serum  the  mortality  has  been  reduced  from  89  per  cent,  to  25  per  cent, 
in  several  severe  epidemics.  The  antiserum  is  to  be  obtained  at  the 
Rockefeller  Institute  in  New  York.  In  the  stage  of  recovery  iodide 
is  useful  in  doses  of  twenty  grains  three  times  a  day  for  adults. 

2.  Meningitis  in  Infants ;  Hydrocephalus.  —  Infants  appear  to  be 
much  more  liable  to  the  infection  of  meningitis  than  adults,  and  in 
them  the  picture  of  the  disease  dififers  greatly  from  that  already  pre- 
sented. Both  sexes  are  equally  liable.  The  majority  of  cases  occur 
during  the  first  and  second  years  of  life,  though  no  age  is  exempt. 
Barlow's  statistics  point  to  a  greater  susceptibility  between  the  third 
and  sixth  months.  Catarrhal  diseases  of  the  intestines,  bronchi,  or 
nose  precede  the  onset  in  many  cases.  A  history  of  injury  to  the 
head  is  obtained  in  not  a  few. 

The  symptoms  develop  acutely,  and  are  well  marked  within  three 
days  of  the  onset.  Vomiting,  convulsions,  retraction  of  the  head, 
screaming,  and  irritability  of  temper  are  the  first  symptoms,  and  are 
soon  followed  by  sleepiness  and  languor,  by  bulging  of  the  fontanelle, 
by  strabismus  and  nystagmus,  and  by  some  inequality  or  abnormality 
in  the  action  of  the  pupils,  by  champing  movements  of  the  lower  jaw, 

^  Jour,  of  Experimental  Medicine,  Jan.,  1908. 


720  CEREBRAL  MENINGITIS. 

lips,  and  tongue,  and  by  extensor  spasms  of  the  limbs,  and  in  some 
cases  by  opisthotonus.  Paralysis  of  the  face  and  limbs  is  occasionally 
seen,  but  is' rare.  The  infant  undoubtedly  suffers  from  great  pain,  as 
is  shown  by  the  constant  crying  and  screaming,  which  are  kept  up  at 
night  and  by  day,  and  by  pulling  its  hair.  This  is  an  early  symp- 
tom, and  subsides  later  when  the  stage  of  hydrocephalus  is  reached. 
Irregularity  of  respiration  is  often  noted,  and  the  pulse  is  either  very 
fast  or  very  slow.  The  skin  is  flushed  and  shows  the  mark  of  any 
irritation  as  a  broad  red  line  or  spot.  The  temperature  varies  greatly 
between  100°  and  106°  F.  The  abdomen  is  often  retracted;  consti- 
pation is  more  frequent  than  diarrhoea.  One  of  the  most  noticeable 
symptoms  is  the  rapid  emaciation,  and  these  children  become  mere 
skeletons  in  the  course  of  a  couple  of  weeks.  Hydrocephalus  usually 
develops  in  the  second  week  of  the  disease.  In  addition  to  the  in- 
crease in  the  size  and  change  in  the  shape  of  the  head,  there  is  often 
a  slight  bulging  of  the  eyeballs,  and  strabismus  appears.  This  con- 
dition may  persist  for  a  long  time,  or  it  may  gradually  subside  as  the 
child  recovers. 

The  course  of  a  simple  meningitis  may  be  rapid,  and  a  fatal  termi- 
nation may  occur  within  a  week.  This  rapid  course  occurs  chiefly  in 
the  vertical  and  sometimes  in  basal  cases.  More  often  the  symptoms 
persist  a  long  time  and  the  case  runs  a  course  which  extends  over 
several  weeks.  The  variation  is  so  great  in  different  cases  that  no 
average  duration  can  be  stated.  There  are  some  cases  which  continue 
even  for  four  or  five  months,  and  then  recover.  That  a  large  percent- 
age die  must  be  admitted.  Authorities  make  the  mortality  from  60  to 
80  per  cent.  It  has  been  thought  by  some  that  children  never  recover 
from  meningitis,  but  this  is  not  my  experience.  I  have  seen  some  well 
pronounced  cases  in  the  Baby's  Hospital  which  terminated  in  recovery. 
Even  cases  which  develop  hydrocephalus,  if  this  is  moderate,  may  come 
to  a  standstill  and  gradually  get  well.  These  patients  may  be  left  blind 
or  deaf,  or  they  may  show  a  spastic  gait  for  years,  or  they  may  show 
mental  defects,  but  nevertheless  they  recover  from  the  acute  meningitis. 
Occasionally  during  the  stage  of  improvement,  or  even  after  recovery 
appears  to  be  complete,  sudden  death  occurs.  There  is  no  disease, 
therefore,  in  which  the  prognosis  is  more  uncertain. 

The  treatment  must  be  quite  similar  to  that  described  in  the  epi- 
demic form.  Leeches  behind  the  ears,  ice  to  the  head  and  neck,  or  a 
cold  coil,  antipyretics,  and  cool  sponging  are  of  service  in  keeping  down 
the  temperature.  The  child  should  be  nourished  as  well  as  possible 
on  milk  properly  modified  to  its  age,  and  on  broths.  It  may  be  neces- 
sary to  feed  by  gavage  if  there  is  much  vomiting.  There  are  no  drugs 
which  act  on  the  bacteria,  and  hence  there  is  no  means  of  decreasing 
the  pathological  process.  In  some  cases  iodide  has  been  of  service, 
and  it  may  be  given  if  the  stomach  will  retain  it  in  one  to  three-grain 
doses  four  or  five  times  a  day. 

It  is  not  to  be  forgotten  that  some  cases  are  secondary  to  otitis  media ; 
hence  if  any  evidence  of  this  is  present  free  drainage  must  be  secured 


SYMPTOMS.  721 

by  incising  the  drum  or  by  operating  on  the  mastoid.  Cases  are  now 
frequently*^reported  where  the  mastoid  operation,  followed  by  trephin- 
ing, has  resulted  in  the  evacuation  of  a  small  collection  of  pus  and  has 
been  followed  by  recovery. 

3.  Septic  and  Secondary  Forms  of  Meningitis.  —  The  symptoms  of  a 
septic  meningitis  always  develop  after  some  one  of  its  causative  con- 
ditions has  been  in  progress  for  some  time.  Hence  they  are  preceded 
by  the  symptoms  of  a  cranial  fracture  or  operation,  an  otitis  media,  a 
chronic  nasal  disease,  or  an  abscess,  or  erysipelas  of  the  face.  If,  in 
the  course  of  any  of  these  affections  the  patient  has  a  sudden  chill, 
with  rise  of  temperature,  rapid  pulse,  severe  continuous  general  head- 
ache, and  delirium  or  convulsions,  and  if  thus  an  illness  begins  which 
is  evidently  septic,  is  attended  by  a  stuporous  mental  state,  som- 
nolence, vertigo,  vomiting,  and  a  fever  running  from  101°  to  104°  F. 
daily,  there  is  every  reason  to  believe  that  a  septic  meningitis  has  de- 
veloped. The  general  symptoms  mentioned  usually  appear  before  any 
local  signs  of  brain  disease.  And  these  will  depend  largely  on  the  area 
which  is  affected  by  the  meningitis.  Thus,  in  fractures  of  the  skull 
which  lie  over  the  motor  or  sensory  areas  there  appear  local  spasms,  or 
aphasia,  or  paralysis,  or  disturbances  of  sensation,  first  hypersesthetic 
conditions,  and  later  loss  of  sensation.  After  otitis  media  there  may 
be  no  local  signs  of  meningitis,  as  the  temporal  lobe  on  the  base  has  no 
known  function  ;  but  if  the  disease  advances  upward  on  the  left  side  a 
sensory  aphasia  may  appear.  If  the  meningitis  is  on  the  base  the 
cranial  nerves  may  become  involved ;  nystagmus,  strabismus,  twitch- 
ing, or  paralysis  of  the  face,  grinding  of  the  teeth,  and  optic  neuritis 
develop.  These  sometimes  occur  after  otitis  media.  If  the  meningitis 
starts  from  nasal  disease  there  are  no  local  signs  excepting  loss  of  smell 
and  a  greater  degree  of  mental  disturbance  than  in  the  other  cases. 

The  same  general  symptoms  developing  in  the  course  of  any  of  the 
infectious  diseases  will  indicate  that  meningitis  has  begun  as  a  compli- 
cation. The  headache  which  is  always  present  in  meningitis  is  severe 
and  causes  great  agony.  The  patient  grasps  the  head  and  moans  with 
pain  even  during  his  delirium  and  stupor.  Children  pull  the  hair, 
knock  the  head  upon  the  pillow,  and  scream,  even  in  their  sleep.  The 
sudden  piercing  cry  of  pain  which  wakens  the  child  is  known  as  the 
cry  of  meningitis.  The  pain  varies  in  intensity,  and  is  worse  at  night 
or  when  the  temperature  rises.  The  delirium  is  another  characteristic 
symptom  of  meningitis.  It  is  sometimes  a  low  muttering  delirium 
from  which  the  patient  can  be  momentarily  aroused,  but  he  does  not 
answer  questions  intelligently.  At  times  it  is  a  more  active  delirium, 
the  patient  wishes  to  get  out  of  bed,  to  dress  and  go  out,  or  is  wildly 
excited,  and  struggles  with  his  nurses  and  has  to  be  tied  in  bed.  In 
the  intervals  of  delirium  there  is  a  stupor  which  may  deepen  into  coma. 
The  fever  of  meningitis  is  high.  It  dose  not  fall  or  rise  suddenly,  as 
in  thrombosis  of  the  lateral  sinus,  or  in  pyaemic  states,  but  runs  a 
course  between  100°  and  104°  F.,  and  occasionally  the  patient  may 
have  a  chill.  The  pulse,  which  at  first  was  rapid^  varies  as  the  disease 
46 


722  CEBEBBAL  MENINGITIS. 

goes  on.  It  often  becomes  intermittent  and  irregular.  It  may  for  a 
few  hours  be  slow,  even  40.  It  does  not  vary  with  the  temperature. 
The  general  hypersesthetic  state  of  all  the  senses  is  very  characteristic 
of  meningitis ;  the  patient  begs  to  be  left  alone  in  the  dark,  starts  at 
slight  noises,  and  cannot  endure  to  be  touched  or  examined.  There 
are  twitchings  of  the  muscles,  which  are  increased  by  tapping,  and 
there  is  often  an  increase  in  all  the  tendon  reflexes.  The  neck  and 
back  become  stiff  and  painful  early  in  the  course  of  the  disease.  And 
various  cranial  nerve  symptoms  indicate  that  the  eifusion  on  the  base 
has  begun. 

The  pupils  are  usually  contracted  at  first,  do  not  dilate  in  the  dark, 
and  do  not  react  to  light.  Later  they  may  become  dilated,  are  often 
unequal,  and  the  condition  of  the  pupil  varies  from  day  to  day.  In 
some  cases  the  pupil  contracts  to  light,  but  immediately  dilates  again, 
and  continues  to  change  in  size;  this  is  termed  hippus.  Ptosis,  par- 
alysis of  some  of  the  ocular  muscles,  with  strabismus  and  nystagmus, 
soon  develop.  Optic  neuritis  occurs  in  about  60  per  cent,  of  the  cases. 
The  facial  nerves  may  be  irritated,  causing  a  twitching  of  the  facial 
muscles  and  trismus  or  paralysis.  As  the  case  progresses  general  con- 
vulsions may  occur,  followed  by  coma.  Monoplegia  or  hemiplegia  may 
develop  at  any  stage.  There  is  an  irritation  or  paralysis  of  the  vaso- 
motor system  shown  by  blueness  of  the  extremities,  a  reddening  of  the 
skin  when  scratched  or  rubbed,  and  profuse  sweating.  There  is  ob- 
stinate constipation.  The  urine  may  be  retained  or  may  dribble.  It 
often  contains  albumin,  and  is  of  high  specific  gravity  and  scanty. 
The  spleen  is  enlarged.  In  the  later  stages  there  may  be  a  tendency 
to  bed-sores. 

The  duration  of  a  meningitis  may  be  from  four  days  to  two  weeks. 
In  rapid  cases  all  the  symptoms  appear  in  great  intensity,  the  tem- 
perature rises  high,  the  pulse  is  weak  and  rapid,  coma  comes  on,  and 
the  patients  have  Cheyne-Stokes  respiration,  and  die  either  in  coma  or 
in  convulsions.  In  slow  cases  the  stage  of  irritation  gives  place  to  a 
stage  of  paralysis  and  the  patient  is  exhausted  by  the  fever. 

I  have  seen  several  cases  which  ran  a  slower  course  and  in  which 
,  symptoms  persisted  for  four  or  even  six  weeks.  In  these  cases  remis- 
sions in  the  symptoms,  with  subsequent  exacerbations,  took  place ;  but 
the  general  symptoms  of  fever,  headache,  somnolence,  and  progressive 
emaciation  were  constant.  The  formation  of  abscess  in  the  brain  or 
the  occurrence  of  thrombosis  of  one  of  the  sinuses,  either  of  which 
may  be  a  complication,  makes  the  prognosis  hopeless. 

The  diagnosis  of  meningitis  is  to  be  made  by  an  examination  of  the 
cerebro-spinal  fluid  obtained  by  lumbar  puncture  (see  page  706),  and 
this  should  be  tried  in  all  doubtful  cases. 

The  diagnosis  from  abscess  of  the  brain  and  from  thrombosis  of  the 
lateral  sinuses  has  been  considered  on  pages  558  and  565. 

The  prognosis  in  septic  and  secondary  meningitis  is  always  serious. 
Patients  rarely  recover.  Nevertheless,  the  possibility  of  recovery  has 
been  proven  in  many  cases  in  every  variety  of  the  disease,  and  hence 


SYMPTOMS.  723 

it  is  always  legitimate  to  hold  out  some  hope.  It  is  also  to  be  remem- 
bered thai  there  are  many  cases  presenting  serious  nervous  symptoms 
quite  similar  to  those  of  meningitis  in  their  character,  but  not  quite 
equal  to  them  in  severity,  which  pass  off  in  the  course  of  four,  six,  or 
eight  days.  I  have  seen  many  such  cases  in  connection  with  otitis 
media.  I  have  seen  some  in  children  complicating  other  diseases  or 
appearing  alone.  We  do  not  know  what  the  pathology  of  such  cases 
is.  They  have  been  ascribed  to  cerebral  hyperemia,  to  serous  men- 
ingitis, to  oedema  of  the  brain,  to  non-purulent  encephalitis,  and  to 
direct  toxaemia  of  the  brain.  The  only  means  of  diagnosis  of  these 
cases  from  meningitis  is  by  watching  their  course  and  observing  the 
recovery.  Hence  in  the  early  stage  of  supposed  meningitis  of  a  mild 
type  the  prognosis  must  be  guarded,  as  the  diagnosis  may  be  wrong. 

The  treatment  of  septic  meningitis  or  of  secondary  meningitis  should 
begin  by  an  active  interference  with  the  cause.  Fractures  should  be 
trephined  and  a  possible  source  of  infection  sought  and  free  drainage 
opened.  In  middle-ear  disease  an  early  exploration  of  the  mastoid 
cells  and  prompt  opening  of  the  skull,  with  drainage,  are  often  neces- 
sary. In  abscess  of  the  brain  and  sinus  thrombosis  operation  affords 
relief.  In  other  cases  operations  are  impossible.  Absolute  quiet  in 
bed,  ice  to  the  head,  brisk  purgatives,  leeches  to  the  mastoid  region  or 
temples,  blisters  to  the  neck,  and  the  free  use  of  narcotics,  the  coal- 
tar  products,  and  morphine  are  to  be  employed.  The  temperature  is 
to  be  controlled  by  cool  sponging  rather  than  by  baths,  as  it  is  impos- 
sible to  move  the  patient  into  a  tub.  Antipyrine  and  phenacetin  may 
help  to  reduce  it  if  it  rises  above  103°  F.  The  heart  may  require 
stimulation,  and  for  this  caffeine  is  better  than  either  alcohol  or 
strychnine.  The  delirium  may  be  somewhat  diminished  by  the  free 
use  of  bromide  and  chloral.  If  the  persistent  headache  is  soon  followed 
by  optic  neuritis  it  is  probable  that  a  large  effusion  has  occurred,  and 
it  is  possible  that  lumbar  puncture  may  reduce  this.  In  some  cases  it 
has  succeeded  and  an  improvement  has  followed.  From  50  to  100 
c.c.  of  fluid  have  been  withdrawn  daily  for  several  days  under  strict 
antiseptic  precautions. 

4.  Tuberculous  Meningitis.  —  Children  between  the  ages  of  two  and 
fifteen  years  are  more  commonly  affected  by  tuberculous  meningitis 
than  are  adults,  though  no  age  is  exempt. 

The  symptoms  of  this  form  of  meningitis  are  numerous  and  differ 
so  widely  in  different  stages  of  the  progress  of  the  case  that  all  authors 
prefer  to  group  them  in  periods.  There  are  (I)  a  period  of  incubation, 
when  the  symptoms  are  slight  and  rather  indefinite,  but  really  well 
marked  when  one  looks  backward  and  contrasts  the  condition  with  that 
of  previous  health  ;  (II)  the  period  of  irritative  symptoms  ;  (III)  the 
period  of  paralytic  symptoms  and  coma. 

I.  In  the  period  of  incubation  the  child,  without  reason,  ceases  to 
gain  weight  and  begins  to  lose,  is  fretful  and  uneasy,  shows  little  desire 
to  play,  and  cannot  be  amused  or  diverted  for  any  length  of  time,  very 
often  manifesting  a  marked  change  in  disposition  and  unusual  likes 


724  CEREBRAL  MENINGITIS. 

and  dislikes.  At  night  it  does  not  sleep  soundly,  wakes  on  slight 
noises  and  cannot  be  put  to  sleep,  or  groans  and  cries  in  sleep,  and 
sometimes  grinds  the  teeth.  In  the  daytime  it  may  be  drowsy. 
Vomiting,  occurring  without  apparent  cause  and  recurring  in  attacks, 
is  usually  an  early  symptom,  and  is  attended  by  considerable  prostra- 
tion. Headache  soon  appears,  being  complained  of  by  children  who 
can  talk  and  being  shown  by  crying,  tearing  the  hair,  and  beating  the 
head  in  infants.  During  this  period  the  temperature  is  usually  100° 
or  101°  F.  at  night,  but  nearly  normal  in  the  morning,  the  pulse  is 
more  rapid  than  usual,  and  the  respiration  regular,  but  attended  by 
yawning  and  sighing.  The  appetite  is  poor,  the  bowels  are  often 
constipated;  occasionally  there  is  diarrhoea.  The  urine  is  usually 
increased  in  amount,  and  may  show  high  specific  gravity  and  albumin 
in  small  amount.  The  prodromal  stage  is  longer  in  tuberculous  menin- 
gitis than  in  other  forms. 

As  tuberculous  meningitis  is  secondary  to  tuberculosis  elsewhere  in 
about  80  per  cent,  of  the  cases,  some  symptoms  referable  to  the  lungs, 
or  intestines,  to  the  joints,  or  the  bones,  or  the  spine,  to  the  brain,  or 
to  the  lymphatic  glands,  are  to  be  detected  when  the  child  is  carefully 
examined.  These  symptoms  should  lead  to  the  diagnosis,  even  in  the 
stage  of  incubation.  In  any  case  of  doubtful  nature  the  Calmette  test 
will  prove  the  existence  of  tuberculosis ;  and  lumbar  puncture  will 
yield  fluid  in  which  tubercle  bacilli  can  be  found.  This  stage  may  last 
from  four  days  to  three  or  four  weeks. 

II.  The  stage  of  irritative  symptoms  appears  suddenly,  usually  with 
a  general  convulsion,  followed  by  stupor.  The  child,  who  has  grad- 
ually become  weaker  and  sicker,  complains  of  most  intense  headache, 
is  hypersensitive  all  over,  and  cannot  endure  being  touched,  shuns  the 
light  and  noise,  and  cries  when  disturbed.  It  is  somnolent,  but  cries 
out  in  sleep,  grinds  its  teeth,  and  rolls  its  head,  wearing  oif  the  hair 
from  the  occipital  region.  Then,  without  warning,  it  has  a  general 
convulsion,  which  may  be  repeated  at  intervals.  When  it  comes  out 
of  the  convulsion  it  is  stupid  and  cannot  be  aroused,  but  cries  when 
disturbed.  There  is  usually  some  rigidity  of  the  neck,  which  increases 
rapidly  and  extends  to  the  spine,  so  that  the  entire  body  is  stiff,  and 
after  a  time  the  head  is  drawn  backward.  Sometimes  the  limbs  become 
rigid  and  contracted.  The  reflexes  are  exaggerated.  The  pupils  are 
contracted  and  do  not  respond  to  light,  and  are  often  of  unequal  size. 
There  may  be  nystagmus  and  hippus.  The  temperature  begins  at  this 
time  to  rise  at  night  to  102°  F.,  the  pulse  becomes  faster  and  is  now 
occasionally  intermittent  and  irregular,  and  the  respiration  is  irregular, 
a  series  of  inspirations,  each  deeper  than  the  last,  being  followed  by  a 
sigh  and  cessation  for  a  few  seconds.  This  stage  may  last  for  two  or 
three  days,  or  even  ten  days,  during  which  time  the  stupor  deepens, 
and  the  child  sleeps  more  and  more  continuously,  and  it  is  harder  to 
arouse  or  interest  it.  Delirium  is  a  common  symptom,  the  child  talks 
to  itself,  occasionally  screams  out,  and  may  not  know  its  parents. 
Toward  the  close  of  the  stage  muscular  twitching  is  frequent  and  par- 


SYMPTOMS.  725 

tial  convulsions  may  occur ;  the  abdomen  is  retracted,  tdche  c^rSbrale 
is  well  marked,  and  an  ophthalmoscopic  examination  shows  choked 
disks.  The  skin  is  dry,  and  ulcers  may  form  about  the  ears  or  on  the 
buttocks.  In  spite  of  the  severity  of  the  symptoms,  however,  the  child 
may  at  times  seem  for  an  hour  or  more  to  be  better,  may  be  conscious 
and  alert,  and  have  no  pain.  But  these  intervals  become  infrequent 
as  the  last  stage  approaches.  In  this  stage  lumbar  puncture  usually 
shows  the  presence  of  tubercle  bacilli  in  the  cerebro-spinal  fluid. 

III.  The  stage  of  paralysis  or  coma  comes  slowly.  The  child  gets 
worse,  the  stupor  deepens,  so  that  it  is  impossible  to  arouse  it,  and  para- 
lysis of  some  of  the  cranial  nerves  appears,  causing  strabismus,  facial 
paralysis,  or  difficult  swallowing,  and  general  paralysis  of  the  limbs,  or 
hemiplegia.  The  pupils  are  now  dilated  and  fail  to  act  to  light ; 
choked  disk  appears,  if  it  has  not  before ;  tubercles  may  be  seen  in  the 
choroid ;  the  opisthotonus  is  more  marked,  and  the  abdomen  is  re- 
tracted. The  temperature  begins  to  rise  in  the  morning  as  well  as  at 
night,  the  pulse  becomes  very  rapid  and  weak,  respiration  is  irregular 
and  labored  and  of  the  Cheyne-Stokes  character.  It  is  almost  impos- 
sible to  feed  the  child,  and  emaciation  is  rapid.  The  urine  is  retained 
or  is  passed  involuntarily.  The  coma  deepens,  and  the  child  dies  of 
exhaustion  or  in  a  convulsion.  The  duration  of  this  stage  is  from  two 
days  to  a  week,  depending  on  the  nutrition  and  inherent  strength  of 
the  child.  A  quarter  of  the  cases  die  in  the  first  week  after  the  second 
stage  begins,  a  quarter  in  the  second,  a  quarter  in  the  third,  and  the 
remainder  within  eight  weeks.  In  infants  whose  fontanelles  are  not 
completely  closed  a  condition  of  hydrocephalus  is  liable  to  develop  and 
to  go  on  increasing  unil  death.  There  is  no  doubt  that  almost  all  cases 
die,  and  we  have  no  means  of  arresting  the  progress  of  the  disease. 
Yet  cases  have  been  reported  in  which  recovery  has  occurred,  even 
when  tubercle  bacilli  have  been  found  in  the  cerebro-spinal  fluid. 
Treatment  is  palliative  only,  the  various  symptoms  being  treated  as  they 
arise.  It  has  been  proposed  to  open  the  skull  and  drain  the  base.  This 
has  been  done  once  with  apparent  success  by  Ord  and  Waterhouse,  but 
their  example  has  not  been  followed  by  others.  As  all  cases  are  hope- 
less, such  a  measure,  though  theoretically  of  little  value,  may  be  worth 
a  trial.  It  failed  in  one  case  under  my  care.  Injections  of  tuberculin 
have  failed  to  arrest  the  disease. 

The  following  case  is  a  good  illustration  of  the  course  of  the  disease : 
Girl,  aged  two  years,  of  healthy  parents,  began  to  have  slight  attacks 
of  sudden  loss  of  consciousness  with  convulsions,  on  November  7th, 
and  complained  of  headache,  nausea,  and  vomiting.  During  the  first 
two  weeks  these  symptoms  continued,  and  she  was  very  fretful,  dull, 
and  stupid,  emaciated  rapidly,  and  had  a  temperatuse  of  100°  F.,  at 
night.  During  the  third  week  the  temperature  reached  102°  F.,  at 
night  and  did  not  go  below  100°  F.,  in  the  morning;  her  pulse  was 
120,  regular,  respiration  regular;  she  had  much  headache,  retained 
little  food,  cried  out  in  sleep,  and  was  drowsy  and  dull.  On  Novem- 
ber 28th  she  had  a  convulsion  limited  to  the  right  side  of  the  face  and 


726  CEREBRAL  MENimiTW. 

right  arm  lasting  an  hour  and  a  half,  after  which  she  slept  for  two 
hours  and  had  a  temperature  of  104°  F.  There  was  no  paralysis  after 
the  convulsion,  but  she  failed  to  recognize  her  mother  and  nurse  and 
kept  her  hand  on  the  left  side  of  her  head,  with  signs  of  pain.  Her 
pupils  were  dilated,  reacted  sluggishly,  and  dilated  in  the  light,  being 
undulatory.  Her  optic  disks  were  normal.  During  the  following 
week  she  failed  steadily,  being  in  a  state  of  stupor,  and  finally  of  coma. 
Her  temperature  varied  from  101°  to  104  °F.,  pulse  became  140  and 
intermittent,  respiration  sighing,  irregular,  and  finally  Cheyne-Stokes. 
She  had  one  right-sided  convulsion.  Her  pupils  were  contracted  when 
she  was  let  alone,  but  dilated  widely  when  the  eyes  were  open  and  un- 
dulated. The  optic  disks  became  cloudy  and  finally  choked.  The 
face  was  flushed,  and  there  was  tdehe  Gi.r^brale.  The  abdomen  became 
retracted.  There  was  no  paralysis,  but  the  knee-jerks,  which  in  the 
first  weeks  were  absent,  became  exaggerated,  and  ankle  clonus  was 
obtained.  She  died  on  December  5th.  The  autopsy  showed  tubercles 
scattered  over  the  pia,  especially  on  the  base  and  in  the  fissures  of  Syl- 
vius. There  was  much  serum  under  the  pia.  The  ventricles  were 
greatly  distended  by  cloudy  serum.  There  was  some  oedema,  but  no 
inflammation  of  the  brain  and  no  apparent  cause  for  the  limitation  of 
the  convulsion  to  the  right  side,  though  there  was  more  serum  over  the 
left  hemisphere,  about  the  motor  zone,  than  elsewhere.  In  this  case  it 
was  evident  at  the  autopsy  that  no  operation  would  have  had  any  efiect 
to  relieve  the  condition. 

5.  Syphilitic  Meningitis.  —  The  symptoms  of  this  form  of  meningitis 
develop  more  slowly  than  those  of  the  other  forms,  and  are  more  easily 
and  successfully  treated.  They  resemble  the  symptoms  of  brain  tumor, 
but  are  more  rapid  in  their  progress.  The  patients  suifer  from  general 
sensations  of  discomfort  in  the  head,  from  headache,  which  is  often 
occipital,  sometimes  frontal,  sometimes  diffused  over  the  entire  head, 
and  is  usually  worse  toward  evening,  and  keeps  them  awake  in  the 
night.  They  become  nervous,  cannot  control  the  emotions,  and  can- 
not think  quickly  or  keenly ;  hence  they  are  unfitted  for  business. 
They  are  subject  to  attacks  of  vertigo,  and  may  at  times  vomit  unex- 
pectedly without  preceding  nausea.  After  a  time  dimness  of  vision 
may  be  noticed,  and  an  ophthalmoscopic  examination  shows  an  optic 
neuritis;  or  a  third  nerve  palsy  may  suddenly  occur,  with  ptosis,  ex- 
ternal strabismus,  and  immobility  of  the  eyeball  and  pupil ;  or  a  facial 
palsy  or  sixth  nerve  affection,  with  internal  strabismus  may  occur.  It 
is  more  rare  for  the  other  cranial  nerves  to  be  affected  although  intense 
neuralgia  of  the  face,  deafness,  and  difficulty  of  swallowing  have  been 
observed.  After  some  one  or  more  of  the  cranial  nerves  on  one  side 
have  become  paralyzed  a  hemiplegia  of  the  opposite  side  not  infre- 
quently develops,  not  complete,  but  attended  by  increase  of  reflexes. 
Sensory  disturbance  is  rare.  Polyuria  is  a  very  common  symptom  in 
these  cases.  Occasionally  a  cerebellar  gait  has  been  noticed.  In  one 
of  my  cases  general  convulsions  occurred  several  times  before  treat- 
ment began  to  produce  an  effect.     The  symptoms,  therefore,  are  quite 


TREATMENT.  727 

like  those  of  tumor  on  the  base  of  the  brain.  Sometimes  the  gummy 
exudation^'^is  in  the  Sylvian  fissure  and  causes  compression  of  the  blood- 
vessels supplying  the  cortex.  In  these  cases  sudden  attacks  of  aphasia 
or  hemiplegia  may  occur. 

The  meninges  of  the  convexity  are  occasionally  involved  in  a  syph- 
ilitic meningitis,  and  in  these  cases  symptoms  resembling  a  localized 
brain  tumor,  attacks  of  Jacksonian  epilepsy,  or  of  aphasia,  followed 
by  monoplegia,  may  occur.  The  headache  may  be  localized  at  one 
spot  and  may  be  attended  by  tenderness  on  percussion  of  the  head. 
Mental  symptoms  are  prominent  in  these  cases,  resembling  those  of 
paresis,  but  with  dementia  more  evident  than  delusions.  The  symj)- 
toms  may  be  very  irregular  and  intermittent,  as  in  the  basilar  type, 
severe  on  one  day,  slight  on  the  next ;  they  may  vary  in  their  location, 
and  they  are  rarely  as  permanent  and  continuous  as  in  brain  tumor. 
The  occurrence  of  an  optic  neuritis  is  the  exception  in  menigitis  of  the 
convexity.  The  difficulty  of  diagnosis  between  a  specific  meningitis 
of  the  convexity,  paresis,  and  brain  tumor  is  so  great  that  in  every 
case  where  the  question  of  diagnosis  arises  it  is  well  to  try  antisyphili- 
tic  treatment.  If  this  fails  operative  treatment  may  be  considered,  for 
successful  removal  of  gummy  tumors  has  been  recorded. 

The  various  symptoms  named  develop  gradually  in  the  course  of  a 
month  or  six  weeks,  increase  in  intensity,  and  prostrate  the  patient 
completely.     But  when  treatment  is  begun  they  soon  begin  to  subside,  ^ 
and  finally  may  entirely  pass  away,  leaving  the  patient  quite  well. 

In  cases  of  long  duration,  where  the  nerves  or  the  brain  have  been 
seriously  compressed,  and  where  degeneration  in  the  nerve  trunks  or 
in  the  brain  tracts  has  been  produced,  the  recovery  is  much  delayed  or 
may  be  incomplete.  Thus  in  one  of  my  cases  which  showed  paralysis 
of  the  sixth,  seventh,  and  eighth  nerves  .on  the  left  side,  and  a  marked 
cerebellar  gait,  with  staggering  toward  the  left,  the  cranial  nerve  palsy 
recovered,  the  gait  became  natural,  but  there  has  remained  at  the  end 
of  three  years  a  sense  of  uncertainty  of  position  and  some  vertigo  on 
rapid  walking  and  an  exaggerated  knee-jerk  on  the  right  side.  In 
another  case,  after  a  period  of  occipital  pain,  worse  at  night,  the  right 
sixth  and  seventh,  then  the  fifth,  then  the  third  nerves  were  paralyzed, 
and  then  a  left  hemiplegia  appeared.  These  symptoms  subsided  under 
treatment,  but  after  a  few  months  deafness  in  the  right  ear  and  a 
marked  staggering  to  the  right  ride,  with  polyuria,  developed.  These 
also  subsided  under  treatment,  and  for  ten  years  this  man  has  been  well 
and  able  to  work,  but  suffers  from  vertigo. 

Prognosis.  —  The  prognosis  is  fairly  good  in  syphilitic  meningitis, 
as  treatment  promptly  causes  the  absorption  of  the  exudation  ;  but  it 
must  not  be  forgotten  that  symptoms  due  to  an  actual  destruction  of 
cerebral  tissue  are  permanent. 

Treatment.  —  Treatment,  no  matter  at  what  stage  in  the  course  of 
syphilis,  should  l)e  by  means  of  inunctions  of  mercury.  One  drachm 
of  })lue  ointment  is  to  be  well  rubbed  into  the  body  daily,  and  the 
absorption  is  increased  by  a  daily  bath  in  water  of  a  temperature  of  100° 


728  CRBEBBAL  MENINGITIS. 

to  108°  F.  for  half  an  hour.  This  bath  may  be  followed  oy  cool 
sponging  to  prevent  too  great  sweating  and  taking  cold.  The  applica- 
tion of  mercury  should  be  to  a  different  part  of  the  body  each  day,  and 
this  part  should  be  bandaged,  so  that  any  ointment  left  on  the  skin  may 
be  absorbed  and  not  rubbed  off  by  the  clothing.  I  prefer  this  method 
to  that  by  hypodermic  injection  of  corrosive  sulDlimate,  though  this  may 
be  resorted  to  in  some  cases.  Pills  of  mercuric  iodide,  red  or  green,  usu- 
ally derange  the  stomach  and  their  effect  is  less  rapid  than  that  of  the 
inunctions.  After  the  mercury  has  been  given  for  one  week  iodide  of 
potassium  is  to  be  begun  in  addition  to  the  mercury.  It  is  my  rule  to 
begin  with  a  dose  of  twenty-five  grains  three  times  a  day,  as  thus  the 
coryza  which  is  caused  by  small  doses  is  avoided.  The  amount  is  then 
increased  rapidly,  one  grain  at  each  dose  until  three  hundred  grains 
daily  are  being  taken.  On  reaching  a  dose  of  fifty  grains  it  is  better 
to  increase  the  number  of  doses  than  to  increase  the  size  of  each  dose. 
If  the  iodide  is  given  well  diluted  in  water,  in  some  gaseous  mineral 
water  or  in  milk,  it  can  usually  be  taken  for  several  months.  The 
mercury  should  be  continued  with  the  iodide  until  marked  improvement 
is  manifest  or  until  salivation  is  caused.  This  should,  of  course,  be  pre- 
vented by  the  use  of  a  mouth  wash  of  chlorate  of  potash,  and  by  clean- 
ing the  teeth  after  each  meal  and  after  each  dose  of  medicine.  Usually 
three  ounces  of  blue  ointment  are  sufficient  but  more  may  be  needed. 
The  general  strength  of  the  patient  should  always  be  kept  up  to  the 
best  point ;  every  possible  means  being  used  to  promote  health.  An 
out-of-door  life  in  good  country  air,  moderate  exercise,  the  best  food 
possible,  and  a  generous  diet,  with  moderate  use  of  stimulants  and 
tonics  of  all  sorts  are  to  be  advised.  The  benefit  of  a  course  of  treat- 
ment at  hot  springs  or  in  a  sanitarium  is  partly  due  to  the  more  health- 
ful surroundings  than  city  life  affords. 


PART  III. 
FUNCTIONAL  DISEASES. 


CHAPTER   XLII. 

THE  PAINFUL  NEUEOSES. 
Neuralgia.     Meralgia.     Herpes  Zoster.     Eeferred  Pains. 

Neuralgia  is  a  disease  of  a  sensory  nerve  characterized  by  pain  in 
the  course  of  the  nerve  or  in  its  peripheral  distribution. 

Meralgia  diifers  from  neuralgia  in  that  it  occurs  in  the  legs  and  only 
on  standing  and  walking,  but  never  at  rest.  This  form  is  often  at- 
tended by  paraesthesia  in  the  distribution  of  the  nerve  and  by  slight 
anaesthesia. 

Herpes  zoster  is  a  trophic  disturbance  of  the  skin  situated  always  in 
the  domain  of  a  sensory  nerve  —  and  often  attended  by  neuralgia. 

Referred  pains  are  painful  sensations  felt  in  some  region  of  the  body, 
but  actually  due  to  some  disease  of  an  internal  organ,  whose  central 
nervous  mechanism  is  located  in  the  part  of  the  nervous  system  with 
which  the  region  in  which  the  pain  is  felt  is  connected. 

NEURALGIA. 

Etiology,  —  Neuralgia  may  be  due  to  toxic  agents  of  external  or  of 
internal  origin  ;  organic  or  inorganic  ;  causing  an  irritation  of  the  neu- 
rones, or  of  their  axones.  It  may  be  caused  by  congestion,  without  or 
with  a  serous  exudation  in  the  sheath  of  the  nerve,  and  consequent 
compression  and  irritation  of  the  nerve  fibres.  It  is  often  due  to  dis- 
ease in  the  bloodvessels  accompanying  the  nerve,  which  prevents  those 
processes  of  osmosis  necessary  to  proper  nutrition.  It  is  also  the  first 
symptom  of  organic  disease  in  the  nerve  fibres,  or  in  the  endoneurium, 
of  a  primary  inflammatory  character,  since  many  cases  of  neuritis  are 
preceded  by  attacks  of  neuralgia.  It  not  uncommonly  develops  in 
nerves  which  pass  through  or  near  organs  which  are  the  seat  of  dis- 
ease. And  it  is  a  frequent  manifestation  of  certain  disorders  of  nutri- 
tion, indigestion,  stomach  or  intestinal  fermentation,  rheumatism,  gout, 
diabetes,  anaemia,  chlorosis,  as  well  as  of  the  various  infectious  diseases, 
especially  malaria  and  syphilis. 

The  majority  of  authorities,  however,  do  not  regard  neuralgia  as 
necessarily  a  peripheral  disease,  althougli  its  manifestations  are  periph- 
eral. The  sensory  nerves  send  their  impulses  inward  to  the  gray  mat- 
ter of  the  spinal  cord  or  brain  axis,  where  such  impulses  are  primarily 
received  in   a  peculiar  substance  —  the  gelatinous  substance  of  Ro- 

729 


7S0  neuhalgta. 

lando  —  which  appears  to  be  a  fiue  felt-like  structure  containing  many 
minute  cells  or  nuclei.  Thence  these  impulses  are  sent  up  by  the  cen- 
tral sensory  tracts  to  the  cerebral  cortex,  where  they  are  consciously 
perceived.  An  irritation  in  the  gelatinous  substance  or  in  the  central 
tracts  is  capable  of  producing  pain  referred  to  the  periphery,  as  many 
cases  of  organic  disease  in  these  parts  prove.  Hence  it  has  been 
thought  that  some  disturbance  in  nutrition  of  the  central  sensory  re- 
gion is  the  usual  cause  of  neuralgia,  and  this  theory  receives  support, 
as  Gowers  has  held,  from  the  facts  that  pain  may  be  felt  in  adja- 
cent parts  of  several  nerve  regions,  may  radiate  into  adjacent  nerve 
regions,  or  may  be  felt  in  a  region  not  that  of  the  irritated  nerve. 

Neuralgia  may  be  hereditary.  It  rarely  develops  during  childhood, 
but  from  youth  up  to  old  age  no  one  is  exempt.  Women  appear  to 
suffer  more  than  men.  It  occurs  more  frequently  in  nervous  and  hys- 
terical individuals,  and  emotional  excitement  is  said  to  be  a  cause.  It 
is  particularly  frequent  in  sensitive  persons  in  a  climate  where  damp- 
ness is  common.  Attacks  bear  a  distinct  relation  to  atmospheric 
changes ;  a  low  barometer,  a  high  degree  of  humidity,  and  a  falling 
temperature  combined  are  often  exciting  causes. 

Certain  electric  states  of  the  atmosphere,  of  which  as  yet  we  have 
no  precise  knowledge,  and  no  means  of  recording,  are  capable  of  caus- 
ing attacks ;  and  persons  who  are  subject  to  neuralgia  are  conscious  of 
sensations  which  coincide  with  changes  in  electrical  tension.  Thus 
many  persons  are  warned  by  a  neuralgic  attack  of  the  coming  of  a 
thunder  shower,  or  of  a  severe  storm,  some  hours  before  it  arrives. 
Attacks  occur  with  much  greater  frequency  in  winter  than  in  summer. 
Exposure  to  cold  is  the  most  common  cause  of  an  attack,  and  when  a 
nerve  is  painful  a  draught  of  air  often  causes  a  sudden  paroxysm, 
while  heat  almost  uniformly  relieves.  Patients  are  usually  hypersen- 
sitive to  cold  in  the  painful  area  and  instinctively  protect  it  when 
exposed. 

Symptoms.  —  The  symptom  of  neuralgia  is  pain.  This  is  sharp, 
sudden,  shooting  along  the  nerve,  and  transient,  being  succeeded  by  an 
interval  of  freedom.  The  pain  is  often  accompanied  by  numbness, 
tingling,  or  burning  in  the  surface  to  which  the  sensory  nerve  goes ; 
and  after  a  time  it  is  followed  by  a  tender  sensitiveness  of  this  part 
and  also  of  the  entire  nerve  trunk.  Pressure  on  the  nerve  elicits  un- 
usual tenderness  and  starts  an  attack  of  pain.  Sometimes  the  pain  is 
so  severe  as  to  cause  reflex  or  automatic  spasms  in  muscles  whose  motor 
centres  are  in  close  relation  to  the  sensory  root  involved.  Thus  tri- 
geminal neuralgia  often  causes  a  twitching  of  the  eyes  and  face  ;  inter- 
costal neuralgia,  a  bending  of  the  entire  trunk  ;  sciatica,  a  drawing  up 
of  the  leg.  Any  movement,  especially  if  sudden,  is  liable  to  start  a 
paroxysm  of  pain,  hence  patients  instinctively  keep  quiet,  move  cau- 
tiously, and  avoid  effort.  Sometimes  vasomotor  and  trophic  distur- 
bances attend  or  follow  a  neuralgic  attack.  Such  are  flushing  of  the 
surface,  unusual  sweating,  or  herpetic  eruptions  on  the  skin,  or  falling 
of  the  hair.     A  distinct,  unusual  pulsation  in  the  vessels  of  the  pain- 


Pathology.  731 

ful  region  or  nerve  can  often  be  felt.  A  slight  swelling  of  the  pain- 
ful region  may  also  occur. 

Herpes  zoster  is  a  frequent  accompaniment  of  neuralgia  in  any  nerve. 
It  sometimes  occurs  without  neuralgia  but  is  always  limited  in  its  ex- 
tent to  the  domain  of  one  or  more  nerves,  as  Head  has  demonstrated.^ 
It  is  particularly  frequent  in  connection  with  neuralgia  of  the  thoracic 
region.  It  may  precede  or  it  may  follow  an  attack  of  neuralgia,  or  it 
may  occur  as  the  only  symptom  of  the  affection  of  the  ganglia. 

The  course  of  tlie  disease  is  very  variable.  The  pain  occurs  In  par- 
oxysms and  these  vary  in  severity  and  duration.  The  pain  may  come 
every  few  seconds,  lasting  several  seconds,  for  many  hours.  Such  an 
attack  may  recur  regularly  every  day  at  a  definite  hour.  It  often 
subsides  at  night,  though  this  is  not  uniformly  the  case,  and  in  some 
patients  the  pain  is  worse  at  night.  Attacks  rarely  continue  for  more 
than  three  or  four  days  when  they  are  succeeded  by  a  free  interval  of 
several  days,  weeks,  or  even  months.  Some  women  suffer  from  neu- 
ralgia with  each  menstrual  period. 

The  tendency  to  a  recurrence  after  one  attack  is  well  known,  and 
almost  everyone  is  conscious  of  some  nerve  in  his  body  of  little  resis- 
tance, liable  to  give  him  pain  when  he  is  ill.  A  person  who  has  such 
a  tender  nerve  rarely  suffers  from  neuralgia  in  other  nerves,  and  neu- 
ralgia is  rarely  bilateral.  Occasionally  it  develops  in  different  nerves 
at  different  times.  If  there  is  discovered  a  cause  which  can  be  re- 
moved, which  is  rarely  the  case,  treatment  of  a  rational  nature  can  be 
instituted.  But  in  the  majority  of  cases  palliative  remedies  have  to  be 
employed.  When  herpes  occurs,  with  or  without  neuralgia,  the  dura- 
tion of  the  attack  is  longer.  The  vesicles  remain  for  at  least  two 
weeks,  often  occur  in  successive  crops  and  sometimes  persist  for  two 
months.     They  may  leave  permanent  scars. 

Pathology.  —  As  fatal  cases  are  rare  no  autopsies  are  on  record. 
But  surgeons  have  cut  out  both  nerves  and  ganglia  for  the  relief  of 
neuralgia,  and  from  the  examination  of  these  much  information  has 
been  obtained  concerning  the  pathology  of  the  disease.  All  the  various 
processes  of  neuritis,  with  degeneration,  and  atrophy  in  the  nerve 
fibres  have  been  seen.  But  the  more  important  lesions  appear  to  be 
in  the  neurone  bodies  which  lie  in  the  ganglia.  These  neurone  bodies 
have  been  found  in  different  stages  of  degeneration,  with  chromatolysis, 
vacuolization,  and  atrophy.  Some  neurones  stain  only  faintly  and  are 
most  irregular  in  outline,  being  so  shrunken  as  to  leave  the  space  in 
which  they  lie  almost  empty.  Some  neurones  are  very  small,  without 
nucleus  or  necleolus.  The  fibres  within  the  ganglion  are  often  swollen, 
the  medullary  sheath  is  disintegrated,  the  axone  is  granular.  In  other 
fibres  the  sheath  is  full  of  a  disintegrated  mass  in  which  the  axone 
cannot  be  distinguished.  In  still  others  the  sheath  is  empty.  These 
changes  are  shown  in  Plate  III.  The  connective  tissue  about  the 
neurone  bodies  and  also  about  the  fibres  has  been  found  to  be  increased 
in  density.     The  small   bloodvessels  in  the  ganglion  are  sometimes 

1  Brain,  1895. 


732  NEUBALQTA. 

closed  by  the  proliferating  tissue  in  their  walls,  and  all  forms  of 
atheroma  and  sclerosis  have  been  seen  in  the  larger  vessels.  These 
changes  are  shown  in  Plate  XXVIII.,  taken  from  Keen  and  Spiller.' 
Other  authors  have  found  marked  thickening  of  the  walls  of  the 
bloodvessels  which  lie  in  the  endoneurium  of  the  affected  nerves,  and 
this  thickening  may  be  present  both  in  the  media  and  intima.  In 
many  cases  true  atheromatous  degeneration  of  the  smaller  arteries  has 
been  found.  Similar  changes  have  been  found  by  Thomas  in  the 
ganglia  on  the  posterior  nerve  roots  in  the  early  stage  of  tabes  (see 
Chapter  XIX.),  and  that  disease  begins  with  neuralgic  pains.  In 
cases  of  neuralgia  attended  by  herpes,  hemorrhages  have  been  found 
in  the  ganglia.  It  is,  therefore,  probable  that  neuralgia  has,  in  every 
chronic  case,  some  organic  lesion. 

While  these  changes  are  characteristic  of  chronic  and  long-standing 
cases  of  neuralgia,  especially  in  cases  of  trigeminal  neuralgia,  it  is  not 
to  be  thought  that  they  are  present  in  the  acute  and  transient  cases. 
They  are,  however,  indications  of  a  long-continued  malnutrition,  and 
hence,  we  may  conclude  that  in  the  acute  cases  some  temporary  malnu- 
trition of  the  sensory  neurones  is  present. 

Treatment.  —  The  causal  treatment  of  neuralgia  consists  in  elimi- 
nating all  factors  capable  of  producing  the  disease.  These  have  been 
mentioned  in  discussing  the  etiology.  Everything  which  tends  to 
build  up  the  strength  and  health  of  the  patient ;  a  life  with  interests 
but  without  anxiety ;  a  healthful  regimen  with  proper  diet  and  exer- 
cise ;  daily  baths,  either  cold  or  hot,  followed  by  cold  sponging ;  regu- 
lation of  the  digestive  functions  and  of  the  bowels ;  washing  the  kid- 
neys out  by  drinking  an  excess  of  water  ;  and  adding  to  the  diet  those 
tonic  medicines  which  occasionally  strengthen  the  nervous  system,  such 
as  cod-liver  oil,  quinine,  strychnine,  iron,  arsenic,  and  the  glycero- 
phosphates will  eventually  help  to  cure  neuralgia.  When  any  sus- 
picion of  infection  arises,  especially  when  malaria  is  detected,  a  mer- 
curial purge  followed  by  quinine  in  large  doses  (20  grains),  or  by 
Warburg's  tincture  (5ij)  (which  I  consider  superior  to  quinine  in 
many  types  of  neuralgia),  will  give  relief.  The  quinine  or  Warburg's 
extract  should  be  given  in  large  doses  four,  and  again  three,  hours  be- 
fore the  attack  of  neuralgia  is  due.  In  the  malarial  type  a  distinct 
periodicity  in  the  attack  is  so  common  that  this  time  can  easily  be 
reckoned.  A  small  dose  of  the  drug  may  be  used  between  the  attacks 
at  intervals  of  six  hours.  When  syphilis  is  suspected  as  a  cause  (and 
in  such  cases  the  attack  usually  occurs  in  the  afternoon),  mercurials, 
combined  with  large  doses  of  iodide  of  potash,  are  indicated. 

In  many  cases  of  obstinate  neuralgia  climatic  conditions  must  be 
considered,  and  very  often  a  change  of  air  will  be  of  great  benefit. 
A  sojourn  at  the  seashore,  or  in  the  mountains,  according  to  the  ex- 
perience of  the  patient  as  to  the  benefit  previously  derived  from  travel, 
will  often  appear  to  start  a  cure.     The  regime  of  a  sanitarium,  or 

^Keen  and  Spiller.  "On  Resection  of  the  Gasserian  Ganglion,"  American  Journal 
of  the  Medical  Sciences,  November,  1898. 


DESCRIPTION  OF  PLATE  XXIX. 


Fig.  1. — Portion  of  the  Gasserian  ganglion  at  the  entrance  of  the  third  branch  of 
the  trifacial  nerve.  The  medullary  sheaths  are  most  irregularly  swollen,  and  at  the 
right  of  the  field  empty  nerve  sheaths  are  seen  (method  of  Azoulay). 

Fig.  2. — Portion  of  the  second  branch  of  the  trigeminal  nerve  near  the  Gasserian 
ganglion.  The  axis  cylinders  have  entirely  disappeared,  and  the  medullary  sheaths  are 
greatly  swollen.  In  many  places  the  medullary  substance  of  two  or  more  nerve  fibres 
has  united  into  irregularly  shaped  masses  (osmic  acid  stain). 

Fig.  3. — One  of  the  nerve  bundles  within  the  Gasserian  ganglion.  Numerous 
swollen  and  irregularly  formed  axis  cylinders  may  be  seen.  In  most  portions  of  the 
field  these  appear  as  drops  of  a  red,  hyaline-like  substance,  but  in  one  portion  an  axis 
cylinder  of  considerable  length  may  be  seen. 

Fig.  4. — Bloodvessels  from  the  Gasserian  ganglion.  The  walls  are  greatly  thickened, 
and  the  lumen  of  the  large  vessel  has  been  almost  entirely  obhterated.  In  one  place 
the  innermost  layers  of  the  vessel  have  contracted  from  the  outer  during  the  process 
of  hardening.      Smaller  vessels  in  the  upper  part  of  the  field  are  entirely  closed. 

Fig.  5.^A  nerve  bundle  of  the  trigeminus  close  to  the  Gasserian  ganglion.  Only  a 
few  nerve  fibres  are  present,  and  everywhere  an  abundance  of  connective  tissue  is  seen. 
Three  much  swollen  medullary  sheaths  are  in  the  field. 


PLATE   XXIX 


Fig^   1. 


Fig.  2. 


.i4v.   „>-- 


Fig.  3. 


Fig.  4. 


Fig.  5. 


^    # 


TRIGEMINAL  NEURALGIA.  733 

water-cure,  as  well  as  the  change  of  air,  change  of  habits  and  sur- 
roundings, '^nd  freedom  from  work  or  home  care,  are  of  great  benefit 
to  many  patients.  And  if  this  can  be  combined  with  foreign  travel  by 
sending  patients  to  some  health  resort  abroad,  much  relief  will  be 
obtained.  The  dry  climate  of  the  Nile,  the  rarefied  air  of  the  Enga- 
dine,  or  high  Swiss  valleys,  or  Colorado,  are  very  beneficial. 

The  treatment  of  herpes  zoster  consists  in  making  applications  of 
zinc  ointment  in  the  early  stage  and  protecting  the  surface  by  linen 
bandages.  Collodion  may  be  applied  in  the  later  stage  when  there  is 
no  longer  any  liquid  secretion  in  the  vesicles.  If  the  vesicles  fill  with 
pus  they  should  be  punctured  and  washed  with  a  solution  of  corrosive 
sublimate  1  to  5,000. 

The  palliative  treatment  of  neuralgia  will  be  considered  in  discussing 
the  special  forms  of  the  disease. 

SPECIAL  FORMS  OF  NEURALGIA. 

Trigeminal  Neuralgia.  —  Neuralgia  of  the  fifth  nerve.  Tic  dou- 
loureux.    This  is  the  most  common  form  of  neuralgia. 

Etiology.  —  Women  are  more  subject  to  trigeminal  neuralgia  than 
men.  It  is  a  disease  which  is  very  uncommon  under  the  age  of  twenty 
years,  the  majority  of  cases  occurring  between  the  ages  of  twenty-five 
and  fifty  years,  but  no  age  is  exempt,  and  as  the  disease  is  a  chronic 
one,  the  patients  may  suffer  from  it  until  the  close  of  a  long  life.  I 
have  seen  it  in  patients  over  eighty  years  of  age.  The  disease  is  dis- 
tinctly an  hereditary  one,  many  patients  admitting  that  their  parents 
or  grandparents  have  suffered  from  it.  It  develops  more  commonly 
in  winter  than  in  the  summer,  which  gives  support  to  the  theory  that 
the  majority  of  cases  are  due  to  exposure  to  cold.  Neurasthenic  indi- 
viduals and  those  who  suffer  from  anaemia,  chlorosis,  and  gout,  or 
diabetes,  are  more  subject  to  the  disease  than  others.  Head^  has 
denied  this  very  strongly,  but  my  observations  do  not  support  his 
statements. 

Slight  attacks  of  trigeminal  neuralgia  may  occur  in  the  course  of 
any  slight  illness,  such  as  a  disorder  of  the  stomach,  an  acute  intestinal 
infection,  chronic  constipation,  congestion  of  the  kidneys,  a  bad  coryza, 
or  angina,  or  an  attack  of  grippe.  In  many  gouty  or  rheumatic  indi- 
viduals it  is  a  frequently  recurring  symptom  of  the  general  disorder. 
Some  persons  feel  a  sudden  neuralgic  pain  in  the  trigeminal  nerve 
while  eating  ice-cream  or  drinking  very  cold  drinks.  Attacks  of  neu- 
ralgia are  not  infrequent  in  connection  with  migraine.  In  many  per- 
sons who  are  affected  with  astigmatism,  or  defective  accommodation, 
neuralgia  of  the  trigeminal  nerve  may  follow  any  strain  of  the  eyes. 
Ear  disease  is  also  said  to  have  caused  it  (Moos).  Such  attacks  are 
extremely  temporary  and  need  no  special  treatment  further  than  the 
treatment  of  the  causal  condition,  of  which  they  are  the  effect.  But 
trigeminal  neuralgia  may  become  a   more  serious  and   troublesome 

^  Allbutt's  System  of  Medicine,  vol.  vi.,  p.  732. 


734  NEURALGIA. 

disease,  and  such  cases  require  careful  study.  In  chronic  lead  poison- 
ing it  appears  to  be  a  not  infrequent  symptom. 

Many  cases  can  be  traced  to  an  attack  of  some  one  of  the  infectious 
diseases,  of  which  malaria,  grippe,  and  typhoid  are  the  most  common. 
In  this  country  when  a  patient  has  once  had  malaria,  it  is  not 
uncommon  for  subsequent  attacks  of  this  disease  to  manifest  them- 
selves exclusively  by  attacks  of  trigeminal  neuralgia.  Usually  such 
attacks  are  daily  in  occurrence,  at  a  definite  time  of  the  day,  and 
last  for  a  few  hours  and  disappear  as  suddenly  as  they  came  on. 
Organic  disease  of  any  kind  upon  the  base  of  the  brain,  or  in  the 
course  of  the  trigeminal  nerve,  is  capable  of  producing  trigeminal 
neuritis,  with  a  consequent  neuralgia.  The  syphilitic  affections  of  the 
base  of  the  brain,  also  tuberculous  affections,  tumors  of  the  brain,  or 
fractures  of  the  base  of  the  skull,  and  aneurisms  of  the  internal  carotid 
artery,  or  of  the  circle  of  Willis,  bullet  wounds  of  the  base  of  the 
skull,  any  disease  of  the  bones  of  the  head  and  face  and  catarrhal 
affections  of  the  nose  and  throat,  dental  caries,  and  any  functional  dis- 
turbance of  the  eye  or  ear,  are  all  capable  of  setting  up  a  neuritis  in 
contiguous  nerves  and  producing  either  local  or  trigeminal  neuralgia. 

Symptoms.  —  The  chief  symptom  of  trigeminal  neuralgia  is  intense 
pain  in  the  course  of  the  nerve  and  upon  the  face,  usually  deep,  some- 
times on  the  surface.  This  pain  occurs  in  sharp  paroxysms  which  are 
repeated  frequently,  a  series  of  sharp,  shooting  pains  being  followed 
by  an  interval  of  relief.  Pain  is  so  intense  as  to  be  agonizing.  It  is 
frequently  a  burning  pain.  It  makes  the  patient  cringe,  it  causes  a 
flow  of  tears,  and  in  very  many  cases  a  sharp,  quick  contraction  of  the 
muscles  of  the  face,  a  closing  of  the  eye  and  drawing  up  of  the  mouth. 
The  patient  may  even  turn  the  head  downward  with  pain.  Changes 
in  the  condition  of  the  atmosphere,  an  increase  of  the  humidity  or 
variations  of  barometric  pressure  or  of  temperature  may  excite  an 
attack  of  pain.  Some  patients  have  annual  attacks  during  the  winter 
months  and  are  free  during  warm  weather.  Any  draught  upon  the 
face  or  exposure  to  cold,  any  movement  of  the  muscles  of  the  face,  the 
acts  of  whistling,  of  talking,  of  chewing,  of  swallowing,  are  capable 
of  arousing  the  pain,  and  the  patient  will  very  often  starve  rather  than 
incur  the  pain  of  eating.  Mental  anxiety  or  intellectual  efforts  seem 
capable  of  starting  up  an  attack.  Pain  shoots  out  into  the  entire  dis- 
tribution of  the  trunk  of  the  nerve  which  is  affected.  In  some  cases 
only  one  trunk  is  involved,  in  other  cases  two  trunks,  in  most  severe 
cases  all  three  trunks.  The  most  common  form  of  neuralgia  is  the 
supraorbital,  in  which  the  pain  is  felt  above  the  eye  and  at  the  notch 
or  foramen  through  which  the  supraorbital  nerve  makes  its  exit  upon 
the  forehead,  and  over  the  forehead  and  in  the  hair  as  high  as  the  ver- 
tex. Sometimes  the  pain  radiates  into  the  eyeball,  and  occasionally 
pain  in  the  eyeball  is  the  only  manifestation  of  neuralgia  of  the  supra- 
orbital nerve.  When  the  infraorbital  branch  is  the  one  which  is 
affected,  pain  is  felt  upon  the  cheek  and  in  the  upper  teeth,  and  espe- 
cially in  the  antrum  and  malar  bone.     It  may  then  go  as  far  out  as 


TRIGEMINAL  NEURALGIA,  735 

the  temple  and  the  lobe  of  the  ear.  When  the  third  or  lower  branch 
of  the  nerv^  is  affected,  the  pain  is  felt  upon  the  cheek  and  lip  and  in 
the  lower  teeth,  also  within  the  mouth,  even  in  the  tongue.  I  have  seen 
cases  in  which  the  neuralgia  was  limited  to  the  tongue,  pain  being  felt 
chiefly  in  the  side  and  lower  surface  of  the  organ,  being  much  inten- 
sified by  the  act  of  swallowing,  or  talking,  or  chewing.  The  pain 
usually  extends  in  chronic  cases  from  the  branch  of  the  nerve  first 
affected  into  the  other  branches,  so  that  eventually  the  entire  face  is 
the  seat  of  pain.  In  one  patient  a  touch  along  the  lower  part  of  the 
cheek  on  the  right  side  caused  intense  pain  in  the  right  half  of  the 
tongue,  and  there  was  also  a  tender  spot  over  the  ramus  of  the  jaw 
just  in  front  of  the  ear,  but  no  anaesthesia  or  loss  of  taste.  A  very 
hypersensitive  condition  of  the  skin  in  the  domain  of  the  branch 
affected  is  quite  frequent.  There  is  a  constant  sense  of  tingling,  and 
an  unusual  appreciation  of  little  variations  of  temperature ;  and  a  slight 
touch  with  the  fingers,  or  with  cotton-wool  is  disagreeable  and  painful. 
For  this  reason  patients  very  often  instinctively  protect  the  affected 
part  by  applications  of  woollen  cloths  in  order  to  keep  the  part  at  an 
even  temperature  and  to  avoid  sudden  and  unexpected  contacts.  The 
face  does  not  often  show  any  change  in  color,  but  occasionally  it  is  a 
little  red  and  the  conjunctiva  and  mucous  membrane  of  the  mouth  and 
nose  may  be  congested.  Sometimes  an  increased  pulsation  of  the 
bloodvessels  accompanies  the  attack  of  neuralgia  and  the  arteries  may 
be  felt  to  be  fuller  than  usual.  It  is  to  be  recollected  that  the  most 
common  lesion  found  in  neuralgia  is  an  arterial  sclerosis,  which  may 
account  for  the  thickening  of  the  arteries  that  is  felt.  Sometimes  the 
attack  is  accompanied  by  a  secretion  of  tears  or  of  saliva.  Trophic 
changes  in  the  skin  of  the  face  have  occasionally  been  observed,  the 
most  frequent  of  which  is  the  appearance  of  herpes  zoster.  Some 
authors  have  spoken  of  an  unusual  grayness  of  hair  and  of  falling  of 
the  beard,  but  this  I  have  never  seen. 

Although  the  fifth  nerve  conveys  the  sensation  of  taste,  this  is  not 
affected  in  any  way  in  trigeminal  neuralgia  —  a  fact  that  proves  that 
the  taste  fibres  are  independent  of  those  of  the  fifth  nerve  proper. 

The  nerve  trunks  are  extremely  tender  during  an  attack  of  neuralgia 
at  their  exits  upon  the  face,  namely,  over  the  eye,  upon  the  malar 
bone,  and  upon  the  chin.  Any  pressure  at  these  points  produces  very 
great  increase  of  the  pain  during  an  attack.  It  must  be  remembered, 
however,  that  these  points  are  normally  sensitive  to  pressure.  In 
Plate  IV  the  distribution  of  the  various  branches  of  the  fifth  nerve  in 
the  face  is  shown. 

Course.  —  The  course  of  the  disease  varies  very  much  in  different 
cases.  Usually  a  neuralgia  begins  with  a  sudden  attack,  which  lasts 
for  several  days  and  then  passes  off  under  the  use  of  remedies.  Such 
an  attack  may  recur  from  time  to  time,  and  in  the  severer  type  the 
attacks  become  more  and  more  frequent,  so  that  the  patients  who  have 
had  l>ut  one  attack  in  one  or  two  years,  after  a  few  years  may  be  hav- 
ing an  attack  every  week.     In  the  most  severe  type  of  case  the  pain 


736  NEURALGIA. 

comes  on  every  few  minutes,  day  and  night,  lasting  at  times  for  several 
weeks  in  this  manner,  and  is  quieted  only  by  the  strongest  opiates,  to 
recur  as  soon  as  the  effect  of  the  remedy  has  worn  oflP.  It  is  in  the 
severer  type  of  case  that  surgical  interference  is  willingly  permitted. 
In  any  case  of  facial  neuralgia  one  attack  predisposes  to  another,  and 
patients  who  have  once  had  an  attack  appear  to  be  subject  to  the  dis- 
ease for  the  rest  of  their  lives.  The  intervals,  however,  may  be  so 
long  (several  years)  and  the  attacks  may  yield  so  readily  to  a  sympto- 
matic or  causal  treatment  as  to  preclude  any  idea  of  the  division  of  the 
nerve.  I  have  several  patients  who  never  go  through  the  winter 
without  a  severe  attack.  One  such  patient,  who  had  been  a  sufferer 
for  many  years,  escaped  while  spending  the  winter  on  the  Nile.  In 
many  cases  a  warm  winter  climate  is  advisable.  The  general  dele- 
terious effect  of  severe  pain  upon  the  constitution  and  nutrition  of 
the  patient  is  often  demonstrated  in  cases  of  trigeminal  neuralgia.  The 
sleep  is  poor,  the  assimilation  of  food  is  impaired,  even  where  the 
patients  are  not  prevented  by  pain  from  eating ;  disorders  of  digestion, 
especially  constij)ation,  are  common,  and  a  state  of  mental  disquiet, 
apprehension,  and  hopelessness  may  develop ;  in  fact,  some  cases  have 
gone  on  to  melancholia  and  suicide. 

Diagnosis.  —  The  diagnosis  of  trigeminal  neuralgia  is  very  simple; 
the  only  question  that  can  arise  is  whether  the  neuralgia  is  based  upon 
an  actual  neuritis,  or  is  a  purely  functional  disease.  The  existence  of 
continuous  pain,  the  paroxysms  alternating  with  remissions  rather  than 
intermissions,  continuous  tenderness  in  the  course  of  the  nerves,  the 
development  of  anaesthesia  in  the  skin  of  the  face,  and  the  determina- 
tion of  some  organic  disease  as  a  cause,  such  as  syphilis,  tumors,  etc., 
mentioned  in  the  section  of  etiology,  make  the  diagnosis  of  neuritis 
rather  than  neuralgia  probable.  The  facts  which  are  presented  in  the 
chapter  upon  referred  pain  should  not  be  overlooked  in  making  the 
diagnosis,  and  neuralgia  should  never  be  confounded  with  migraine. 
The  pain  in  migraine  is  felt  in  the  temple  and  side  of  the  head,  and  is 
not  located  in  the  course  of  the  branches  of  the  fifth  nerve. 

Treatment.  —  The  treatment  must  first  be  causal;  if  an  organic 
affection  can  be  ascertained  as  a  basis,  it  must  be  treated  and  removed. 
In  malarial  cases  and  in  any  case  that  is  of  uncertain  cause,  quinine 
should  be  tried.  It  is  well  to  precede  this  by  a  mercurial  purge — 
calomel  yL  grain  every  hour  until  1  grain  is  taken — and  to  give  the 
quinine  in  a  dose  of  20  to  30  grains,  which  should  be  administered 
within  an  hour  and  about  three  hours  before  the  paroxysm  of  pain  is 
due.  Thus  the  maximum  effect  of  the  quinine,  which  is  felt  two  hours 
after  its  ingestion,  will  coincide  with  the  time  of  the  paroxysm  and 
will  prevent  its  occurrence.  While  caries  of  the  teeth  is  an  occasional 
cause  of  neuralgia,  and  the  condition  of  the  teeth  should  always  be 
investigated,  as  the  pain  may  not  be  felt  in  the  region  of  the  infected 
tooth,  yet  in  my  experience  this  cause  is  an  infrequent  one,  and  many 
patients  have  had  all  the  teeth  removed,  on  the  supposition  that  they 
were  the  cause  of  the  affection,  without  any  relief  whatever.     The  so- 


TRIGEMINAL  NEURALGIA.  737 

called  Riggs'  disease,  a  dental  exostosis  to  which  great  importance  has 
been  ascribed,  is  not  a  common  cause  of  neuralgia,  and  its  treatment  is 
rarely,  if  ever,  followed  by  relief  of  the  pain,  if  this  is  truly  neuralgic. 

In  all  conditions  of  trigeminal  neuralgia  the  general  health  of  the 
patient  must  be  considered  as  well  as  the  symptomatic  treatment. 
These  patients,  as  a  rule,  are  anaemic,  and  are  rapidly  run  down  by  the 
starvation  that  is  consequent  upon  the  pain  of  the  act  of  chewing.  It 
is  especially  important  that  every  possible  means  should  be  used  to 
build  up  the  general  health.  They  should  be  well  fed  by  means  of 
fluid  food,  reinforced  by  nutritive  eneraata  in  case  solid  food  cannot  be 
taken.  Alcohol,  cod-liver  oil,  beef  marrow  or  medullary  glyceride, 
glycero-phosphate  of  soda,  hypophosphites,  arsenic,  iron,  and  strych- 
nine should  be  used  freely  as  general  tonics,  and  the  nutrition  generally 
improved  by  daily  baths  and  douches  and  by  massage.  A  change  of 
air  is  often  the  one  thing  that  will  make  the  symptomatic  remedies 
efficacious,  and  the  important  thing  is  to  secure  a  change  from  sea  to 
mountains,  or  mountains  to  sea,  according  to  the  place  where  the 
patient  is  accustomed  to  live.  In  some  persons  the  damp  of  the  sea 
air  tends  to  produce  a  return  of  the  neuralgia,  and  in  these  persons  a 
dry  climate  should  be  sought.  A  warm  climate  is  essential,  and  one 
free  from  dampness.  Egypt,  lower  Florida,  southern  California,  are 
all  excellent.  There  are  no  special  baths  or  health  resorts  that  claim 
to  be  efficacious  in  the  treatment  of  neuralgia,  but  the  regime  secured 
in  a  foreign  water-cure  is  sometimes  of  benefit. 

Remedies  for  the  pain  are  very  numerous.  The  most  important  are 
the  newer  analgesics,  namely,  phenacetin,  10  grains  to  15  grains  every 
two  hours  ;  acetanilid,  5  grains  every  two  hours  ;  antipyrine,  1 5  grains 
every  two  hours,  or  salophen,  15  grains  every  two  hours.  It  is  well 
to  combine  with  each  dose  of  these  drugs  a  grain  of  caffeine  and  one- 
hundredth  of  a  grain  of  strychnine  to  avoid  their  depressing  action  upon 
the  heart.  A  hot  alcoholic  drink  sometimes  gives  immediate  relief. 
Quinine  may  be  used  with  it. 

Aconitine  is  the  best  remedy.  It  is  given  in  pill  or  tablet  form, 
the  French  pills  of  Chapoteaux  being  the  best  preparation.  Each  pill 
contains  g-i-g-  grain  {^  milligramme),  and  the  remedy  must  be  used 
with  sufficient  frequency  to  produce  constitutional  effects.  It  is  iny 
rule  to  begin  with  one  pill  every  four  hours  and  decrease  the  intervals 
each  day  one-half  hour  until  one  pill  every  two  hours  is  being  given, 
or  until  constitutional  effects  are  evident,  namely,  tingling  of  the 
tongue  and  fingers,  sense  of  general  weakness  and  feebleness  of  the 
pulse.  It  is  well  to  combine  with  this  a  fiftieth  of  a  grain  of  strych- 
nine during  the  first  two  days  and  then  yo'w  ^^  ^  grain  of  strychnine, 
when  the  aconitine  is  being  given  every  two  hours.  If  the  strychniue 
does  not  agree  with  the  patient,  or  produces  twitchings,  the  dose  of 
this  may  be  reduced,  or  caffeine  two  grains,  or  spartein  -gL-  grain  may 
be  given  in  its  place.  Some  heart  stimulant  is  to  be  used  in  connec- 
tion with  the  aconitine,  when  the  larger  doses  are  given  and  the  pa- 
tient should  be  warned  against  making  any  sudden  muscular  efforts, 
47 


733  NEURALGIA. 

and  during  the  week  or  ten  days  of  such  treatment  should  walk  very 
little. 

Gelsemium  is  the  remedy  next  in  favor,  and  this  is  to  be  begun 
in  small  dose,  10  drops  of  the  tincture,  or  10  drops  of  the  fluid 
extract,  being  given  every  three  hours,  and  the  dose  increased  by  1 
drop  each  time  until  the  patient  perceives  physiological  effects,  which 
are  a  heaviness  of  the  upper  eyelids  and  a  difficulty  in  opening  the 
eyes.  In  one  obstinate  case  30  drops  of  the  fluid  extract  every  three 
hours  uniformly  cured  the  attack.  This  remedy  may  be  kept  up  at 
the  point  wliich  produces  this  physiological  effect  for  several  days  and 
very  often  gives  marked  relief.  In  patients  of  my  own  this  remedy 
has  succeeded  when  aconitine  had  failed  completely. 

The  tincture  of  colchicum  has  been  used  with  considerable  benefit, 
especially  in  the  gouty  cases.  The  wine  of  colchicum  may  be  given  in  5- 
drop  doses  every  two  or  three  hours  until  a  purgative  action  is  produced, 
and  then  the  dose  may  be  decreased  in  amount,  or  the  frequency  of 
the  dose  may  be  lessened.     The  effect  can  be  continued  for  several  days. 

Butyl  chloral  hydrate  in  5-grain  doses  every  hour  for  4  doses  has 
been  highly  recommended  as  a  remedy  for  neuralgia  of  the  fifth  nerve. 

Preparations  of  arsenic ;  especially  the  cacodylate  of  sodium,  ^  to  :^ 
gr.,  three  times  a  day,  are  of  considerable  service.  They  may  be  used 
in  conjunction  with  the  remedies  already  mentioned,  or  alone,  great 
care  being  taken  to  avoid  toxic  effects. 

The  most  certain  and  satisfactory  remedy  is  opium  or  morphine. 
This  may  be  given  in  the  form  of  extract  of  opium,  or  the  tincture 
of  opium  in  increasing  doses,  by  the  stomach,  or  morphine  may  be 
used  hypodermically.  In  the  majority  of  cases  of  trigeminal  neuralgia 
it  will  eventually  be  used,  although  if  possible  patients  should  be  pre- 
vented from  resorting  to  it  too  soon,  or  too  freely,  as  there  is  no 
disease  in  which  the  morphine  habit  is  a  more  common  sequel  than 
trigeminal  neuralgia.  Yet  in  the  paroxysms  of  pain  it  is  imperative 
to  give  relief,  and  the  mere  fear  of  engendering  the  habit  should  not 
prevent  one  from  using  it  in  appropriate  cases,  especially  in  the  early 
stage  of  the  disease  and  until  other  remedies  can  have  the  time  to 
exert  a  constitutional  effect. 

Strychnine  has  been  used  by  Dana  hypodermically  in  large  doses  for 
the  relief  of  various  forms  of  neuralgia,  a  dose  being  begun  of  ^  of  a 
grain  and  increased  as  high  as  -jig-  of  a  grain  hypodermically  once  in 
twenty-four  hours.  I  have  seldom  seen  any  benefit  result  from  this 
treatment,  but  I  have  seen  severe  strychnine  poisoning  ensue. 

In  individuals  who  are  the  subject  of  arterial  sclerosis  nitro-glycerin 
is  a  remedy  of  considerable  efficacy,  which  may  be  used  in  -^^  or  yJ-^ 
of  a  grain  and  repeated  with  sufficient  frequency  to  produce  a  percep- 
tible effect  upon  the  arterial  tension.  Nitrite  of  sodium  in  1-  or  2- 
,grain  doses  is  equally  efficacious. 

The  extract  of  cannabis  indica  has  some  reputation  in  the  treatment 
of  neuralgia.  It  is  to  be  given  in  tablet  or  pill  form,  ^  of  a  grain  at 
a  dose  several  times  a  day. 


TRIGEMINAL  NEURALGIA.  739 

Preparations  of  the  bromides,  and  bromide  combined  with  chloral 
may  be  oi  some  service  in  the  lighter  forms  of  neuralgia,  but  in  the 
severe  attacks  do  not  have  sufficient  effect  to  quiet  the  pain.  If  a  small 
amount  of  morphine  be  added  to  full  doses  of  these  two  drugs  a  use- 
ful remedy  is  obtained. 

Local  applications  to  the  face  sometimes  give  considerable  relief. 
Applications  of  ice  bags  or  freezing  the  face  by  a  spray  of  chloride  of 
ethyl  applied  to  the  point  of  exit  of  the  branch  of  the  nerve  affected 
are  sometimes  of  considerable  service.  The  majority  of  patients,  how- 
ever, prefer  hot  applications ;  and  a  hot-water  bag  or  a  poultice  or  an 
application  of  cotton  covered  with  oiled  silk,  outside  of  which  a  hot 
bag  or  a  Japanese  hand  furnace  can  be  placed,  will  often  give  consid- 
erable relief.  Most  of  these  patients  prefer  to  protect  the  face  by 
some  woollen  substance  and  thus  avoid  exposure  to  cold  or  to  changes 
of  temperature. 

Sometimes  local  applications  of  lotions  and  salves  are  of  service. 
Weak  chloroform  liniment,  evaporating  lotions  of  opium  and  lead,  or 
ointments  containing  aconite,  morphine,  atropine,  and  veratrine,  have 
been  used  with  some  success.  Rubbing  the  face  with  a  menthol  pencil 
may  give  relief.  The  face  may  be  painted  with  coUodium  in  which 
iodoform,  1  to  15  grains,  has  been  dissolved,  a  thick  coating  being  ap- 
plied over  the  exit  of  the  branch  of  the  nerve  affected.  Camphor  and 
chloral  rubbed  together  into  a  paste  and  applied  has  been  of  benefit. 

Inhalations  of  chloroform  may  be  given  with  good  effect,  but  this, 
of  course,  must  be  used  with  great  caution. 

Electricity  in  the  form  of  galvanism,  a  mild,  continuous  current,  with 
the  positive  pole  upon  the  painful  point  and  the  negative  on  the  back, 
has  been  recommended.  The  application  should  be  five  minutes  in 
duration  over  each  branch  of  the  nerve  and  great  care  should  be  taken 
not  to  make  or  break  the  current  during  the  application,  it  being  be- 
gun very  gradually  by  the  aid  of  a  rheostat.  Sometimes  a  severe  ver- 
tigo may  be  produced  by  applications  of  galvanism  to  the  head  and 
face.  I  have  never  seen  any  good  result  from  this  method  of  treat- 
ment. 

Massage  of  the  face  is  usually  very  painful  in  conditions  of  neuralgia 
and  has  never  in  my  experience  been  of  much  service  in  the  treatment 
of  the  attacks.  Vibrations  maintained  by  a  tuning-fork  electrically 
vibrated,  the  end  of  the  fork  being  in  contact  with  the  affected  branch 
of  the  nerve,  and  vibrations  with  the  end  of  the  fingers  of  an  expert 
masseur,  have  given  relief  in  some  cases. 

The  actual  cautery  cannot  be  applied  to  the  face  on  account  of  the 
scars  which  remain,  and  hence  is  not  used  in  trigeminal  neuralgia. 

Surgical  Treatment.  —  For  many  years  division  of  individual 
branches  of  the  nerve  has  been  practised  for  the  relief  of  neuralgia, 
the  nerve  being  merely  divided  or  being  divided  and  the  central  end 
stretched,  or  a  considerable  section  of  the  nerve  being  removed.  To 
reach  the  peripheral  parts  of  the  supraorbital  or  infraorbital  nerves, 
semilunar  incisions  of  the  eyebrow  or  cheek  are  made  in  the  natural 


740  NEURALGIA. 

lines  of  the  skin,  and  chiselling  out  of  the  orbital  bones  gives  access 
to  the  nerve.  Division  of  the  third  branch  of  the  nerve  within  the 
mouth,  as  it  passes  into  the  ramus  of  the  jaw,  is  a  comparatively  simple 
operation  within  the  reach  of  almost  any  surgeon.  The  deeper  opera- 
tions of  division  of  these  various  roots  at  their  exit  from  the  skull  re- 
quires much  greater  surgical  skill  and  is  very  complicated  and  bloody. 

Recently  surgeons  have  resorted  to  the  injection  of  a  IJ  per  cent, 
solution  of  osmic  acid  for  the  relief  of  mild  cases  of  neuralgia  limited 
in  extent  to  one  branch  of  the  nerve.  Two  minims  of  the  solution 
may  be  injected  slowly  through  a  very  fine  needle  into  the  sheath  of 
the  nerve  after  it  has  been  exposed  as  described.  The  osmic  acid  de- 
stroys the  nerve  fibre  by  dissolving  its  medullary  sheath.  This  opera- 
tion may  be  performed  under  cocaine,  but  the  injection  of  osmic  acid 
causes  severe  pain,  and  therefore  ether  anaesthesia  is  preferable.  I 
have  known  of  two  cases  cured  by  this  method. 

Another  method  of  treatment  is  by  the  injection  of  2  c.c.  of  a  95 
per  cent,  solution  of  alcohol  containing  4  grains  of  cocaine  to  the  ounce 
into  the  branch  of  the  nerve  affected.  This  must  be  done  at  its 
deepest  accessible  point  just  at  its  exit  from  the  skull  by  means  of  a 
stylet,  10  cm.  long  and  15  mm.  in  diameter.  At  the  moment  of  in- 
jection severe  pain  is  felt  in  the  nerve  and  subsequently  its  area  of  the 
skin  is  anaesthetic  for  months.  Success  has  been  reported  by  many,  ^ 
but  is  not  very  lasting  as  the  pain  is  likely  to  recur  after  a  year  or  less. 
Many  unfavorable  complications,  such  as  severe  cellulitis  of  the  face 
and  hard  indurations,  also  perforation  of  the  Eustachian  tube  and  per- 
manent deafness,  have  occurred  after  such  injections,  and  fatal  results 
have  been  recorded.  It  is  a  method  to  be  employed  only  by  one  who 
is  expert. 

While  such  operations  may  give  relief  for  some  months  or  years, 
they  are  not  positively  curative,  but  they  may  be  performed  when  the 
case  is  a  chronic  one  and  the  neuralgia  is  limited  to  a  single  branch  of 
the  nerve.  In  the  majority  of  cases  the  neuralgia  eventually  extends 
to  other  branches,  and  in  such  cases  the  more  radical  operation  advised 
by  Krause  and  Hartley  simultaneously,  of  exsection  of  the  Gasserian 
ganglion  within  the  skull,  may  be  performed.  This  operation  is  car- 
ried out  by  making  a  horseshoe-shaped  incision  through  the  skin, 
periosteum,  and  temporal  and  parietal  bones,  and  laying  down  a  flap 
which  should  be  at  least  four  inches  in  diameter,  the  base  being  half 
an  inch  above  the  base  of  the  skull.  The  dura  is  not  incised,  but  with 
the  brain  is  then  carefully  lifted  away  from  the  base  of  the  skull.  The 
point  of  exit  of  the  fifth  nerve  is  determined,  great  care  being  taken 
to  avoid  injuring  the  optic  and  oculomotor  nerves ;  the  free  branches 
of  the  nerve  are  seized,  the  Gasserian  ganglion  is  pulled  outward 
through  the  dura,  and  the  trunk  of  the  nerve  divided  behind  the  gan- 
glion. Traction  on  the  nerves  is  made  and  they  are  divided  as  far  as 
possible  from  the  ganglion,  which  is  thus  removed  with  a  half-inch  of 
the  nerves.  The  result  of  this  operation  is  to  deprive  the  face  entirely 
of  sensation,  and  the  operation  is  always  followed  by  a  permanent  con- 

*  Patrick.     Jour,  Amer.  Med.  Assoc,  Dec.  11,  1909,  gives  references  and  technique. 


BRACHIAL  NEUBALGIA.  741 

dition  of  tmgling  and  numbness,  with  anaesthesia  of  the  face,  and  loss 
of  taste  on  one  side  of  the  tongue ;  but  is  usually  gives  permanent 
relief  from  the  neuralgia.  The  agonizing  pain  is  so  great  that  these 
patients  are  willing  to  submit  to  any  operation  that  promises  a  cure. 
I  have  seen  a  large  number  of  permanent  cures  in  patients  of  my  own 
upon  whom  Hartley  has  performed  this  operation,  and  Keen,  Krause, 
Horsley  and  others  have  published  many  cases  of  cure.  Recently, 
Frazer  and  Keen  have  obtained  good  results  by  dividing  the  central 
end  of  the  nerve  just  behind  the  Gasserian  ganglion,  without  removing 
the  ganglion. 

Oervico-occipital  Neuralgia. — Neuralgia  of  the  posterior  branches 
of  the  cervical  plexus  and  especially  of  the  great  occipital  nerve,  which 
passes  up  the  back  of  the  neck  and  over  the  back  of  head  to  the 
vertex,  is  occasionally  met  with,  though  it  is  by  no  means  as  common 
as  trigeminal  neuralgia.  Pain  is  felt  along  the  course  of  the  nerves  in 
the  neck,  but  particularly  below  the  occiput  and  in  the  vertex.  There 
are  painful  spots  near  the  exit  of  the  nerve  at  the  base  of  the  skull, 
over  the  occiput,  and  upon  the  vertex.  The  scalp  is  very  likely  to  be 
tender  when  the  pain  is  intense.  Frequently  the  pain  is  bilateral,  and 
very  often  is  continuous  rather  than  paroxysmal,  though  sharp  attacks 
of  pain  may  follow  any  motion  of  the  head.  The  patients  usually  hold 
the  head  in  a  fixed  position  to  avoid  the  pain  produced  by  movement. 
Sometimes  a  swelling  of  the  cervical  glands  accompanies  the  neuralgia. 
In  one  case  reported  by  Johnson,  the  superior  ganglion  and  its  cord 
were  fixed  by  adhesions  which  were  freed  by  operation,  after  which 
the  patient  was  relieved  of  the  neuralgia.  Occasionally  an  attack  of 
trigeminal  neuralgia  is  associated  with  the  cervical  neuralgia.  This 
may  be  due  to  the  central  radiation  of  irritation,  which  is  erroneously 
referred,  or  it  may  be  due  to  a  common  cause  producing  both  affec- 
tions. The  characteristics  of  the  disease  are  similar  to  those  of  trigem- 
inal neuralgia  and  the  treatment  is  that  of  neuralgia  in  general.  In 
one  very  obstinate  case  under  my  care,  McBurney  divided  the  great 
occipital  nerve  at  its  exit  from  the  muscle.  This  had  only  a  tempo- 
rary effect  and  it  was  several  months  before  a  spontaneous  recovery 
ensued. 

Brachial  Neuralgia. — The  various  nerves  of  the  brachial  plexus 
are  occasionally  the  seat  of  neuralgia.  The  pain  may  be  felt  chiefly  in 
and  about  the  plexus  in  the  neck,  but  usually  shoots  outward  into  the 
branches  down  the  arm  and  into  the  forearm  and  hand.  There  are 
painful  points  where  the  nerves  are  superficial  and  can  be  easily  com- 
pressed against  the  bones.  Thus  in  the  axilla,  over  the  circumflex, 
near  the  deltoid,  over  the  musculospiral  as  it  curves  about  the  humerus, 
over  the  ulnar  at  the  elbow,  and  over  the  nerves  at  the  wrist,  pressure 
causes  pain.  The  pain  is  made  worse  by  any  movements  of  the  arm, 
and  the  patient  instinctively  keeps  it  quiet.  The  pain  is  usually  worse 
at  night,  probably  because  of  pressure  exerted  during  sleep,  and  hence 
sleep  is  often  interfered  with.  There  is  often  much  hypersensitiveness 
of  the  skin  and  a  burning  pain,  which  Weir  Mitchell  has  named  "caus- 
algia."     Herpes  zoster  is  a  frequent  complicating  symptom,  and  the 


742  NEVBALGIA. 

position  of  the  vesicles  corresponds  exactly  with  the  distribution  of  the 
cutaneous  branches  of  the  nerves.  Glossy  skin  and  trophic  changes  in 
the  nails  appear  only  when  a  true  neuritis  has  developed. 

Intercostal  Neuralgia.  —  While  the  intercostal  nerves  from  their 
protected  situation  are  usually  exempt  from  injuries  and  wounds^  ex- 
cepting in  cases  of  stab  wounds  of  the  back  or  chest,  they  are  fre- 
quently the  seat  of  neuralgia.  The  neuralgic  pain  is  sharp  and  shoot- 
ing around  the  side  of  the  chest  and  is  often  felt  chiefly  in  the  terminal 
filaments  of  the  nerve  in  front  or  at  its  middle  branch  in  the  line  of 
the  axilla.  At  these  two  points  of  exit  of  the  branches  and  also  at  the 
point  of  exit  of  the  posterior  branch  near  to  the  spine,  points  of  tender- 
ness are  usually  present.  If  the  upper  dorsal  nerves  are  affected  the 
pain  may  also  be  felt  in  the  inner  side  of  the  arm  and  may  be  attended 
by  tachycardia.  The  pain  is  increased  by  breathing,  by  coughing,  or  by 
any  movement  of  the  chest.  A  dull,  aching,  pressing  pain  is  felt  be- 
tween the  attacks,  and  patients  usually  like  to  support  the  side  and 
compress  it.  Others  cannot  endure  the  touch  of  their  clothing.  In- 
tercostal neuralgia  is  very  often  followed  by  an  attack  of  herpes  zoster. 
The  cases  of  Kaposi  have  proved  that  when  herpes  occurs  the  disease 
is  sometimes  due  to  a  hemorrhage  within  the  posterior  spinal  ganglion 
of  the  nerve  affected.  In  other  cases,  a  parenchymatous  and  intersti- 
tial neuritis  have  been  found.  The  herpes  may  precede  the  neuralgia 
and  the  latter — especially  in  old  people — may  persist  long  after  the 
eruption  has  subsided. 

Any  of  the  ordinary  causes  of  neuralgia  may  produce  intercostal 
neuralgia.  Intercostal  neuralgia  is  frequently  due  to  a  secondary  im- 
plication of  the  nerve  from  disease,  either  in  the  bones  of  the  spine  or 
chest,  or  in  infectious  conditions  of  the  pleura.  Thus  empyema  is 
occasionally  complicated  by  intercostal  neuralgia.  It  has  occurred 
from  pressure  by  aneurisms.  Its  relative  frequency  in  women  suggests 
that  the  same  causes  that  produce  mastodynia  {q.  v.^,  a  variety  of 
intercostal  neuralgia,  may  also  cause  the  disease. 

Diagnosis. — The  diagnosis  of  intercostal  neuralgia  is  made  from  the 
exceeding  sharp,  shooting  character  of  the  pain,  and  by  the  existence 
of  painful  points  upon  the  chest  at  the  exit  of  the  nerves.  It  may 
suggest  angina  pectoris  when  tachycardia  occurs,  but  the  course  of  the 
two  diseases  differs  so  widely  that  a  diagnosis  is  easily  reached. 

Pleurodynia,  which  is  a  muscular  rheumatism  of  the  intercostal  mus- 
cles, or  may  be  due  to  a  rupture  of  muscular  fibres  after  coughing,  pro- 
duces a  somewhat  similar  pain,  increased  by  respiration  ;  but  in  this 
case  the  tenderness  is  not  felt  especially  at  the  point  of  exit  of  the 
nerve.  In  pleurisy  the  pain  is  less  exactly  limited  in  its  distribu- 
tion, and  the  physical  signs  of  the  disease  enable  a  diagnosis  to  be 
reached.  The  fact  that  disease  of  the  viscera  may  give  rise  to  pain  in 
the  chest,  as  described  in  the  section  on  referred  pains,  is  not  to  be 
forgotten  (see  page  752). 

Treatment. — The  treatment  is  by  local  applications  along  the  course 
of  the  nerve  ;  blisters  or  the  actual  cautery  over  the  exit  of  the  spinal 
branches,  and  special  measures  such  as  are  described  in  the  treatment 


WMBO-ABt)OMmAL  NEtJ&ALGlA.  743 

of  neuralgia  in  general.  Edinger  recommends  a  spray  of  chlorsethyl. 
For  the  r^ief  of  the  herpes,  applications  of  oxide  of  zinc  ointment 
may  be  made,  or  the  herpetic  vesicles  may  be  painted  with  collodion, 
to  which  a  small  amount  (1  per  cent.)  of  carbolic  acid  or  thymol  has 
been  added.  Care  should  be  taken  to  avoid  any  septic  infection  of 
the  exposed  skin  when  the  vesicles  have  ruptured.  In  old  persons 
opiates  should  be  very  carefully  given. 

Neuralgia  of  the  Breast — Mastodynia. — This  form  of  neuralgia 
is  very  rare  and  occurs  chiefly  in  women.  It  may  develop  during 
pregnancy  and  is  especially  frequent  during  the  last  weeks.  It  occa- 
sionally occurs  during  lactation  and  may  be  so  severe  as  to  interfere 
greatly  with  nursing.  It  sometimes  develops  in  women  during  the 
menstrual  period,  or  in  the  course  of  uterine  or  ovarian  disease.  In 
one  of  my  cases  the  attacks  lasted  three  days  at  each  monthly  period 
for  several  years,  requiring  the  use  of  morphine  at  the  time.  It  also 
occurs  as  a  direct  result  of  carcinoma  and  other  tumors  of  the  breast ; 
but  occasionally  it  occurs  as  a  result  of  anaemic  or  neurasthenic  states 
without  concurrent  afiection  of  the  genital  organs.  In  fact,  any  one 
of  the  general  causes  of  neuralgia  may  be  capable  of  producing 
mastodynia. 

The  pain  is  usually  located  deep  in  the  gland,  is  very  severe,  and 
occurs  in  paroxysms,  but  there  is  usually  a  dull,  heavy  feeling  or 
aching  pain  all  the  time.  Sometimes  the  pain  is  superficial,  and  the 
skin  of  the  breast,  especially  about  the  nipple,  is  exquisitely  sensitive ; 
the  nipple  may  be  congested  and  the  entire  breast  red  and  swollen. 
Erb  has  seen  a  case  in  which  the  pain  excited  the  function  of  the 
gland.  There  are  tender  points  along  the  spinous  processes  of  the 
second  to  the  sixth  dorsal  vertebrse.  The  course  of  the  case  is  a  slow 
one.  Sometimes  the  pain  persists  in  spite  of  treatment  until  preg- 
nancy is  over,  or  until  lactation  ceases. 

In  addition  to  general  measures  of  treatment  for  neuralgia  already 
described,  some  relief  attends  the  adjustment  of  a  support  to  the  breast, 
or  a  firm  bandage  to  the  breast.  Local  applications  of  cocaine  in  4 
per  cent,  solution  to  the  nipple  sometimes  quiets  the  pain.  Ointment 
of  belladonna  and  aconite  or  evaporating  solution  of  lead  and  opium 
may  be  of  benefit.  Nageli  has  afforded  relief  by  stretching  the  nerve, 
the  entire  breast  being  firmly  grasped  in  both  hands  and  slowly  but 
steadily  lifted  for  twenty  or  thirty  seconds  several  times  a  day.  This 
is  a  simple  method  which  should  be  tried  in  every  case. 

Lumbo-abdominal  Neuralgia. — The  lower  dorsal  nerves  and  the 
lumbar  nerves  send  branches  over  the  back  and  abdomen.  These 
branches  are  occasionally  the  seat  of  neuralgia,  which  resembles  inter- 
costal neuralgia,  to  which  it  is  strictly  homologous.  The  pain  shoots 
about  the  side  of  the  body  and  is  at  times  intense  and  often  burning 
in  character.  There  are  tender  points  at  the  posterior,  lateral,  and 
anterior  exits  of  the  branches  of  the  nerves.  Neuralgia  in  this  locality 
is  especially  likely  to  be  attended  by  herpes.  I  have  seen  a  case  in 
which  abdominal  neuralgia  followed  intercostal  neuralgia,  both  being 
attended  by  herpes.     In  this  case,  as  in  many  others,  the  course  of  the 


744  NEUBALGIA. 

case  was  slow,  the  pain  lasting  several  weeks,  and  resisting  all  forms 
of  treatment.  The  patient  was  a  middle-aged  woman,  the  subject  of 
chronic  arterial  changes  and  cirrhosis  of  the  kidneys.  The  pain  in 
lumbo-abdominal  neuralgia  is  often  dull  and  continuous.  It  fre- 
quently extends  downward  into  the  groin  and  is  felt  in  the  penis  or 
labium,  on  which  parts  herpes  appears.  This  condition  is  not  to  be 
confounded  with  neuralgia  of  the  testicles  and  ovaries,  which  is  an 
affection  of  the  sympathetic  system  supplying  these  organs. 

There  are  no  special  therapeutic  indications  in  this  form  of  neuralgia. 

Crural  Neuralgia. — The  anterior  crural  nerve,  which  extends 
down  the  inner  surface  of  the  thigh  and  leg  and  reaches  the  ankle,  is 
occasionally  the  seat  of  neuralgia.  The  condition  may  develop  in 
the  course  of  sciatica.  It  is  more  common  in  men  than  in  women, 
and  usually  develops  after  hard  muscular  work  or  long  marches, 
exposure  to  cold,  or  injuries.  It  is  to  be  remembered  that  pain  in  this 
nerve  is  often  the  first  sign  of  caries  of  the  spine.  It  is  said  that  in 
diabetes  crural  neuralgia  and  sciatica  are  the  most  frequent  forms  of 
neuralgia  produced.  The  most  obstinate  case  of  the  disease  which  I 
have  seen  was  in  a  diabetic  patient.  Painful  points  are  found  in  the 
inguinal  canal,  on  the  front  of  the  thigh,  on  the  inner  side  of  the  knee, 
and  on  the  inner  malleolus.  Herpes  frequently  appears.  This  form 
is  not  to  be  mistaken  for  the  reflex  pain  often  felt  by  women  at  the 
menstrual  period  or  with  ovarian  disease  in  this  locality.  The  treat- 
ment is  the  same  as  that  for  sciatica. 

Painful  Knee.  —  Haffa^  has  called  attention  to  the  causes  of  pain 
in  the  knee  not  due  to  crural  neuralgia.  They  are  (1)  arthritic  muscu- 
lar atrophy.  Inactivity  of  the  hip  joint  or  knee  joint  from  any  cause 
may  lead  to  an  atrophy  of  the  quadriceps  femoris.  This  muscle  holds 
the  capsule  of  the  knee  joint  tense  and  if  it  is  relaxed  the  capsule  may 
get  between  the  patella  and  the  condyle  of  the  femur  or  between  the 
condyles  of  femur  and  tibia,  causing  pain  on  walking.  It  is  to  be 
cured  by  rest  in  bed  with  massage  of  the  muscle  and  its  constant  exer- 
cise by  electricity  till  the  atrophy  is  removed.  (2)  A  second  cause  of 
pain  in  the  knee  is  the  dislocation  of  a  meniscus  in  the  joint.  This 
follows  slight  injuries  and  causes  a  sudden  pain  and  a  flexion  of  the 
leg  with  rotation  away  from  the  injured  meniscus,  the  hard  edge  of 
which  can  be  felt  and  is  tender.  This  needs  operative  treatment.  (3) 
A  third  condition  causing  a  painful  knee  is  a  mass  of  fat  in  the  joint 
between  the  condyles  growing  from  a  papilla  in  the  capsule.  This 
causes  a  swelling  and  must  be  removed. 

Sciatica.  —  Sciatica  is  neuralgia  of  the  sciatic  nerve.  It  is  a  very 
conunon  affection,  more  frequently  met  with  in  males  than  in  females, 
and  in  adults  than  in  persons  under  the  age  of  twenty  years.  Persons 
between  the  age  of  forty  and  fifty  years  are  most  liable  to  the  disease. 

Etiology.  —  Gout  and  rheumatism  are  the  chief  causes  of  sciatica. 

It  can  often  be  directly  traced  to  exposure  to  cold.     It  is  particularly 

frequent  in  diabetic  patients.     It  may  follow  any  of  the  infectious 

diseases  or  may  be  due  to  alcoholism.     The  poisonous  agents  which 

^  Berliner  klin.  Wochen.,  1904  ;  Nos.  1  and  2. 


SCIATICA.  745 

cause  multiple  neuritis  may  cause  sciatica.  Direct  pressure  upon  the 
sciatic  nerve  by  sitting  in  an  uneasy  position  or  in  hard  chairs  with 
sharp  edges,  or  pressure  exerted  from  causes  acting  within  the  pelvis, 
such  as  accumulations  within  the  rectum,  pelvic  inflammation,  or 
tumors  of  the  uterus  or  ovaries,  or  the  condition  of  pregnancy  may 
cause  sciatica.  Disease  of  the  sacral  or  thigh  bones  may  produce  sci- 
atica. In  one  case  under  my  care  a  long  standing  sciatica  was  finally 
explained  by  the  development  of  an  osteosarcoma  of  the  pelvis.  In- 
juries are  a  frequent  cause  of  sciatica,  as  in  falls  upon  the  buttocks  and 
fractures  of  the  thigh.  Lifting  heavy  weights  has  brought  on  sciatica. 
Workers  who  stand  or  move  the  legs  at  machines  are  particularly 
liable.  The  sciatic  nerve  is  well  supplied  with  bloodvessels,  and  it  is 
possible  that  a  venous  congestion  from  pressure  is  an  exciting  cause 
of  disturbance  of  function  in  the  nerve  in  these  cases.  Sciatica  often 
follows  hemorrhoids  and  varicose  veins  in  the  leg.  It  is  difficult  to 
separate  sharply  sciatica  from  sciatic  neuritis,  and  in  many  cases  where 
the  disease  lasts  a  considerable  length  of  time,  it  is  reasonable  to 
suppose  that  a  neuritis  is  present.  In  fact,  in  all  the  cases  in  which 
an  autopsy  has  been  obtained,  an  interstitial  neuritis  with  congestion 
of  the  vessels,  hemorrhages  in  the  sheath,  and  secondary  degenerations 
in  the  nerve  fibers  have  been  found.  Many  cases  are  so  short  in  their 
duration,  the  symptoms  are  so  distinctly  intermittent,  and  the  cessation 
of  the  pain  is  so  instantaneous  either  upon  change  of  position  or  upon 
local  applications,  as  to  make  it  improbable  that  a  true  neuritis  has 
developed. 

Symptoms.  —  The  symptoms  of  sciatica  are  pain  in  the  back  of  the 
thigh  and  in  the  outer  side  of  the  leg,  referred  quite  distinctly  to  the 
course  of  the  nerve.  Sometimes  when  the  upper  branches  of  the  nerve 
are  involved  pain  is  felt  over  the  sacrum  and  buttock  as  high  as  the 
waist  line.  In  other  cases  these  branches  escape.  Usually  the  pain 
is  most  intense  about  the  sciatic  notch  and  down  the  back  of  the  thigh 
to  the  knee.  Sometimes  it  is  limited  to  the  lower  branches  of  the 
nerve  upon  the  outer  side  of  the  leg  to  the  foot. 

In  the  severer  type  of  case  the  entire  distribution  of  the  nerve  is  the 
seat  of  pain.  The  pain  is  not  as  diffuse  as  in  muscular  rheumatism, 
and  when  the  patient  is  asked  to  indicate  its  position  he  follows  down 
with  the  tips  of  his  fingers  the  line  of  the  nerve  from  its  exit  in  the 
sciatic  notch  to  the  external  condyle  of  the  ankle.  The  pain  occurs 
in  paroxysms  and  may  be  agonizing  in  its  intensity ;  is  usually  in- 
creased by  movement,  by  walking,  especially  by  going  up  stairs,  and 
can  always  be  elicited  by  hyperextension  of  the  leg  upon  the  thigh  and 
of  the  thigh  upon  the  pelvis — a  position  which  stretches  the  sciatic 
nerve.  It  is  attended  by  tenderness  at  those  points  where  the  nerve 
can  easily  be  compressed  between  the  fingers  and  a  prominent  portion 
of  the  bone,  namely,  above  the  hip-joint  near  the  posterior  iliac  spine, 
at  the  sciatic  notch,  at  the  middle  of  the  thigh,  behind  the  knee,  below 
the  head  of  the  fibuhi,  behind  the  external  condyle  of  the  ankle,  and 
on  the  back  of  the  foot.  The  pain  is  usually  dull  in  character  and 
constantly  present,  but  there  are  acute  exacerbations  and  these  are 


746  neuhalgia. 

often  attended  by  sensations  of  burning  or  feeling  as  if  water  were 
trickling  along  the  limb,  or  sharp,  darting  pains  through  the  length 
of  the  nerve.  The  pain  is  a  deep-seated  one  and  is  referred  to  the 
muscles  or  the  bone,  never  to  the  skin.  It  may  be  increased  by  ex- 
tension of  the  leg  and  thigh  together.  It  is  also  increased  by  abduction 
against  resistance  of  the  unaffected  thigh  (Bonnet's  sign)  and  by  flexion 
against  resistance  of  the  unaffected  thigh  (Martin's  sign).  Pains  are 
usually  worse  at  night,  when  the  patient  gets  warm  in  bed,  though,  as 
a  rule,  warmth  to  the  limb  is  grateful  and  the  patients  instinctively 
avoid  exposure  to  cold. 

The  gait  is  affected  in  sciatica,  the  patient  limping  and  moving  the 
entire  limb  stiffly.  The  toe  is  turned  out,  the  leg  is  slightly  abducted, 
and  the  patient  instinctively  rotates  the  spine  to  the  well  side.  In  ex- 
treme cases  there  may  develop  a  tendency  to  lateral  curvature  of  the 
spine  away  from  the  affected  side.  In  the  more  intense  cases  the 
patient  is  confined  to  the  bed  by  the  pain,  cannot  bear  his  weight  upon 
the  limb,  cannot  bear  to  have  it  moved,  and  finds  it  easy  only  in  one 
position,  which  varies  in  different  cases.  The  leg  is  usually  abducted, 
rotated  outward,  and  flexed  at  the  knee.  It  is  best  to  allow  the  patient 
to  ascertain  the  position  in  which  he  is  most  comfortable  and  to  keep 
the  limb  in  that  position 'supported  by  pillows.  The  pain  may  be  so 
intense  as  to  cause  reflex  spasms  and  twitchings  of  the  leg,  and  is  very 
conunonly  associated  with  tingling  and  numbness  in  the  leg,  or  by 
sensations  of  pins  and  needles,  the  foot  being  asleep  constantly.  When 
the  sciatica  goes  on  to  a  neuritis,  anaesthesia  may  develop  in  the  outer 
side  of  the  leg  below  the  knee  and  in  the  back  of  the  foot,  and  the 
muscles  of  the  calf  of  the  leg  may  be  weak  or  even  paralyzed  and 
atrophied,  showing  a  reaction  of  degeneration. 

The  course  of  the  disease  varies  very  much  in  different  cases.  There  is 
sometimes  a  sudden,  acute  onset  which  in  cases  due  to  exposure  to  cold  or 
to  rheumatism,  or  to  gout,  is  often  attended  by  a  slight  rise  of  tempera- 
ture and  constitutional  disturbances.  Usually  the  onset  is  gradual.  Pain 
occurs  in  paroxysms,  which  become  more  and  more  frequent  and  severe 
during  several  days,  and  by  the  end  of  a  week  the  pain  has  become  con- 
tinuous. In  bad  cases  the  patient  limps  or  is  unable  to  walk.  "  This  con- 
dition may  remain  for  several  weeks  or  may  subside  gradually.  Recovery 
ensues  within  three  or  four  weeks,  though  in  many  cases  months  pass 
before  the  patient  is  free  from  pain.  There  is  a  great  tendency  to  relapse 
in  cases  of  sciatica.  I  have  rarely  known  a  patient  to  escape  a  second 
and  third  attack.  Even  when  recovery  has  ensued,  pain  can  be  pro- 
duced by  the  slightest  tendency  to  overstretch  the  nerve,  or  by  too  great 
exertion  in  walking.  I  have  never  seen  a  bilateral  sciatica,  though  a 
number  of  cases  are  recorded.  Even  in  the  chronic  cases  improvement 
after  a  time  occurs,  but  where  a  neuritis  has  been  set  up  the  pain  is  re- 
mittent rather  than  intermittent.  In  the  cases  which  are  due  to  pressure 
within  the  pelvis  the  pain  is  more  likely  to  be  in  the  periphery  of  the 
nerve  and  its  terminal  distribution  than  in  the  trunk  upon  the  thigh. 
In  the  cases  where  neuritis  is  present  there  is  usually  tenderness  along  the 
entire  course  of  the  nerve,  as  well  as  at  the  painful  points  of  Valleix. 


SCIATICA.  747 

Herpes  is  a  common  complication  of  sciatica.  It  is  sometimes  very 
extensive  over  the  back  of  the  thigh  and  on  the  buttock.  The  her- 
petic vesicles  may  become  large  and  confluent,  so  that  the  affection  of 
the  skin  may  give  as  much  discomfort  as  the  pain.  In  this  condition 
the  skin  should  be  protected  by  applications  of  oxide  of  zinc  ointment 
and  bandaging  with  cotton-wool. 

French  authors  have  called  attention  to  the  characteristic  position  of 
the  limb  in  standing  and  in  walking,  which  is  characteristic  of  sciatica 
and  serves  to  distinguish  it  from  muscular  rheumatism,  or  from  hip- 
joint  disease.^  In  sciatica  the  thigh  is  somewhat  adducted,  the  fold 
of  the  buttock  is  depressed,  and  the  knee  is  slightly  flexed,  even  when 
standing  in  an  upright  position.  The  entire  spine  is  somewhat  de- 
flected with  convexity  to  the  affected  side.  This  is  due  to  an  instinc- 
tive effort  to  support  the  weight  on  the  well  limb.  There  is  some- 
times a  slight  wasting  of  the  muscles  in  the  limb. 

Diagnosis. — The  diagnosis  of  sciatica  is  usually  evident  from  the 
characteristic  symptoms,  but  it  has  been  mistaken  for  disease  of  the 
hip-joint,  for  caries  of  the  sacrum,  and  for  lesions  in  the  cauda  equina. 
In  hip-joint  disease  the  pain  is  usually  felt  first  in  the  inner  side  of  the 
knee.  It  is  not  associated  with  tenderness  along  the  course  of  the 
sciatic  nerve,  though  pain  may  be  felt  all  about  the  hip-joint.  There 
is  usually  a  limitation  of  the  motion  of  the  joint  and  possibly  some 
shortening  of  the  thigh,  and  a  characteristic  rigid  position  of  adduc- 
tion with  slight  rotation  is  found  in  hip-joint  disease.  In  caries  of  the 
sacrum,  while  the  pain  may  be  felt  in  the  sciatic  nerve,  there  are  usually 
symptoms  of  tenderness  along  the  sacrum,  tenderness  in  the  motion  of 
the  pelvic  bones  upon  the  sacrum,  and  the  pain  is  less  unilateral  than 
in  sciatica.  A  rheumatic  affection  of  the  sacro-iliac  tendons  and  liga- 
ments does  not  produce  pain  below  the  exit  of  the  sciatic  nerve  in  the 
thigh.  In  muscular  rheumatism  the  pains  are  diffused  and  are  not 
located  exactly  in  the  course  of  the  sciatic  nerve. 

In  affections  of  the  cauda  equina,  meningeal  thickening,  hemor- 
rhages or  tumors,  the  symptoms  are  rarely,  if  ever,  unilateral  in 
character.  Considerable  pain  is  usually  felt  over  the  sacrum,  and 
incontinence  of  urine  and  feces,  with  anaesthesia  about  the  rectum,  are 
early  symptoms  which  are  not  present  in  sciatica. 

Locomotor  ataxia  very  often  produces  severe  pains  in  the  course  of 
the  sciatic  nerve,  and  although  the  earliest  sign  of  this  disease  is  more 
often  pain  in  the  distribution  of  the  crural  nerve,  sciatica  may  be  its  first 
symptom.  In  every  case  of  sciatica,  therefore,  the  knee-jerk  should 
be  tested  and  the  pupil  be  carefully  observed  to  detect  any  lack  of 
reaction  to  light;  these  reflexes  are  not  affected  in  sciatica.  Anaesthetic 
areas  should  be  sought  for,  as  these  are  not  present  in  true  sciatica, 
but  develop  early  in  locomotor  ataxia; 

Prognosis. — The  prognosis  in  sciatica  is  fairly  good  as  regards 
eventual  recovery,  but  no  statement  can  be  made  with  regard  to  the 
duration  of  any  particular  attack,  for  although  the  duration  of  each 

'See  also  II.  Eliret,  Mittheilungen aus der  Grenzgebiet der Med.  u.  Chirurg.,  1898,  695. 


748  NEURALGIA. 

attack  is  somewhat  in  proportion  to  the  severity  of  the  symptoms, 
relapses  are  so  common  that  statements  in  regard  to  duration  are 
never  precise.  I  have  known  cases  to  last  two  or  three  years  and 
then  recover. 

Hyde  has  given  some  interesting  facts  in  his  analysis  of  200  cases 
which  may  be  quoted  here.  The  duration  was  less  than  one  month  in 
45  cases;  from  one  to  three  months  in  70  cases;  from  three  to  six 
months  in  29  cases ;  from  six  to  twelve  months  in  29  cases ;  two  years 
in  15  cases;  three  years  in  6  cases;  four  years  in  1  case;  five  years  in 
2  cases,  and  six,  seven,  and  ten  years  in  one  case  each.  Forty-three 
of  Hyde's  cases  had  had  previous  attacks. 

Treatment.  —  In  addition  to  the  general  statements  with  regard  to 
the  treatment  of  neuralgia,  all  of  which  may  be  applied  to  sciatica,  a 
few  special  indications  may  be  given.  The  cause  must  be  removed 
if  possible.  In  all  cases  active  purgatives  must  be  given  at  the  outset. 
In  cases  due  to  cold  or  rheumatism  salicylates  and  salophen  are  of  the 
greatest  service.  Salophen  or  aspirin  may  be  given  in  15-grain  doses 
every  two  hours.  Absolute  rest  of  the  limb  is  most  important,  and 
the  patient  should  be  advised  to  remain  upon  the  bed  or  couch,  even 
from  the  beginning  of  the  disease,  being  allowed  to  assume  the  position 
which  gives  the  greatest  comfort,  as  this  varies  in  different  individuals. 
All  positions  and  all  movements  which  intensify  the  pain  should  be 
avoided.  I  have  not  seen  any  benefit  from  fixation  of  the  limb,  either 
by  a  long  splint,  as  has  been  recommended  by  Hammond,  or  in  a  plas- 
ter bandage  as  has*  been  suggested  by  others.  Hot  applications  are  of 
the  greatest  service  both  in  the  acute  and  chronic  stage  of  the  disease. 
The  old-fashioned  remedy  of  ironing  the  limb  with  a  hot  iron  is  of  dis- 
tinct service.  The  application  of  flaxseed  poultices  is  of  benefit  in  the 
acute  stage,  but  the  most  efficacious  remedy  is  the  application  of  the 
Paquelin  cautery  along  the  course  of  the  nerve.  This  should  be  ap- 
plied daily  from  the  very  beginning  of  the  disease.  Or  small  fly  blis- 
ters should  be  applied  at  the  points  of  tenderness  along  the  trunk,  and 
should  be  repeated  every  other  day.  If  the  cantharides  is  applied  by 
means  of  collodium  the  blisters  need  not  be  more  than  one-quarter  of 
an  inch  in  diameter,  and  as  many  as  twenty  may  be  placed  in  the 
course  of  the  nerve. 

A  very  efficacious  method  of  the  treatment  of  sciatica  is  exposure  of 
the  limb  to  very  high  temperature  in  an  apparatus  in  which  hot  air 
can  be  produced.  Thus  the  temperature  of  the  air  may  be  raised  to 
250°  or  300°  F.,  the  limb  being  encased  in  the  apparatus.  And  such 
applications  may  be  repeated  twenty  minutes  in  duration  daily. 

It  often  gives  much  relief  to  expose  the  limb  to  a  stream  of  steam 
along  the  course  of  the  sciatic  nerve.  Hot- water  massage  is  also  of  a 
great  deal  of  service,  such  as  is  applied  at  Wildbad,  Ragatz,  and  Bath,  at 
Aix-les-Bains  and  at  the  Hot  Springs  of  Virginia,  the  stream  of  water 
under  pressure  from  twenty  to  thirty,  or  even  forty  pounds,  being 
directed  upon  the  limb,  the  painful  points  along  the  nerve  being  pro- 
tected by  the  hand.     Long-continued  hot  sitz  baths  are  often  of  ser- 


NEURALGIA   OF  THE  TESTICLES  OB  OVARIES.  749 

vice  aud  give  the  patients  relief.  Richfield  Springs  has  a  good  repu- 
tation in  tlfe  treatment  of  sciatica,  and  I  have  known  patients  to 
recover  after  a  course  of  sulphur  baths  there.  Massage  of  the  limb  is 
sometimes  of  service  if  it  is  done  with  great  skill  and  pressure  is  but 
lightly  exerted  upon  the  nerve  trunk.  Mud  or  sand  baths  heated  to 
100°  F.  are  of  great  service.  A  spray  of  chloride  of  ethyl  which 
causes  local  freezing  sometimes  gives  immediate  relief.  Hypodermic 
injections  into  the  nerve  have  been  practised  and  recommended. 
Hypodermically  an  injection  of  chloroform,  of  a  2  per  cent,  solution 
of  cocaine,  or  of  a  1  per  cent,  solution  of  atropine,  or  simply  of  dis- 
tilled water,  may  be  given.  Good  results  have  been  obtained  by  hypo- 
dermic injections  of  normal  salt  solution  at  a  freezing  temperature,  32° 
F.,  not  injected  into  the  nerve  but  into  the  thigh  near  the  nerve  trunk, 
30  c.c.  being  used  daily.  Osmic  acid  solutions  are  dangerous.  In 
a  few  cases  where  the  nerve  has  been  felt  to  be  much  swollen  puncture 
by  a  needle  has  resulted  in  the  evacuation  of  serum  from  the  sheath, 
and  has  given  relief.  Long  needles  are  to  be  used  under  strict  aseptic 
precautions.  They  are  to  be  inserted  and  left  in  an  hour,  the  limb 
being  kept  immovable  during  this  time.  Liniments  and  ointments 
containing  belladonna  or  aconite  may  be  rubbed  in  along  the  course  of 
the  nerve,  and  are  sometimes  of  benefit.  The  general  medicinal  treat- 
ment by  internal  remedies  may  be  conducted  along  the  line  already 
described  in  the  treatment  of  neuralgia.  Electricity  I  have  never 
found  of  much  service,  but  long-continued  applications  of  galvanism 
of  very  mild  strength  (five  or  six  milliamperes)  from  large  sponges 
may  be  tried. 

In  chronic  cases  where  all  other  means  have  failed  to  relieve  it  has 
been  proposed  to  stretch  the  nerve,  and  this  is  probably  of  service  in 
cases  where  an  interstitial  neuritis  has  produced  adhesions  between  the 
adjacent  bundles  of  nerve  fibres  and  constriction  of  the  blood-vessels. 
A  nerve  may  be  stretched  by  using  hyperextension  of  the  leg,  a  pro- 
ceeding which  has  to  be  done  under  chloroform,  as  it  is  very  painful. 
Or  the  nerve  may  be  stretched  by  cutting  down  upon  it  just  below  its 
exit  in  the  sciatic  notch,  dividing  the  sheath  and  stretching  it  with  the 
fingers.  Some  cases  have  been  reported  of  improvement  after  this 
procedure.  I  have  seen  one  cure  in  a  very  obstinate  case.  I  have 
seen  several  failures.  Other  cases  have  been  reported  in  which  nerve 
stretching  has  been  followed  by  paralysis  in  the  muscles  supplied  by 
the  affected  nerve.  It  should  only  be  resorted  to  after  every  other 
means  has  been  tried,  and  no  positive  promise  of  relief  should  be 
given. 

Neuralgia  of  the  Testicles  or  Ovaries. — Neuralgia  in  these  organs 
is  usually  the  result  of  some  congestion  of  the  veins  due  to  organic 
disease  or  to  pressure.  But  occasionally  it  may  be  due  to  some  one 
of  the  general  causes  of  neuralgia  already  mentioned.  The  pain  is 
very  intense  and  most  demoralizing.  It  causes  great  restlessness  and 
much  mental  depression.  It  is  occasionally  attended  by  sexual  excite- 
ment. Neuralgia  of  the  ovaries  may  coincide  with  the  menstrual 
function. 


750  NEURALGIA. 

The  treatment  is  first  directed  to  reducing  local  congestion.  This 
may  be  done  by  elevating  the  pelvis  and  letting  gravitation  empty  the 
veins.  Applications  of  heat,  especially  hot  poultices  to  the  testes,  and 
hot  vaginal  douches  or  rectal  injections,  to  be  taken  in  a  recumbent 
position,  are  usually  of  great  service.  Resort  may  be  had  to  the 
stronger  narcotics  early,  as  it  is  better  to  cut  an  attack  short  than  to 
allow  it  to  go  on.  And  attention  to  general  measures  for  building  up 
the  health  must  not  be  omitted. 

Neuralgia  of  the  perineum,  of  the  prostate  gland,  and  of  the  neck 
of  the  bladder  in  males,  and  neuralgia  of  the  rectum  in  both  sexes,  is 
occasionally  complained  of.  If  no  local  disease  is  present  and  the 
affection  is  not  traceable  to  lithsemia  or  to  hemorrhoids,  it  is  probably 
caused  by  local  congestion.  Massage  of  the  perineum,  the  pelvis  being 
raised,  will  give  relief,  or  hot  applications  to  the  perineum  may  be 
effective.  If  these  fail,  massage  of  the  prostate  through  the  rectum, 
or  stripping  the  seminal  vesicles  may  do  good.  A  continuous  irriga- 
tion of  the  rectum  with  hot  water  by  means  of  the  double  tube  devised 
by  Keyes  is  of  much  service  in  these  cases.  Sitz  baths,  either  hot  or 
cold,  are  of  value.  As  in  neuralgia  of  the  genital  organs  prompt 
relief  must  be  given  by  narcotics  to  avoid  a  state  of  mental  depression. 
I  have  found  an  ointment  or  suppository  of  cocaine  and  hyoscyamus 
of  great  service.  Weir  Mitchell  has  seen  this  form  of  neuralgia  as  an 
early  sign  of  tabes,  an  observation  which  I  can  confirm,  having  seen 
two  such  cases.  I  have  also  known  it  to  occur  in  lithsemic  individuals 
and  in  persons  whose  circulation  was  poor,  after  sexual  excitement, 
both  of  a  natural  and  of  an  unnatural  kind. 

Coccygodynia.  —  Neuralgia  of  the  coccygeal  nerves  is  an  exceed- 
ingly rare  affection.  But  pain  in  the  tip  of  the  spine  is  very  common, 
and  has  received  the  name  of  coccygodynia.  It  may  be  a  local  affec- 
tion, due  to  injuries,  especially  in  women  after  confinement ;  to  falls 
on  the  seat  or  to  caries  of  the  spinal  bone.  It  is  usually  in  my  opinion 
a  referred  pain  of  central  origin,  and  may  develop  in  very  many  func- 
tional or  organic  diseases  of  the  nervous  system.  In  neurasthenic, 
hysterical  and  ansemic  persons,  chiefly  in  women,  it  is  a  frequent  com- 
plaint. It  may  be  associated  with  pain  in  the  nape  of  the  neck  or 
between  the  shoulders,  with  irritable  spine,  or  with  any  hysterical  state. 
It  is  a  very  common  complaint  in  cases  of  traumatic  neurosis.  In  the 
majority  of  cases  it  is  a  purely  hysterical  symptom,  and  is  increased  by 
any  local  treatment  or  by  anything  which  directs  attention  to  it.  In 
several  such  cases  where  gynecologists  have  exsected  the  coccyx  the 
pain  has  persisted  after  the  operation.  As  the  hysteria  or  neurasthenia 
passes  off  the  pain  is  forgotten.  Hence  it  should  never  be  treated 
directly,  but  only  as  a  symptom  of  a  general  condition.  In  the  few 
cases  which  are  true  neuralgia  the  same  treatment  as  that  of  sciatica 
will  be  applicable. 


REFERRED  PAINS.  751 


REFERRED  PAINS. 


There  are  a  great  many  conditions  of  disease  in  which  more  or  less 
severe  pain  is  felt  in  various  parts  of  the  body  at  a  distance  from  a  dis- 
eased organ.  The  explanation  for  the  referring  of  these  pains  to  a 
part  of  the  body  which  is  really  not  affected  is  as  follows  : 

The  branches  of  the  visceral  nerves  and  of  the  general  sympathetic 
nervous  system  enter  the  spinal  cord  at  various  It^vels  throughout  its 
entire  length.  Irritation  sent  into  the  spinal  cord  through  these  nerves 
set  up  sensory  impulses  in  the  various  segments  of  the  cord,  each  seg- 
ment receiving  impulses  from  a  certain  organ.  These  sensory  impulses 
are  sent  upward  to  the  brain,  and  become  conscious  perceptions. 
They  are  referred  by  consciousness  not  to  their  actual  point  of  origin, 
but  to  the  part  of  the  body  from  which  sensations  usually  come  when 
received  at  the  particular  segment  irritated.  Thus,  as  in  general  ex- 
perience, sensations  and  pains  coming  from  the  various  segments  of 
the  spinal  cord  have  been  due  to  irritation  in  the  surface  of  the  body 
corresponding  to  these  segments ;  these  various  visceral  sensations  are 
referred  to  the  surface  of  the  body.  Some  examples  of  such  referred 
pains  will  make  this  matter  clearer. 

It  is  not  at  all  uncommon  in  eyestrain  to  have  a  pain  felt  in  the  fore- 
head or  in  the  back  of  the  neck,  neither  of  which  parts  is  in  direct 
connection  with  the  eye. 

The  pain  produced  by  decayed  teeth  may  be  felt  in  the  temple 
or  behind  the  ear,  instead  of  in  the  jaw. 

Severe  pain  in  the  back  of  the  head  is  a  common  symptom  of  uterine 
disease  or  of  inflammation  of  the  bladder. 

Pain  down  the  left  arm  is  a  common  symptom  of  heart  disease,  and 
may  be  attended  by  hyperaesthesia  in  the  region  of  the  fourth  and  fifth 
dorsal  nerves  on- the  chest. 

Pain  in  the  wrist  on  the  flexor  surface  is  frequently  felt  in  disease 
of  the  uterus,  ovaries,  or  bladder. 

Pain  under  the  right  shoulder-blade  is  frequently  felt  in  disease  of 
the  liver,  and  is  often  attended  by  hyperaesthesia  in  the  domain  of  the 
eighth  to  the  twelfth  dorsal  nerve. 

Pain  under  the  left  shoulder-blade  is  common  in  enlargement  of  the 
spleen. 

Pain  between  the  shoulder-blades  is  a  very  common  symptom  of 
gastric  affections  of  any  kind.  It  may  be  attended  by  hypersesthesia 
in  the  epigastric  region,  and  the  nearer  the  disease  to  the  cardiac  end 
of  the  stomach  —  e.  g.,  ulcer  —  the  higher  the  pain  is  felt.  In  severe 
vomiting  pain  may  be  felt  on  the  back  of  the  arms  or  even  down  the 
back  of  the  forearms. 

Pain  across  the  small  of  the  back  is  common  in  colitis  or  in  impac- 
tion of  feces  within  the  colon. 

Pain  across  the  upper  sacral  region  is  very  common  in  uterine 
disease. 


752 


NEURALGIA. 


Pain  over  the  outer  side  of  the  hip  is  usually  due  to  ovarian  con- 
gestion. 

Pain  down  the  inner  side  of  the  les;  is  also  due  to  the  same  cause. 


Fig.  299. 


J'/Dtcnyed  teeth 


Neurasthenia 
(Bpinal  irritation ) 


m/nsmia 

^Neurasthenia 

Ovaries? 


The  location  of  referred  paius  and  their  cause.     (Dana. ) 


Area.     Cerebrospinal 
nerves. 


Distribution. 


Associated  ganglia  of 
sympathetic. 


Distribution, 

I.    Trigeminus,  facial.    Face  and  anterior  scalp.  4  cerebral.                           Head. 

II.    Upper  4  cervical.        Occiput,  neck.  1st  cervical.                         Head,  ear. 

III.    Lower  4  cervical        Upper  extremity.  2d  and  3d  cervical,  1st      Heart. 

and  1st  dorsal.  dorsal. 

IV.     Upper  6  dorsal.            Thorax.  1st  to  6th  dorsal. 

V.    Lower  6  dorsal.           Abdomen,  upper  lum-  6th  to  12th  dorsal, 
bar. 

VI.    12th  dorsal  and  4      Lumbar  region,  upper  1st  to  5th  lumbar, 
lumbar.                        gluteal,  anterior  and 
inner  thigh  and  knee. 

VII.    5th  lumbar  and  5      Lower  gluteal,  posterior  1st  to  5th  sacral, 
sacral.                          thigh  and  leg. 


Lungs. 

Viscera  of  abdomen  and 

testes. 
Pelvic  organs. 


Pelvic  organs  and  legs. 


Pain  on  the  inner  side  of  the  knee  is  au  early  symptom  of  hip-joint 
disease. 

Pain  in  the  heel  is  a  frequent  symptom  in  lithaemia,  and  may  also 
be  felt  in  ovarian  disease. 

Dana^  has  studied  the  location  of  these  reflex  or  referred  pains 
'  New  York  Medical  Journal,  July  23,  1887. 


REFERRED  PAINS. 


753 


very  carefully,  and  his  figures  (Fig.  299),  which  are  here  reproduced, 
demonstrate  the  areas  of  pain  of  sympathetic  origin. 

Head  ^  and  Mackenzie  ^  have  added  a  few  facts  to  Dana's  statements. 
Head  has  called  attention  to  the  fact  that  when  a  viscus  is  diseased 
there  may  be  detected  by  examination  with  a  sharp  instrument  an  area 
of  hyperalgesia  on  the  skin  of  the  back  and  abdomen,  each  viscus 


Fig.  300. 


Gall  Bladder 


Caecum  and 
Appendix 

Ovary  and  Tube 


^  /  Stomach 
(Left  Half) 


Kidney 


Ureter 


The  general  location  and  outline  of  the  zones  of  cutaneous  hyperalgesia  for  some  of  the  abdominal 
viscera.  Anterior  view  The  maxima  are  deeply  shaded.  Only  the  left  half  of  the  gastric  zone  is 
given.    The  ureteral  zone  consists  of  a  series  of  maxima  (diagrammatic).      (Elsberg  and  Neuhof. ) 

having  its  particular  area.  Figs.  300  and  301  show  the  areas  related 
to  the  various  viscera,  as  recently  determined  by  Elsberg  and  Neuhof,^ 
whose  results  confirm  Head's  statements. 

There  is  hardly  any  viscus  in  which  disease  may  not  give  rise  to 
some  of  these  referred  pains,  and  it  is  evident  that  without  some 
knowledge  of  the  localities  of  these  referred  pains  the  presence  of  such 
pain  might  mislead  the  physician.  I  have  had  many  patients  brought 
to  me  for  supposed  spinal  disease  because  of  pain  in  the  dorsal  region 
and  tenderness  of  the  dorsal  spine,  with  pain  in  the  epigastrium,  gen- 
eral weakness  and  neurasthenic  conditions,  and  increased  knee-jerks, 
who  have  been  relieved  by  the  correction  of  a  chronic  gastric  dyspepsia. 

1  Brain,  1893,  1894,  1890.  ==  Brain,  1902. 

4g  *Araer.  .Jour.  Med,  8ci.,  Nov.,  1908, 


754 


NEURALGIA. 


It  is  evident  that  in  these  referred  pains  the  treatment  must  be 
directed  to  relief  of  the  functional  or  organic  disease  in  the  viscus 
which  is  affected.      It  may  be  added  that  not  infrequently  sharp  counter- 

FiG.  301. 


lOth  Dorsal  Spine 


-1st  Sacral 


The  general  location  and  outline  of  the  posterior  parts  of  the  zones  (diagrammatic). 

irritation  over  the  seat  of  pain  is  followed  not  only  by  relief  of  the  pain, 
but  also  by  improvement  in  the  visceral  condition  which  is  its  cause. 
It  is  also  to  be  remembered  that  aneurism  of  the  aorta  and  tumors 
of  the  thorax  or  abdomen  often  cause  pain  referred  to  the  thorax  or 
abdomen  at  a  distance  from  the  origin  of  the  pain. 


CHAPTER  XLIII. 


HEADACHE  AND  MIGRAINE. 


I.     HEADACHE. 

Headache  is  a  symptom  of  very  common  occurrence  and  its  cause 
may  be  quite  difficult  to  determine  in  any  particular  case.  Its  variety 
in  intensity,  duration  and  location  is  extreme. 

Etiology.  —  Its  causes  may  be  classified  as  follows: 
Organic.     Injuries  of  the  head  and  fractures. 
Meningitis,  general  or  localized. 
Meningeal  hemorrhage. 
Tumor  or  abscess  of  the  brain. 
Arterial  changes  preceding  apo])lexy. 
Syphilis  of  the  meninges  or  brain. 
Toxic.         The  infectious  diseases. 

Autointoxication  from  disease  of 
the  kidneys, 
the  liver, 

the  stomach  and  intestines, 
the  ductless  glands. 
Intoxication  by  alcohol  and  drugs, 

by  lead  and  other  poisons, 
by  tobacco, 

by  bad  air  in  close  places. 
From  impaired  blood  states  — 
Anaemia, 

Rheumatism  and  gout, 
Diabetes, 

Malignant  disease, 
Hodgkin's  disease. 
Reflex  from  disease  of  eyes  and  ears, 
nose  and  pharynx, 
teeth, 

viscera,  uterus  and  ovaries. 
Neuralgia. 

Neurasthenia  and  the  traumatic  neuroses. 
Psych  asthenia. 
Hysteria. 
Fatigue. 

Climatic  changes. 

Symptoms.  —  The  characteristics  of  some  of  these  forms  of  headache 
require  special  attention, 

755 


756  HEADACHE   AND   MIGBAINE. 

Headache  from  organic  disease  is  attended  by  so  many  other  symp- 
toms which  point  to  the  diagnosis  that  it  needs  no  special  description, 
especially  as  it  has  been  already  discussed  in  connection  with  brain 
tumors  (page  584),  abscess  (page  553),  meningitis  (page  717),  arte- 
rial sclerosis  (page  469),  syphilis  (page  691). 

Headache  from  toxic  causes  presents  many  varieties.  All  the  infec- 
tious diseases  begin  with  fever  and  this  is  usually  ushered  in  or  accom- 
panied by  headache  of  a  general  dull  intense  kind  increased  by  any 
effort  of  mind  or  muscle.  The  degree  of  the  pain  is  proportionate  to 
the  height  of  the  temperature  and  to  the  intensity  of  the  infection.  In 
many  fevers,  notably  typhoid  and  typhus,  the  headache  remains  for  a 
long  time,  but  in  the  children's  infections  it  subsides  after  a  few  days. 
Malarial  headaches  are  very  common  and  may  be  of  any  degree  of 
intensity  and  in  various  positions.  The  particular  characteristic  is  its 
tendency  to  come  on  suddenly ;  to  recur  at  a  definite  time  of  the  day, 
either  daily  or  every  other  day,  or  once  a  week,  and  to  cease  as  sud- 
denly as  it  came  after  a  few  hours.  Such  headaches  may  be  the  only 
evidence  of  malarial  infection ;  though  an  examination  of  the  blood 
will  establish  the  diagnosis.  The  remedies  are  a  systematic  course 
of  treatment  by  quinine,  Warburg's  tincture  or  arsenic. 

In  nephritis  the  headache  is  due  to  the  ursemic  state  and  may  be  the 
first  symptom  of  that  condition.  If  very  intense  it  may  presage  a  con- 
vulsion, as  in  the  puerperal  state.  If  dull  and  continuous  it  may  show 
a  chronic  uraemia  or  the  state  of  arterial  sclerosis  to  which  the  nephri- 
tis is  due.     It  is  then  a  pulsating  headache  made  worse  by  efforts. 

Jaundice  is  sometimes  attended  by  headache,  and  chronic  cirrhosis 
of  the  liver  even  without  jaundice  is  liable  to  cause  it,  as  it  interferes 
with  the  chemistry  of  digestion.  Such  a  headache  is  not  intense,  is 
rather  dull  and  felt  all  over  the  head. 

Gastric  and  intestinal  intoxication  is  probably  the  most  common 
cause  of  all  the  forms  of  headache.  In  dyspepsia  the  patient  feels  a 
dull,  disagreeable  feeling  over  the  eyebrows  which  varies  from  hour  to 
hour,  being  usually  relieved  by  eating  and  then  returning  with  renewed 
intensity  about  an  hour  after  food.  It  is  particularly  liable  to  be 
severe  on  waking. 

In  intestinal  toxaemia  the  pain  is  usually  felt  over  the  entire  fore- 
head, is  dull  and  often  throbbing  in  character  and  is  increased  by 
motions  of  the  bead  or  by  muscular  effort.  It  is  often  associated  with 
pains  in  and  back  of  the  eyes.  Much  relief  is  afforded  in  these  cases 
by  high  irrigations  as  well  as  by  brisk  laxatives,  especially  aloes. 

In  auto-intoxication  by  errors  in  the  ductless  glands,  in  myxoedema 
and  Basedow's  disease  the  headache  may  be  very  intense  at  times,  but 
varies  from  day  to  day,  and  is  not  constant. 

Intoxication  by  alcohol  and  drugs  causes  very  intense  generalized 
pain,  which  persists  until  elimination  is  secured.  It  is  dull  and  goes 
away  very  slowly  and  is  attended  by  mental  apathy,  especially  if  the 
drug  is  opium. 

Lead  causes  very  severe  frontal  pain,  as  does  also  mercury.  Arsenic 
and  copper  do  not  often  cause  headache. 


HEADACHE.  757 

A  few  nprsons  are  affected  by  tobacco  unfavorably  and  after  long- 
continued  inhalation  of  tobacco  smoke  in  a  heavily  laden  atmosphere 
headache  is  not  uncommon. 

The  headache  which  follows  confinement  in  the  close  atmosphere  of 
a  badly  ventilated  hall,  theatre  or  church  is  familiar  to  everyone, 
though  the  exact  nature  of  the  poison  producing  it  is  unknown.  It 
disappears  in  an  hour  or  less  after  deep  inspirations  of  fresh  air. 

Certain  states  of  the  blood  cause  intense  headache.  Ansemia  is  the 
one  most  commonly  observed,  especially  in  young  women.  The  pain 
is  felt  chiefly  on  the  top  of  the  head,  but  may  also  be  over  or  behind 
the  eyes  and  sometimes  is  felt  as  a  band  about  the  head.  It  is  usually 
attended  by  constipation.  Sir  Andrew  Clark  used  to  say  that  he 
preferred  aloes  to  iron  in  the  treatment  of  ansemia  in  women. 

Rheumatic  and  gouty  affections  of  the  scalp  are  a  frequent  cause  of 
pain  in  the  head  and  are  attended  by  tenderness  of  the  scalp  especially 
at  the  forehead  and  occiput.  Sometimes  rheumatic  deposits  can  be 
felt  in  the  fascia  over  the  occiput.  They  may  usually  be  relieved 
promptly  by  a  shampoo  with  hot  water  followed  by  through  massage 
of  the  scalp. 

The  term  reflex  headache  has  been  used  to  indicate  a  pain  referred 
to  the  head  although  due  to  an  irritation  of  some  nervous  mechanism 
or  some  branch  of  the  trigeminal  nerve  by  processes  going  on  else- 
where.    (See  also  Chapters  XLII.  and  LVI.) 

The  use  of  the  eyes  when  there  is  difficulty  in  the  focus  from  astig- 
matism or  asthenopia  or  when  there  is  defective  action  of  the  external 
muscles  is  liable  to  cause  much  irritation,  and  produces  pain  not  only 
in  the  eyes  but  in  the  forehead  and  often  in  the  back  of  the  neck  and 
occipital  region.  Such  pain  is  usually  felt  after  a  use  of  the  eyes 
either  for  far  vision  as  in  looking  at  pictures  in  a  gallery  or  at  the 
stage  in  a  theatre,  or  else  in  near  vision  as  in  reading.  If  the  cor- 
rection of  the  astigmatism  or  of  near  or  far  vision  by  glasses  fails  to 
relieve  this  headache  it  is  well  to  examine  the  condition  of  the  ocular 
muscles  as  an  insufficiency  of  the  internal  or  external  recti,  as  the  case 
may  be,  is  often  a  cause  for  intense  headache.  Such  insufficiency  can 
usually  be  relieved  by  the  use  of  prisms  and  division  or  advancement 
of  the  muscles  is  rarely  necessary. 

Severe  headache,  especially  in  the  temporal  region,  is  occasionally 
due  to  an  irritation  in  the  internal  ear  and  the  condition  of  the  ears 
should  always  be  examined  in  a  case  of  unilateral  headache.  Such 
headaches  are  often  attended  by  vertigo.     See  Chap.  LIII. 

Reflex  headaches  from  irritation  in  the  nose  and  pharynx  are  occa- 
sionally felt  in  the  forehead  and  between  the  eyes.  Children  with  ade- 
noids often  complain  of  headache  in  this  region. 

Pain  in  the  head  as  the  result  of  defects  in  the  teeth  is  sometimes 
felt  in  the  temporal  region,  sometimes  in  the  occipital  region.  Lauder 
Brunton  has  called  attention  to  this  form  of  headache  and  believes  that 
decayed  molar  teeth  in  the  upper  jaw  cause  temporal  headache  which 
is  rather  farther  forward  than  that  caused  by  decayed  molars  in  the 


758  HEADACHE   AND    MIGEAINE. 

lower  jaw.  Diseased  molars  in  the  lower  jaw  may  also  give  rise  to 
occipital  headache,  while  decayed  incisors  or  eye  teeth  are  more  likely 
to  cause  frontal  or  vertical  headache. 

A  headache  is  also  frequently  complained  of  in  diseases  of  the  ovaries 
and  uterus,  and  is  usually  located  in  the  back  of  the  head  just  below 
the  occipital  protuberance.  It  is  often  relieved  by  lying  down  or  by 
lying  on  the  stomach,  and  appears  to  have  some  relation  to  traction 
upon  the  ligaments  of  the  uterus  and  ovaries,  so  that  rest  in  bed  as  a 
rule  relieves  this  form  of  headache.  There  are  numerous  cases  re- 
ported where  a  proper  adjustment  of  the  pessary  holding  the  uterus  in 
place  has  relieved  immediately  this  form  of  headache,  which  has  how- 
ever returned  when  the  pessary  was  removed. 

Neuralgic  headache  is  fully  discussed  under  the  chapter  on  Neuralgia 
of  the  Trigeminal  Nerve. 

The  headache  of  neurasthenia  and  of  the  traumatic  neurosis  is  fully 
described  on  page  841. 

Psychasthenic  headache  has  been  alluded  to  on  page  867.  It  is 
really  a  form  of  delusion  of  pain,  and  the  characteristic  of  this  form  of 
headache  is  its  long  duration.  When  a  patient  complains  that  he  or 
she  has  had  a  headache  constantly  for  months  or  years  without  very 
great  variation  in  the  degree  of  the  pain  and  without  any  evidence  of 
the  malnutrition  which  accompanies  severe  continued  organic  pain,  the 
suspicion  should  be  awakened  that  the  pain  is  of  a  delusional  nature 
or  a  subconscious  pain.  The  reader  is  referred  to  the  discussion  of  this 
subject  on  page  870.  The  only  treatment  that  is  successful  for  this 
type  of  headache  is  hypnotic  suggestion. 

The  headache  of  hysterical  conditions  differs  from  that  of  psychas- 
thenia  in  being  sudden  in  its  onset  and  agonizing  in  its  character.  It 
may  be  felt  at  the  base  of  the  brain  behind  the  eyes ;  it  may  be  felt  as 
a  sharp  deep  agonized  pain  at  the  occiput,  or  it  may  be  felt  on  the  ver- 
tex. Patients  will  complain  that  they  feel  as  though  a  nail  were  being 
driven  through  the  head  on  top  or  at  the  back.  Others  will  complain 
that  they  feel  as  though  a  band  were  being  tied  tight  about  the  head. 
Such  patients  complain  most  bitterly  of  the  headache,  and  until  the 
hysterical  paroxysm  is  over  very  little  relief  is  afforded  by  ordinary 
remedies.  Even  hypodermic  injections  of  morphine,  J  to  |  grain 
seen  to  have  no  effect  upon  this  type  of  headache,  though  sometimes  very 
minute  doses  of  ignatia,  nux  vomica,  strychnine  or  hyoscyamus  repeated 
every  5  minutes  until  ten  doses  are  given  may  relieve  this  form  of  head- 
ache. Hot  applications  to  the  feet,  mustard  foot  baths,  ice  to  the  head 
or  to  the  back  of  the  neck,  or  a  small  blister  to  the  back  of  the  neck, 
may  also  be  tried. 

Another  type  of  hysterical  headache  is  a  dull  continuous  frontal  pain 
which  is  increased  by  any  action  of  the  mind  or  effort  of  the  attention. 
This  resembles  the  psychasthenic  headache  in  being  more  or  less 
constant,  and  probably  has  the  same  origin  in  a  subconscious  state  with 
suggestion. 

The  headache  of  fatigue  is  a  dull  pain   felt  over  the  forehead  near 


HEADACEE.  759 

the  line  of  the  hair  and  back  of  the  eyes.  It  is  more  liable  to  come 
on  after  fatigue  of  the  mind  in  reading,  studying  or  intense  application 
of  any  kind  and  it  is  only  to  be  relieved  by  rest. 

Climatic  changes  not  infrequently  set  up  a  dull  continuous  headache. 
Thus  persons  who  are  obliged  to  live  in  a  very  warm  climate  or  in  a 
damp  climate  such  as  officials  in  the  Philippines  or  travellers  in  the 
tropics  are  very  liable  to  suffer  from  this  form  of  headache.  Those 
who  climb  high  mountains  or  who  go  to  high  altitudes  to  reside  in  the 
summer,  as  in  Switzerland  or  among  the  Rocky  Mountains,  not  infre- 
quently suifer  from  headaches  due  to  the  elevation.  Foreigners  coming 
to  America  appreciate  the  unusual  stimulus  of  our  climate  and  not 
infrequently  suifer  from  chronic  headaches  while  they  remain  here. 
All  such  headaches  if  they  require  treatment  are  best  treated  by  seda- 
tives of  the  nature  of  bromide,  phenacetine,  and  the  coal  tar  products. 

Treatment. — The  treatment  of  headache  will  be  determined  en- 
tirely by  the  cause  of  the  condition  and  when  this  can  be  ascertained 
and  removed  relief  can  be  afforded.  In  some  cases  however  the  cause 
cannot  be  removed,  as  in  organic  diseases  and  in  many  forms  of 
toxaemia.  In  such  cases  it  is  necessary  to  resort  to  symptomatic 
remedies.  Of  these  the  coal  tar  products  are  the  most  efficacious, 
phenacetine,  acetanilide,  aspirin  and  pyramidon  being  the  best.  As 
all  of  these  drugs  are  more  or  less  depressing  to  the  circulation  it  is 
well  to  combine  them  with  a  mild  heart  stimulant,  of  which  caffeine, 
ignatia,  or  brucia  may  be  selected.  It  has  also  been  found  that  if 
they  are  given  very  finely  triturated  they  act  much  more  promptly. 
And  further  the  absorption  in  the  stomach  seems  to  be  aided  by  the 
use  of  an  alkali,  hence  a  small  amount  of  bicarbonate  of  soda  should 
be  added  to  the  powder  given.     The  following : 

R       Phenacetine.  gr.  x 

Caffeine  Citratis.  gr.  j 

Ignatia.  gr.  jhr 

bod.  Ijicarb.  gr.  iij 

M.     Triturate. 
Sig.     Tal  pulv.  q.  3  hr.  with  a  glass  of  water. 

is  a  very  good  symptomatic  remedy  for  headache,  though  any  of  the 
coal  tar  products  may  be  substituted  for  the  phenacetine.  Many  of 
the  headache  powders  sold  by  druggists  and  many  of  the  proprietary 
remedies  widely  advertised  have  been  found  to  consist  of  similar  com- 
binations. This  combination  is  the  one  in  some  tablets  sold.  It 
should  be  remembered  that  tablets  act  much  more  promptly  if  chewed 
up  and  swallowed  well  diluted  with  water. 

Another  symptomatic  remedy  for  headache  is  bromide  in  20  or  30 
grain  dose.  This  is  particularly  indicated  in  headache  from  fatigue, 
from  overwork  and  anxiety  and  from  cerebral  congestion  due  to  any 
cause.  It  is  also  of  service  in  the  infectious  forms  of  headache  and  in 
some  of  the  toxic  states. 

Some  forms  of  headache  are  so  intense  as  to  require  the  use  of  opium. 
Codeine  may  be  added  to  the  powder  already  described  in  ^j^  or  |  grain 


760  HEADACHE   AND    MIGRAINE. 

dose  ;  or  may  be  given  alone.  In  very  intense  pain  hypodermics  of 
morphine  are  needed  as  in  the  pain  of  typhoid  fever  or  of  brain  tumor 
or  meningitis. 

MIGRAINE. 

Migraine,  hemicrania,  or  sick  headache,  is  a  functional  neurosis 
characterized  by  sudden  attacks  of  intense  pain  in  one  side  of  the 
head,  often  preceded  by  evidence  of  irritation  of  various  functions  of 
the  brain  and  followed  by  digestive  disturbances. 

Etiology.  —  Heredity  plays  an  important  part  in  the  development 
of  this  disease.  In  almost  all  cases  the  history  can  be  obtained  of  a 
similar  aifection  in  some  near  member  of  the  family.  The  disease 
develops  at  an  early  age.  Infants  are  rarely  affected,  but  children 
of  the  age  of  seven  or  eight  may  develop  it,  ^nd  as  a  rule  it  remains 
as  a  chronic  condition  throughout  life.  If  the  disease  does  not  develop 
in  childhood,  it  commonly  develops  at  puberty.  There  are  no  known 
predisposing  factors  to  this  disease,  and  it  appears  to  be  due  to  a  con- 
genital defect  in  the  chemistry  of  nutrition,  rather  than  to  any  extra- 
neous cause.  In-door  occupations,  or  a  sedentary  life,  tend  to  increase 
the  liability  in  a  person  predisposed,  and  it  is  a  well-known  fact  that 
those  who  work  in  the  open  air,  or  are  engaged  in  occupations  involv- 
ing considerable  vigorous  exercise,  are  never  affected.  The  active 
cause  of  the  attack  is  undoubtedly  a  sudden  development  in  the  sys- 
tem of  a  poison.  This  poison  at  first  produces  an  excitement  and 
stimulation  of  the  brain  causing  a  sense  of  well-being,  of  increased 
capacity  and  of  exhilaration.  This  is  soon  followed  by  a  secondary 
and  more  lasting  effect  of  pain  and  suspension  of  the  cerebral  func- 
tions, and,  finally,  there  is  an  elimination  of  the  poison  through  the 
stomach,  intestines,  or  kidneys,  and  a  return  to  a  normal  state  of 
health.  The  nature  of  this  poison  is  still  undetermined.  Some  have 
considered  it  uric  acid ;  others  have  thought  it  was  a  leukomain ;  others 
have  considered  it  xanthin.  Although  the  manifestations  are  chiefly 
in  the  domain  of  the  nervous  system,  it  is  generally  admitted  that  this 
disease  is  one  of  nutrition.  In  some  cases  it  has  been  thought  that  an 
insufficiency  of  the  ocular  muscles  has  been  the  cause  of  the  affection ; 
an  insufficiency  of  the  interni  resulting  in  a  constant  strain  when  con- 
vergence in  reading  is  necessary,  and  an  insufficiency  of  the  externi 
leading  to  a  strain  when  distant  vision  is  practiced.  On  this  theory 
attempts  have  been  made  to  cure  the  disease  by  the  use  of  prisms,  by 
division  of  the  ocular  muscles,  or  by  the  use,  in  the  case  of  insuffi- 
ciency of  the  interni,  of  strychnin,  and  in  insufficiency  of  the  externi, 
of  cannibis  indica.  While  it  is  possible  that  irritation  from  the  eyes 
may  act  as  an  excitant  of  the  nervous  system,  and  then  make  it  more 
liable  to  be  affected  by  the  poison  of  the  disease,  it  seems  improbable 
that  these  causes  are  sufficient  to  produce  the  affection. 

There  are  some  who  believe  that  migraine  like  epilepsy  is  a  func- 
tional neurosis  of  the  cerebral  cortex  and  that  the  attacks  are  not  of 


MiGBAINE.  761 

toxic  origin.  Gowers  ^  relates  cases  in  which  the  two  diseases  alternate 
—  and  otfier  cases  in  which  one  has  succeeded  the  other.  He  cites 
the  manifest  irritation  of  certain  cortical  areas  at  the  onset  of  the  attack, 
and  the  exhaustion  of  cortical  activity  at  its  conclusion  as  evidence. 
I  am  inclined  to  regard  the  cortical  symptoms  as  secondary  to  the 
toxaemia. 

Symptoms.  —  An  attack  of  migraine  begins  with  a  prodromal  period 
of  excitement  in  about  one-half  of  the  cases,  and  this  period  lasts 
several  hours.  During  it  the  patients  feel  particularly  well  and  active. 
They  are  bright  mentally,  happy,  and  slightly  exhilarated,  and  are 
likely,  during  this  period,  to  over-exert  themselves,  or  over-tire  them- 
selves, in  whatever  line  of  occupation  they  may  be  interested.  The 
attack  begins  with  a  sudden,  intense  boring  pain  in  the  temple  and  at 
the  back  of  the  head,  or  over  the  entire  side  of  the  head,  attended  by 
a  sense  of  complete  prostration,  inability  to  think,  hypersensitiveness 
to  sound,  light,  and  touch,  so  that  the  patient  prefers  to  lie  in  bed  in  a 
quiet,  dark  room,  wishes  to  be  spoken  to  in  whispers,  and  is  annoyed 
if  the  bed  be  touched  or  shaken.  There  is  also  a  hypersensitiveness 
to  smell  and  taste.  Appetite  is  wholly  wanting,  and  nausea  soon 
develops,  which,  as  a  rule,  is  followed  by  intense  and  frequent  attacks 
of  vomiting.  The  vomited  material  is  first  the  contents  of  the  stomach, 
which  are  usually  excessively  acid,  and  then  bile  mixed  with  acid 
mucus.  The  vomiting  causes  intense  prostration,  a  very  weak,  rapid 
pulse,  and  very  often  pain  between  the  shoulder  blades  and  down  the 
arms.  Any  food  taken  into  the  stomach  is  immediately  rejected. 
Occasionally  attacks  of  diarrhoea  coincide  with  the  vomiting.  As  a 
rule,  very  little  urine  is  passed  during  the  attack,  and  this  is  of  high 
specific  gravity,  dark  in  color,  and  is  loaded  with  uric  acid,  skatol  and 
phosphates.  Xanthin  and  leukomains  have  also  been  found  in  it. 
There  is  usually  a  very  great  increase  in  the  excretion  of  phosphates. 
If  the  attack  comes  on  in  the  daytime,  the  patient  is  immediately  forced 
to  go  to  bed  by  the  intense  pain  and  prostration.  Many  patients  awake 
in  the  morning  from  a  deep  sleep  suffering  from  an  attack,  and  are 
unable  to  rise.  The  attack  lasts  from  twelve  to  seventy-two  hours. 
During  the  attack  the  patient,  as  a  rule,  feels  cold,  and  heat  is  always 
agreeable,  both  to  the  head  and  to  the  body.  In  the  majority  of  cases 
there  is  a  pallor  of  the  surface.  The  nose  and  extremities  are  very 
cold  and  dry,  though  occasionally  there  is  an  acid  perspiration  making 
them  cold  and  clammy.  Occasionally  the  face  is  much  flushed,  espe- 
cially on  the  side  of  the  pain. 

In  some  cases  the  attack  is  preceded,  or  ushered  in,  by  the  appear- 
ance of  a  bright  spot  or  star  on  one  side  of  both  eyes  of  any  color,  or 
flashes  of  light,  or  zigzag  lines.  These  are  true  hallucinations  of  vision 
and  they  are  not  stopped  by  closing  of  the  eyes  ;  as  they  are  hemiopic 
in  their  distribution  they  indicate  irritation  of  the  opposite  occipital 
lobe.     They  may  be  attended  by  temporary  blindness  which  subsides 

^  Clinical  lectures  on  the  Borderland  of  Epilepsy.  Migraine.  Brit.  Med.  Jour., 
Dec.  8,  1906. 


762  HEADACBE   AND   MIOHAINE. 

with  the  pain  ;  such  cases  have  been  named  ophthalmic  migraine.  In 
a  few  cases  temporary  paralysis  of  the  third  nerve  has  been  seen. 
Occasionally  ringing  in  the  ears,  or  the  sound  of  bells,  or  the  running 
of  a  tune  in  the  head,  precedes  or  accompanies  an  attack.  Occasionally 
tingling  and  numbness  of  a  disagreeable  kind  are  felt  in  the  side  of  the 
body  opposite  to  the  side  of  the  pain.  Occasionally  attacks  of  aphasia 
occur  before,  or  in  connection  with  the  attack,  and  the  patient  is  unable 
to  express  his  desires  and  misplaces  words  and  is  reluctant  to  talk, 
although  the  power  of  talking  is  never  suspended.  Occasionally  a 
sense  of  great  mental  confusion  and  inability  to  think  is  appreciated 
during  an  attack.  Oppenheim  has  seen  a  case  associated  with  vertigo 
and  staggering  gait  which  he  considers  of  cerebellar  origin.  In  the 
description  of  the  aura  in  epilepsy  (see  page  814)  very  similar  sen- 
sations will  be  described  as  occurring  in  petit  mal  and  as  the  first 
symptoms  of  grand  mal.  Here  again  therefore  Gowers  finds  a  proof 
of  some  relation  between  the  two  diseases.  In  epilepsy  the  aura  is 
only  momentary  in  duration,  while  in  migraine  these  sensations  remain 
for  twenty  minutes  or  longer.  All  these  symptoms  indicate  distinctly 
an  irritation  of  the  cortex  of  the  brain,  one  hemisphere  only  being 
affected,  namely,  the  hemisphere  on  the  side  on  which  pain  is  felt. 
For  this  reason,  in  some  cases,  it  has  been  supposed  that  there  is  a  con- 
gestion of  the  hemisphere  and  in  other  cases  an  anaemia  of  the  hemis- 
phere, and  on  this  theory  ergot  and  nitroglycerin  have  been  given,  but 
are  of  little  avail.  During  the  attack  of  migraine  the  patient  is  ex- 
tremely somnolent  and  sleeps  for  several  hours  or  even  for  two  days, 
being  however  easily  aroused.  It  is  not  uncommon  for  the  patient  to 
be  slightly  delirious  when  aroused  from  this  sleep  and  to  have  no  recol- 
lection of  the  delirium  when  the  attack  is  over.  The  attack  terminates 
gradually  as  a  rule,  the  pain  passing  off.  A  very  large  flow  of  urine 
terminates  it  and  in  this  urine  an  excess  of  uric  acid,  skatol,  xanthin, 
or  leukomains  have  been  found.  The  patient  feels  much  prostrated 
for  a  day  or  two  and  then  returns  to  his  or  her  normal  state  of  health. 
The  attacks  recur  with  varying  frequency  in  different  patients.  Some 
women  have  these  attacks  uniformly  once  a  month,  coinciding  with  the 
period.  Many  have  attacks  as  often  as  once  in  two  weeks  and  they 
may  increase  in  frequency  until  the  patient  has  one  every  few  days, 
making  the  life  one  of  invalidism.  The  attacks  usually  subside  about 
the  age  of  fifty,  and  old  people  are  rarely  subject  to  them.  In  women 
they  usually  cease  after  the  menopause,  and  I  have  known  severe 
attacks  to  cease  after  ovariotomy.     They  often  cease  during  pregnancy. 

Prognosis.  —  The  prognosis  for  recovery  from  the  disease  is  not  a 
good  one,  as  attacks  are  liable  to  recur  from  time  to  time  until  the  age 
of  fifty,  unless  the  habit  of  life  can  be  radically  changed.  There  is  no 
danger  of  death  in  an  attack,  even  though  it  is  a  very  severe  one,  and 
although  the  symptoms  of  prostration  may  at  times  seen  alarming. 

Treatment.  —  This  consists  of  a  general  regime  and  treatment  of 
the  underlying  digestive  condition  in  the  interval  between  attacks  and 
in  the  treatment  of  the  attack  itself. 


MIGBAINE.  763 

The  genpral  regime.  Diet.  —  A  simple  but  nutritious  diet  of  small 
amount  is  to  be  followed  rigidly.  The  tendency  to  over-eat  should 
be  guarded  against.  Fruit  (except  strawberries),  cereals,  eggs,  fish, 
white  meats,  ham  and  bacon,  game,  vegetables  of  almost  all  kinds, 
custards,  simple  desserts  made  without  pastry  or  sugar  may  be  allowed. 
Rich  sauces  are  to  be  avoided  and  also  things  which  are  fried.  Eggs 
disagree  with  many  of  these  patients  and  so  does  milk.  Red  meat 
may  be  allowed  every  other  day  in  some  form,  but  in  some  patients 
has  to  be  excluded,  though  in  these  individuals  ansemia  is  to  be  pro- 
vided against.  Tomatoes,  asparagus,  eggplant,  cauliflower,  and  salads 
made  with  vinegar,  are  often  found  to  disagree,  and  if  so  may  be 
excluded.  Cheese,  especially  the  French  cheeses,  are  to  be  excluded. 
Sugar  is  to  be  used  very  sparingly  and  all  candy  is  to  be  forbidden. 
Patients  vary  in  susceptibility  to  tea  and  coffee.  In  some  they  are 
injurious.  In  some  a  strong  cup  of  either  will  abort  an  attack. 
Alcohol  in  any  form  must  be  forbidden.  Tobacco  may  be  used  in 
moderation.  Patients  subject  to  migraine  should  drink  at  least  two 
quarts  of  water  daily,  but  this  should  not  be  taken  with  meals.  I 
have  seen  much  benefit  from  the  use  of  piperazine  water.  Any  alka- 
line water  may  prove  useful. 

Exercise  in  the  open  air,  sufficiently  active  to  cause  a  perspiration, 
is  absolutely  essential.  Tennis,  horse-back  riding,  rowing,  and  golf 
are  the  best  forms  of  exercise  and  should  be  persisted  in  daily  both  in 
summer  and  winter. 

Baths  are  of  much  use;  either  a  Turkish  bath  once  a  week;  or 
a  hot  vapor  bath  followed  by  a  cool  sponge  off,  or  a  hot  bath  106°, 
made  alkaline  by  the  addition  of  two  handfuls  of  carbonate  of 
soda,  of  ten  minutes  duration  daily,  followed  by  a  cool  sponge  off. 
The  object  of  the  bath  is  to  stimulate  the  cutaneous  circulation  and 
secure  free  perspiration.  It  may  be  taken  on  rising  or  on  going  to 
bed,  if  it  does  not  prevent  sleep.  Massage  is  also  of  much  service, 
especially  deep  and  forcible  massage,  not  only  of  the  limbs,  but  also 
of  the  back  and  of  the  abdominal  viscera.  Swedish  movements  may 
be  combined  with  massage. 

Treatment  directed  to  the  digestive  functions  is  usually  of  much 
benefit.     A  saline  laxative  of  which  the  best  is  the  following : 

B      Sod.  Phosphatis  exsic.  5  iv 

Sod.  Sulphatis  exsic.  5  x 

Sod.  Salicylatis.  5  ij 

M.     Triturate  and  cork  tightly. 
Sig.     One  teaspoonful  in  a  large  tumbler  of  hot  or  cold  seltzer  water  on  rising 

is  to  be  given  daily.  Once  in  two  weeks  either  a  mercurial  purge, 
calomel,  three  grains,  or  blue  mass,  two  grains,  or  a  pill  containing 
euonymin,  podophyllin  and  aloin,  each  one-quarter  grain,  should  be 
given. 

A  good  intestinal  antiseptic  is  to  be  given  after  each  meal,  put  into 
such  a  capsule  as  will  dissolve  in  the  intestine,  but  not  in  the  stomach. 


764  HEADACHE   AND    MlGJtAttJ^, 

So  called  "  enteric  capsules  "  can  be  obtained  at  the  druggists,  or  any 
capsule  can  be  coated  with  a  mixture  made  up  of  salol  four  parts, 
shellac  one  part,  alcohol  forty  parts,  the  capsule  being  dipped  in  this 
several  times  and  allowed  to  dry.^ 

Permanganate  of  potash,  one  grain,  or  sulphocarbolate  of  soda,  five 
grains,  or  resorcin,  three  grains,  or  benzoate  of  soda,  two  grains,  with 
salol,  three  grains,  or  salol,  five  grains,  put  up  in  enteric  capsules,  are 
all  of  service  and  it  is  wise  to  vary  the  drug  used.  An  occasional 
high  large  enema  of  salt  solution  is  very  beneficial  in  these  cases. 

Patients  who  sufier  from  migraine  are  often  anaemic  and  may  need 
iron,  but  I  have  found  that  they  take  ovoferrin,  or  Gude's  peptoman- 
gan  much  better  than  other  forms  of  iron  which  often  produce  attacks 
of  headache.  Tonics  of  all  sorts  are  to  be  given  especially  those  which 
contain  phosphorus.  If  there  is  any  insufficiency  of  the  interni,  strych- 
nine may  be  given,  but  if  the  externi  are  insufficient  it  should  be  avoided. 
Any  eyestrain  is  better  relieved  by  prisms  than  by  operation  on  the 
muscles.  Seguin  used  cannabis  indica  in  one-quarter  grain  dose  with 
some  benefit.  It  is  of  use  when  there  is  insufficiency  of  the  externi. 
Some  authors  recommend  the  long  continued  use  of  arsenic ;  others 
use  bromides.  Gowers  recommends  the  long-continued  use  of  nitro- 
glycerin, jIq  gr.,  with  strychnine,  -^^  gv.  t.  I.  d. 

A  remedy  of  much  service  is  thyroid  extract.  It  is  to  be  given  in 
one  gram  dose  twice  or  three  times  daily  after  meals  and  kept  up  for 
several  weeks ;  the  dose  being  reduced  if  it  produces  an  increase  of 
more  than  10  beats  of  the  heart  a  minute  above  the  normal,  or  if  it 
causes  a  loss  of  weight. 

I  prefer  the  English  preparation  of  Burroughs  and  Welcome  as 
being  reliable  in  strength. 

The  treatment  must  be  kept  up  steadily  for  a  year,  or  more,  in 
order  to  secure  such  a  permanent  change  in  the  habit  of  metabolism  as 
to  secure  a  cure.  A  course  of  treatment  at  Carlsbad  is  often  beneficial 
to  these  patients. 

The  treatment  of  the  attack.  — It  is  often  possible  to  abort  an  attack 
by  washing  out  the  stomach  with  an  alkaline  solution  several  times 
and  then  leaving  a  strong  solution  of  bicarbonate  of  soda  in  the 
stomach.     If  this  is  done  promptly  it  usually  arrests  an  attack. 

During  the  attack  the  patient  is  to  be  kept  quiet  in  a  dark  room 
with  plenty  of  cool  fresh  air,  but  kept  warm  by  hot  bottles,  or  warm 
flannel  wraps.  Heat  to  the  head  or  ice  bags  may  be  used  according 
to  the  preference  of  the  patient. 

In  many  cases  the  coal  tar  products,  antipyrin,  phenacetin,  acetani- 
lid,  or  pyramidon,  in  one  full  dose,  alone  or  in  some  combination,  will 
arrest  the  attack.  In  other  cases  a  strong  cup  of  black  coffee  or  a 
powder  of  caffeine,  three  grains,  or  the  fluid  extract  guarana,  one 
drachm  dose  will  do  the  same.  In  some  cases  very  minute  doses 
T  oVu"  grain  of  ignatia,  repeated  every  ten  minutes,  till  twenty  doses  are 

^  See  Treatment  of  Leukomain  Poisoning  by  Dr.  B.  K.  Rachford,  Med.  News,  May 
and  October,  1894. 


MIGBAINE.  765 

taken,  will  mitigate  the  attack.  In  some  cases  a  five  grain  dose  of 
chloral  hj^rate  repeated  every  half  hour  will  do  the  same.  I  have 
never  seen  a  good  effect  from  the  use  of  nitroglycerin  in  the  attack, 
though  others  recommend  it.  A  full  dose  of  ergot  without  or  with 
bromide  of  potash  may  give  relief.  Every  patient  comes  at  last  to 
rely  on  some  one  of  these  remedies,  so  if  one  fails  it  is  well  to  try 
another. 

If  everything  else  fails,  a  hypodermic  of  morphine  will  stop  the 
pain  and  secure  sleep  and  when  the  patient  awakens  the  pain  is  gone. 
There  is  no  disease,  however,  in  which  the  danger  of  the  morphine 
habit  is  greater  and  in  which  it  needs  to  be  used  with  such  caution.  I 
believe  that  it  exerts  a  very  detrimental  effect  on  the  metabolism, 
thereby  predisposing  these  patients  to  a  more  frequent  recurrence  of 
attacks. 


CHAPTER  XLIV. 


FUNCTIONAL  PAEALYSIS. 

Myatonia  Congenita  or  Oppenheim's  Disease.     Myasthenia  Gravis, 
mittens  or  Family  Periodic  Paralysis. 


Myasthenia  Inter- 


MYATONIA    CONGENITA. 

Myatonia  congenita  or  Oppenheim's  disease  is  a  rare  condition  occur- 
ring in  infants  and  characterized  by 
a  general  state  of  muscular  weakness, 
probably  due  to  a  defective  develop- 
ment of  the  muscular  tissue. 

At  the  time  when  the  child  should 
begin  to  make  voluntary  movements, 
to  flex  and  extend  its  limbs  and  to 
raise  its  head  it  does  not  do  so,  and 
it  makes  no  eifort  whatever  at  any 
motion.  The  muscles  of  the  eyes, 
tongue,  throat  and  respiration  do  not 
appear  to  be  involved,  so  that  the 
child  can  suck  and  breath.  But  at 
the  age  of  three  or  four  months  the 
parents  notice  the  entire  lack  of  au- 
tomatic or  voluntary  contractions  in 
the  limbs,  and  usually  at  this  time  a 
physician  is  first  consulted. 

On  examination  the  child  is  found 
to  be  apparently  paralyzed,  but  is  usu- 
ally fairly  well  nourished,  very  often 
is  fat  and  there  is  no  atrophy  in  the 
muscles.  If  the  limbs  are  placed  in 
any  position  they  remain  there,  and 
the  joints  are  unusually  movable. 
Thus  the  legs  can  be  doubled  up  on 
the  body  (see  Fig.  303)  showing  a 
lack  of  tone  in  the  muscles  which 
hold  the  joints  in  place.  If  the  child 
is  raised  the  head  falls  backward  and 
it  is  entirely  impossible  for  the  child 
to  sit  up,  as  there  is  a  complete  lack 
of  tone  in  the  muscles  of  the  spine. 
Pig.  302  shows  such  a  child  brought  to  my  clinic  on  a  stretcher  ina- 

766 


Fig. 

302. 

m 

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vl 

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m 

TS 

K        ^^^ 

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p^       ♦  - 

X     ^^1 

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'l^M 

^uj 

^^Bii^f^B 

m 

ri 

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n 

i.m 

t 

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1 

K       ^1 

r 

J 

'i 

'^^i        j^B 

Case  of  myatonia  congenita.  Notice 
the  complete  rel  ixation  of  all  the  limbs. 
The  child  was  carried  on  a  stretcher,  though 
three  years  old. 


MYATONIA    CONGENITA. 


767 


provlsed  by  his  mother.     It  is  impossible  for  the  child  to  turn  over , 
in  bed.     '^11  reflex  activity  is  absent.     There  is  no  disturbance  of 
sensation  and  no  tendency  to  bed  sores  or  to  trophic  changes.     Elec- 
trical examination  shows  a  lessening  of  irritability  to  both  currents 
and  in  some  cases  a  complete  disappearance  of  reaction. 

As  the  child  grows,  the  paralysis  remains,  though  little  by  little 
slight  movements  may  become  possible,  such  as  flexion  of  the  arms  and 
legs,  and  of  the  hands  and  feet,  but  such  movements  are  extremely 
feeble  and  never  are  suflicient  to  enable  the  child  to  move  with  any 
degree  of  force,  to  crawl  or  to  sit  up.  The  muscles  feel  soft  and  flabby 
to  the  touch,  but  the  skin  is  not  cold  as  in  poliomyelitis.  There  is  no 
affection  of  the  bladder  or  rectum  and  if  the  children  survive  the  first 
year  they  are  usually  able  to  exercise  a  voluntary  control  over  them. 

Fig.  303. 


Case  of  myatonia  congenita  showing  complete  absence  of  tone  in  the  muscles  of  the  thigh. 
(Kindnsss  of  Dr.  W.  Y.  Spiller.) 

In  the  majority  of  cases  thus  far  reported  the  children  have  died  from 
some  intercurrent  disease  before  the  end  of  thg  third  year.  In  those 
who  have  survived,  a  slight  improvement  has  taken  place,  but  in  no 
case  has  recovery  been  reported. 

Examination  of  the  muscles  has  shown  an  atrophic  condition  with 
infiltration  of  the  muscle  with  fat  and  an  appearance  not  unlike  that 
observed  in  pseudo-hypertrophic  paralysis.  No  nervous  lesions  have 
been  found.  In  one  case  under  my  observation  the  child  had  reached 
the  age  of  three  years,  showing  no  tendency  to  voluntary  movement, 
and  did  not  show  a  normal  condition  of  intelligence.  This  child 
developed  convulsions  and  died  at  the  age  of  three  and  a  half  years/ 

*  For  complete  record  of  all  the  cases  up  to  date  see  J.  V,  Haberman,  Amer  Jour 
Med.  Sciences,  March,  1910, 


768  FUNCTIONAL   PABALYSIS. 

Another  case  shown  in  Fig.  303  was  a  child  brought  to  the  clinic  in  a 
state  of  good  health  and  good  intelligence,  aged  three,  who  had  never 
walked,  or  sat  up,  and  was  able  to  move  the  hands  only  slightly  and 
was  not  able  to  feed  himself.  There  was  no  tone  to  the  muscles,  and 
the  limbs  were  unduly  flexible.     The  condition  was  stationary. 


MYASTHENIA  GRAVIS. 

History.  —  This  disease  was  first  described  by  Erb  in  1879  under 
the  title  of  "  a  new  probably  bulbar  group  of  symptoms."  Between 
that  time  and  1893  single  cases  were  reported  by  Oppenheim,  Jen- 
drassik,  Eiseulohr  and  others.  Goldflam  then  analyzed  these  cases 
and  drew  a  clinical  picture,  and  in  1895  Jolly  completed  the  clinical 
picture  by  discovering  the  peculiar  electrical  reaction  of  the  muscles 
known  as  the  myasthenic  reaction,  which  is  characteristic  of  the  disease. 
All  these  writers  believed  that  they  were  dealing  with  cases  of  bulbar 
paralysis  in  which  either  an  unexpected  recovery  had  ensued  or  in 
which  no  lesion  had  been  found  after  death.  During  1896—98  a  large 
number  of  cases  were  reported  from  different  countries,  so  that  in  1891 
Laquer  ^  was  able  to  give  a  pretty  satisfactory  summary  of  the  disease, 
and  since  that  time  it  has  been  universally  recognized  as  independent 
of  bulbar  palsy.  Oppenheim^  published  a  monograph  on  it  in  1901, 
and  in  the  same  year  Campbell  and  Bramwell  collected  sixty  cases  in 
"  Brain."  The  best  summary  that  has  appeared  in  this  country  is  that 
prepared  by  Henry  Hun  and  published  in  the  "  Albany  Medical 
Annals,"  January,  1904,  where  117  cases  are  analyzed.  In  1908 
Palmer  collected  126  cases  in  Guy's  Hospital  reports.  I  have  been 
able  to  gather  together  about  250  cases  from  the  literature  up  to  April, 
1912,  and  it  is  from  their  analysis  that  the  facts  here  presented  are 
obtained. 

Etiology.  —  Females  are  more  commonly  affected  than  males,  the 
numbers  being  148  to  92  in  230  cases.  The  age  at  which  the  disease 
appears  is  usually  between  twenty  and  thirty  in  both  sexes.  Cases 
have  been  recorded  in  children,  as  early  as  two.  The  oldest  case  on 
record  was  in  a  man  of  72.  The  table  shows  the  ages  of  cases  col- 
lected. All  races  appear  to  be  liable,  and  negroes  are  not  exempt. 
The  exciting  causes  appear  to  be  over-exertion,  over-work,  fatiguing 
occupations,  emotional  excitement  and  some  infection,  particularly  influ- 
enza. It  has  been  noticed  that  the  disease  occurs  as  a  complication  of 
Basedow's  disease  and  of  goitre.  It  has  also  been  seen  in  patients  with 
myxoedema.  It  has  developed  during  pregnancy  or  lactation  in  seven 
cases.  In  one  of  the  ca^es  pregnancy  exerted  a  favorable  effect.  In 
several  cases  the  termiiiation  of  labor  has  been  followed  by  a  rapid 
increase  of  symptoms. 

'  Volkmann's  Sammlung  Klinische  Vortrage,  No.  205. 
2  Die  Myasthenische  Paralyse,  S.  Karger,  Berlin,  190(>, 


MYASTHENIA    GBAVIS.  769 

Tabl,e. 

Age  of  Cases  of  Myasthenia  Gravis. 

1-5    5-10   10-20   20-30   30-40   40-50   60-60   60-70 

3     1      27      78      51      38      9      7 

It  has  been  supposed  to  be  due  to  some  error  of  secretion  in  the 
ductless  glands,  the  parathyroids  and  the  thymus  being  both  accused. 
Certain  defects  of  metabolism,  such  as  the  deficient  excretion  of  calcium 
salts  in  the  urine  and  faeces,  may  be  secondary  or  may  act  as  causative 
factors.^     No  definite  causation  is  as  yet  determined. 

The  rather  sudden  onset  of  the  symptoms  and  a  complete  recovery  in 
many  cases  has  given  rise  to  the  theory  of  the  toxic  origin  of  myasthenia 
gravis,  it  being  supposed  that  some  poison  developing  in  the  body  has 
a  selective  action  upon  the  muscles,  especially  the  muscles  that  are  in 
the  most  constant  use.  Hence  the  early  involvement  of  the  eyes,  of  the 
muscles  of  the  face,  tongue  and  larynx,  of  the  hands  and  of  the  muscles 
involved  in  walking.  We  know  of  a  similar  condition  occurring  sud- 
denly and  passing  off  rapidly  in  the  form  of  family  periodic  paralysis, 
though  in  that  disease  the  muscles  of  the  face  appear  to  escape. 

Pathology.  —  The  pathology  of  myasthenia  is  still  undetermined. 
The  many  reports  of  negative  findings  in  the  entire  nervous  system 
makes  it  evident  that  the  disease  is  not  due  to  a  central  lesion,  although 
in  isolated  cases  minute  hemorrhages  in  the  pons  and  medulla  and 
degenerations  of  the  nuclei  of  the  cranial  nerves  have  been  occasionally 
observed.  Authorities  all  agree  however  that  there  is  no  uniform 
nervous  lesion. 

In  a  certain  number  of  cases  too  numerous  to  be  explained  on  the 
theory  of  coincidence  an  enlargement  of  the  thymus  has  been  found 
after  death.  The  glandular  tissue  itself  is  hypertrophied,  and  in  many 
cases  tumors  of  the  thymus  gland,  fibroma  and  sarcoma,  have  been 
found.  On  the  other  hand,  there  are  many  cases  that  have  been  exam- 
ined where  no  trace  of  thymus  enlargement  has  been  observed,  hence 
disease  of  the  thymus  cannot  be  regarded  as  an  essential  lesion  of  the 
disease. 

In  the  muscular  system  throughout  the  body  and  also  in  the  glands 
and  viscera  of  the  body  numerous  collections  of  lymphatic  cells  along 
the  course  of  the  lymphatics  and  about  the  blood  vessels  have  been 
observed.  These  have  been  called  lymphorrhages  and  are  so  uniformly 
found  that  they  may  be  considered  as  characteristic  of  myasthenia. 
Fig.  260,  page  620,  shows  such  a  collection  of  lymphatic  cells  within 
the  muscle  in  Hun's  case.  The  origin  of  these  cells  is  uncertain.  The 
reason  for  their  occurrence  is  unknown  and  their  influence  in  pro- 
ducing the  disease  is  not  yet  understood.  But  they  have  been  found 
both  in  cases  that  have  come  to  post  mortem  and  also  in  muscle  tissue 
which  has  been  excised  from  living  patients. 

Symptoms.  —  The  disease  begins  as  a  rule  with  some  form  of  stra- 
})ismus,  either  external  or  internal,  producing  double  vision,  which  is 
the  first  thing  noticed  by  the  patient.  This  is  often  associated  with 
49  '  See  R.  Pemberton,  Amer.  Jour.  Med.  Sci.,  June,  1910, 


770 


FUNCTIONAL  PARALYSIS. 


ptosis,  but  occasionally  with  paralysis  of  the  orbicularis.  This  mode 
of  onset  occurred  in  78  out  of  190  cases.  Weakness  in  the  muscles 
of  the  face,  tongue,  pharynx,  larynx  and  neck  develop  soon  after  the 
ocular  palsy  or  may  precede  it.  Difficulty  of  speech  was  the  symptom 
first  noticed  in  33  cases;  difficulty  in  swallowing  in  16  cases  and  dif- 
ficulty in  using  the  lips  and  tongue  in  6  cases.  The  symptoms  are  not 
uniformly  bilateral.  The  muscular  weakness  usually  extends  down- 
ward to  the  arms  and  hands  and  later  on  to  the  legs.  This  weakness 
at  first  consists  in  a  general  muscular  lassitude  and  undue  fatigue  on 
exertion,  so  that  a  few  movements  exhaust  the  patient  completely  and 
make  him  incapable  of  using  his  hands  for  any  length  of  time  or  for 
walking  or  going  up  stairs. 

The  paralysis  of  the  eyeballs  and  of  the  face  and  mouth  give  rise  to 

an  almost  typical  mask-like  appear- 
ance of  the  face  which  is  very  like 
that  seen  in  bulbar  palsy  (see  Fig. 
258)  and  hence  the  disease  is  usu- 
ally mistaken  for  this  affection. 
It  will  be  remembered  however 
that  in  bulbar  palsy  the  eyes  are 
usually  not  affected,  or  if  at  all, 
very  late  in  the  course  of  the  dis- 
The  double  ptosis  and  the 


Fig.  304. 


ease. 


Ptosis  and  strabismus  in  myasthenia  gravis. 
Icon,  de  la  Salpetriere,  IV.,  62. 


immobility  of  the  face  produce  a 
rather  sleepy,  indifferent  appear- 
ance, and  the  lack  of  facial  expres- 
sion is  very  noticeable  when  the 
patient  laughs  or  cries.  In  speak- 
ing the  voice  becomes  nasal,  and 
very  often  the  speech  is  quite  indis- 
tinct, and  in  fact  in  some  cases 
has  become  impossible,  the  weak- 
ness of  the  laryngeal  muscles  be- 
ing visible  to  the  laryngoscope. 
Swallowing  is  also  interfered  with, 
food  causing  choking  and  fluids 
regurgitating  through  the  nose,  and  it  is  occasionally  necessary  to  feed 
these  patients  with  a  tube.  Sometimes  dyspnoea  of  a  severe  character 
occurs  and  in  the  fatal  cases  reported  failure  of  respiration  or  suffoca- 
tion from  choking  have  been  the  causes  of  death.  The  muscles  that 
are  weak  show  no  fibrillary  tremor  and  do  not  undergo  any  atrophy. 
Therefore  a  patient  suffering  from  the  disease  appears  to  be  well 
nourished  and  differs  decidedly  from  a  person  suffering  from  chronic 
atrophic  paralysis.  The  muscles  that  are  weak  do  not  show  any  sign 
of  reaction  of  degeneration,  but  if  they  are  exercised  by  either  gal- 
vanism, or  faradism  they  show  a  progressively  increasing  lack  of 
response  to  both  currents  so  that  they  become  inexcitable  to  any 
current,  and  this  reaction,  which  is  characteristic  of  the  disease,  is 


MYASTHENIA    GBAVIS.  Ill 

termed  the^"  myasthenic  reaction."  It  is  a  condition  of  electrical  fatigue 
exactly  similar  to  the  fatigue  produced  by  voluntary  effort.  A  similar 
fatigue  can  be  demonstrated  by  percussing  the  muscles.  There  are 
no  sensory  symptoms  whatever. 

The  disease  may  also  begin  in  the  legs  and  extend  upward  to  the 
trunk  and  arms,  finally  reaching  the  face ;  or  in  the  arms  and  extend 
to  the  legs.  This  mode  of  onset  occurred  in  58  cases.  lu  some  cases  it 
has  been  found  that  the  muscles  of  the  heart,  bladder  and  the  accom- 
modation of  the  eye  and  sphincter  of  the  iris,  have  been  affected  as  well 
•as  the  striped  muscles,  giving  rise  to  symptoms  of  heart  weakness  and 
a  difficulty  in  the  control  of  the  sphincters,  but  as  a  rule  these  symp- 
toms are  absent.  Various  neurotic  disturbances  and  hysterical  attacks 
and  conditions  of  mental  depression  in  consequence  of  long-continued 
illness  have  been  noted  in  various  cases  but  are  not  essential  parts  of 
the  disease. 

The  course  of  the  disease  is  a  variable  one  and  shows  very  marked 
irregularity  in  different  individuals.  The  onset  is  rather  sudden  and 
the  symptoms  of  general  weakness  and  of  bulbar  palsy  may  develop 
within  a  few  days  or  two  weeks.  As  such  a  rapid  course  is  never  seen 
in  a  true  bulbar  paralysis  the  diagnosis  in  these  cases  is  comparatively 
easy.  In  other  cases,  however,  there  is  a  much  slower  onset,  but  in  any 
case  within  a  month  of  the  onset  the  general  feebleness  is  well  marked 
and  the  patients  are  confined  to  bed.  But  the  rest  in  bed  very  often 
has  a  beneficial  effect  so  that  after  two  or  three  weeks  the  paralysis 
gradually  disappears  and  the  patient  may  get  about  again  in  comparative 
comfort  and  remain  quite  well  for  months.  Thus,  one  patient  under 
my  care,  a  young  girl  whose  ocular  palsy  had  remained  stationary  for 
several  years,  has  been  confined  to  bed  for  three  months  at  a  time  at 
different  periods  during  the  last  three  years,  but  is  now  quite  able  to  be 
out  and  to  walk  about.  In  some  cases  during  the  period  of  greatest 
weakness,  the  muscles  become  quite  flabby  and  even  atrophic,  but  grad- 
ually regain  their  strength  and  form  after  long  resting.  This  was  the 
case  in  the  patient  just  described,  and  in  her  case  during  the  period  of 
intense  weakness  when  she  could  hardly  turn  over  in  bed,  the  heart 
sounds  were  distinctly  feeble  and  the  pulse  irregular  and  rapid,  show- 
ing that  the  heart  was  involved.  She  has  never  lost  control  of  the 
sphincters. 

In  another  case  under  my  care  the  weakness  of  the  facial,  ocular  and 
tongue  muscles  was  extreme,  but  the  arms  and  legs  were  never  suffi- 
ciently paralyzed  to  compel  the  patient  to  go  to  bed  or  to  cease  to  feed 
himself.  In  this  case  the  laryngeal  muscles  were  the  first  to  be  affected 
and  the  imperfect  speech  followed  by  difficulty  in  swallowing  and  then 
by  paralysis  of  the  tongue  oh  one  side  with  marked  deviation  were  the 
most  prominent  symptoms. 

In  one  woman  of  40,  a  sister  in  a  convent  who  had  done  a  great 
deal  of  hard  work,  tlie  disease  began  with  difficulty  in  the  legs  and 
weakness  on  going  upstairs  and  in  rising  from  a  chair.  This  was 
followed  by  some  weakness  in  the  back,  so  that  she  could  not  lift  heavy 


772  FUNCTIONAL   PAEALYSIS. 

objects,  and  within  a  month  the  arms  were  decidedly  affected  but  not 
entirely  paralyzed.  She  could  feed  herself  but  could  not  lift  heavy 
objects.  At  the  end  of  six  weeks  double  vision  developed,  with  external 
strabismus  and  a  slight  difficulty  in  talking.  All  these  symptoms  sub- 
sided gradually  under  rest  but  recur  at  any  time  that  she  attempts  any 
labor,  so  that  she  remains. in  a  state  of  invalidism.  The  difficulty  has 
been  present  for  six  months. 

The  cases  in  which  the  symptoms  have  been  limited  to  the  muscles 
of  the  head  have  been  contrasted  with  those  in  which  the  muscles  of 
the  entire  body  are  affected  and  an  attempt  has  been  made  to  distinguish 
thus  two  types  of  cases,  but  it  is  probable  that  in  every  case  there  is 
some  degree  of  weakness  in  all  the  muscles. 

In  some  cases  complete  recovery  has  been  reported,  which  has 
remained  for  several  years  before  the  report  was  made. 

Diagnosis.  —  The  diagnosis  is  made  from  the  rapid  development  of 
double  vision  with  ptosis  of  one  or  both  eyes,  difficulty  in  speech  and 
in  swallowing  and  a  weakness  of  the  muscles  of  the  face,  producing  a 
typical  lack  of  expression  under  varying  emotions.  Secondly,  from 
the  development  rapidly  of  a  condition  of  extreme  weakness  in  the 
muscles  of  the  limbs  and  in  many  cases  of  the  head  and  neck.  Thirdly, 
by  the  very  rapid  fatigue  in  all  muscles  on  any  exertion.  Fourth,  by 
the  appearance  of  the  electrical  myasthenic  reaction  in  the  muscles, 
and  lastly  by  the  variation  in  the  intensity  of  the  muscular  weakness 
and  its  prompt  improvement  under  rest.  Negative  symptoms  of  equal 
importance  are  the  absence  of  fibrillary  tremor  in  the  muscles  affected, 
in  the  absence  of  muscular  atrophy,  in  the  absence  of  the  reaction  of 
degeneration  and  the  absence  of  sensory  symptoms. 

The  diagnosis  from  bulbar  paralysis  can  be  made  when  the  following 
points  are  considered.  Bulbar  palsy  begins  either  as  the  result  of  an 
apoplectiform  attack  followed  by  permanent  symj)toms  or  much  more 
commonly  by  a  slowly  advancing  paralysis  beginning  in  the  tongue, 
lips  and  larynx  and  advancing  after  several  months  to  the  face,  but 
rarely  affecting  the  motions  of  the  eyes.  In  a  bulbar  paralysis  the 
muscles  that  are  weak  soon  atrophy,  so  that  the  tongue  is  very  atrophic 
and  is  the  subject  of  fibrillary  twitchings  and  the  face  appears  very  thin. 
In  bulbar  palsy  the  orbicularis  palpebrarum  is  frequently  affected  and 
the  patients  cannot  close  their  eyes.  Ptosis  is  infrequent,  while  in 
myasthenia,  ptosis  and  ocular  palsy  are  the  first  symptoms  in  more 
than  60  per  cent,  of  the  cases. 

In  amyotrophic  lateral  sclerosis  the  symptoms  may  begin  with  a 
bulbar  palsy,  but  here  the  history  is  that  of  a  chronic,  slowly  advanc- 
ing bulbar  palsy  such  as  that  just  described,  and  when  weakness 
develops  in  the  limbs  it  is  attended  by  spastic  rigidity,  by  increased 
mechanical  excitability  in  the  muscles  and  a  marked  increase  in  the 
reflex  activity.  The  rapid  tiring  of  the  muscles  under  exertion  and 
the  myasthenic  reaction  in  the  muscles  to  electricity  are  not  observed 
in  amyotrophic  lateral  sclerosis. 
Chronic  atrophic  paralysis  beginning  in  the  legs  or  arms,  and,  advanc- 


MYASTHENIA    GHAVIS.  773 

ing  slowly^  might  perhaps  be  mistaken  for  the  type  of  myasthenia 
which  begins  in  the  limbs.  But  this  disease  is  slowly  progressive  and 
does  not  recover  under  rest  in  bed.  Muscles  that  are  affected  undergo 
a  progressive  atrophy  and  they  do  not  show  the  myastheuic  reaction. 

The  fact  that  many  of  these  patients  are  rendered  markedly  nervous 
by  their  long-continued  illness  and  the  fact  that  the  symptoms  of  the 
disease  tend  to  vary  in  intensity  from  week  to  week  and  show  great 
improvement  under  rest  has  led  to  the  diagnosis  of  hysteria  in  some 
cases.  Hysterical  weakness,  however,  generally  improves  after  exercise 
and  does  not  disappear  after  rest.  Hysterical  ptosis  is  due  to  a  spasm 
of  the  orbicularis,  never  to  a  paralysis  of  the  levator  palpebrse.  Stra- 
bismus is  never  present  in  hysteria  unless  congenital.  The  difficulty 
in  deglutition  in  hysteria  is  caused  by  a  spasm  of  the  throat  and  liquids 
are  actively  rejected  through  the  mouth,  while  in  myasthenia  the 
paralysis  of  the  throat  leads  to  a  regurgitation  of  fluid  through  the 
nose.  In  hysteria  the  difficulty  in  talking  takes  the  form  of  a  whisper, 
articulation  itself  being  good,  while  in  myasthenia  the  speech  is  slurring 
and  indistinct,  and  is  manifestly  due  to  a  weakness  of  the  muscles  of 
the  tongue  and  face.  Paralysis  of  the  vocal  cords  is  not  seen  in 
hysteria.  Dyspnoea,  if  it  occurs  in  hysteria,  is  attended  by  rapid, 
shallow  respirations  and  an  occasional  spasm  of  the  diaphragm.  In 
myasthenia  the  weakness  of  the  respiratory  muscles  leads  to  continued 
difficulty  in  breathing,  cyanosis  and  accumulation  of  mucus  in  the 
throat.     In  hysteria  there  is  no  myasthenic  reaction  in  the  muscles. 

A  post-diphtheritic  paralysis  may  present  some  symptoms  resembling 
myasthenia,  for  in  that  condition  the  ocular  muscles  may  be  paralyzed 
and  also  the  muscles  of  swallowing.  The  history  of  the  onset  after  an 
attack  of  sore  throat,  the  very  rapid  development  of  the  paralysis  after 
diphtheria,  the  absence  of  ptosis  in  diphtheria  and  also  the  absence  of 
any  paralysis  of  the  face  or  mouth,  the  loss  of  knee-jerks  in  diphtheria 
and  the  appearance  of  ataxia  in  the  limbs  and  the  fact  that  in  diph- 
theritic paralysis  rest  does  not  improve  the  condition,  are  the  points  of 
differentiation. 

Cerebral  tumors  of  the  pons  and  crura  may  give  rise  to  local  symp- 
toms somewhat  resembling  those  of  myasthenia  gravis,  but  in  that  con- 
dition the  onset  of  the  symptoms  will  be  much  slower.  The  headache 
will  be  much  more  constant  and  severe  than  in  myasthenia,  vertigo  and 
vomiting  will  occur  early,  and  the  early  appearance  of  optic  neuritis 
will  make  the  diagnosis  of  a  tumor  clear. 

Prognosis.  —  It  is  difficult  to  give  a  satisfactory  prognosis  in  myas- 
thenia because  of  the  fact  that  cases  run  a  most  irregular  course  with 
rapid  improvement  under  rest  in  some  cases  and  with  very  little  im- 
provement under  any  treatment  in  others.  A  rapidly  fatal  termination 
within  two  weeks  has  been  observed  in  about  5  per  cent,  of  the  cases 
recorded  ;  in  45  per  cent,  of  the  cases  a  fatal  termination  has  taken 
place  within  a  year  of  the  beginning  of  the  symptoms,  the  course  of  the 
case  being  one  of  slowly  increasing  severity  of  weakness  and  paralysis 
with  remissions  or  intermissions  of  shorter  and  shorter  duration.     In 


774  FUNCTIONAL   PAEALYSIS. 

40  per  cent,  of  the  cases  a  state  of  chronic  invalidism  with  intervals 
of  marked  improvement  and  of  apparent  recovery  followed  by  relapse, 
every  six  months  or  a  year  has  been  the  history.  In  the  remaining  10 
per  cent,  of  the  cases  so  far  as  known  a  permanent  recovery  has  taken 
place. 

The  prognosis  appears  to  be  better  in  the  cases  in  which  the  onset  is 
in  the  limbs  than  in  the  cases  where  the  onset  is  in  the  eyes  and  face. 
The  prognosis  in  the  bulbar  cases  is  better  when  the  muscles  of  swallow 
ing  and  respiration  are  not  invaded  by  the  weakness.  It  is  to  be 
remembered  that  in  fatal  cases  death  has  been  due  either  to  choking 
from  inability  to  swallow,  from  suffocation  or  from  respiratory  paralysis. 
The  less  severe  the  symptoms  and  the  shorter  the  attack  of  weakness 
the  better  the  prognosis.  But  cases  have  been  recorded  where  after  a 
short  and  slight  attack  followed  by  great  improvement  a  second  attack 
has  led  to  a  fatal  termination. 

Treatment.  —  Absolute  rest  in  bed  with  the  eyes  closed  in  a  quiet 
room  where  the  patient  will  not  talk  is  essential.  But  the  patient's 
mind  may  be  diverted  by  reading  aloud  or  he  may  be  so  propped  in 
bed  to  allow  of  his  own  reading,  the  book  being  held  by  a  bed-rest. 
The  confinement  to  bed  need  not  be  absolute,  but  the  majority  of  the 
patients  do  better  if  rest  is  insisted  upon. 

It  IS  important  to  maintain  the  nutrition  in  every  way  by  all  forms 
of  food  and  by  digestive  tonics  if  they  are  needed ;  the  bowels  should 
be  kept  open  by  laxatives  or  by  abdominal  massage  and  irrigations. 

There  is  no  known  antidote  to  the  poison  of  the  disease.  Organic 
extracts,  thyroid,  parathyroid,  thymus  and  pituitary  have  been  tried 
in  many  cases,  but  the  results  are  so  contradictory  that  no  statement 
can  be  made  in  regard  to  actual  benefit  derived  from  them.  The  con- 
tinued use  of  calcium  lactate  in  15-grain  doses  three  times  a  day  has 
appeared  to  be  of  some  benefit  in  a  few  cases.  Strychnin  has  been 
used  by  almost  all  observers,  and  some  report  benefit,  while  others 
believe  that  it  does  no  good.  Gentle  massage  may  be  used  to  keep  the 
circulation  and  nutrition  in  good  condition  while  the  patient  is  in  bed, 
but  the  muscles  should  not  be  exercised  either  by  Swedish  movements 
or  by  electricity. 

FAMILY  PERIODIC  PARALYSIS. 

This  disease  is  characterized  by  the  rapid  development  in  the  course 
of  a  few  hours  of  a  general  paralysis  of  all  the  muscles  excepting  those 
of  respiration  and  deglutition,  lasting  for  a  few  hours  or  at  most  for 
three  days  and  subsiding  gradually,  so  that  within  a  short  time  the 
individual  is  in  the  usual  state  of  perfect  health.  The  fact  that  such 
cases  appear  to  be  distinctly  hereditary  m  the  majority  of  instances, 
individuals  in  three  or  four  generations  of  the  family  having  been 
affected,  and  the  fact  that  the  disease  has  a  tendency  to  recur  at  frequent 
intervals,  has  led  to  the  name  family  periodic  paralysis. 

History.  —  Cases  of  sudden  onset  of  paralysis  of  short  duration  were 


FAMILY   PEEIODIC    PARALYSIS.  775 

described  by  Gibney  and  Buzzard  in  1882  and  were  thought  to  be 
malarial  because  of  their  rapid  recovery  under  quinine.  The  first  case 
of  this  disease  was  probably  reported  by  Westphal  in  1885,  who  likened 
his  case  to  that  of  Gibney,  but  affirmed  that  there  was  no  malarial 
element  in  it.  From  that  time  onward  isolated  cases  were  reported, 
until  in  1898  E.  W.  Taylor  ^  was  able  to  collect  about  fifty  cases  of  the 
affection  and  since  that  time  I  have  found  records  of  about  fifty  more. 

Symptoms. — The  disease  is  undoubtedly  due  to  the  sudden  develop- 
ment in  the  blood  of  a  poison  which  acts  very  much  as  curare  acts 
upon  the  muscular  system,  suspending  its  action  completely,  so  as  to 
produce  complete  paralysis  of  a  flaccid  type,  and  this  is  attended  by  an 
abolition  of  the  reflexes  and  by  a  complete  loss  of  electrical  excitability, 
so  that  neither  faradic  or  galvanic  current  will  produce  a  contraction 
of  the  muscles  at  the  height  of  the  period  of  paralysis.  The  sensory 
side  of  the  nervous  system  is  in  no  way  affected.  In  the  majority  of 
cases  the  bladder  and  rectum  preserve  their  contractility;  in  some  of 
the  cases  however  it  has  been  necessary  to  empty  them  mechanically 
during  the  attack.  The  facial  muscles  are  rarely  paralyzed,  but  the 
voice  becomes  weak  and  swallowing  may  be  difficult.  Eespiration  con- 
tinues regular  during  the  attack  but  a  voluntary  long  breath  is  im- 
possible. Consciousness  is  preserved  throughout  and  the  mind  is  not 
affected. 

Patients  suffering  from  this  disease  have  attacks  of  varying  severity. 
Sometimes  there  is  a  general  feeling  of  weakness  with  intense  fatigue 
on  exertion  not  amounting  to  an  actual  paralysis.  In  other  cases  the 
patients  have  to  remain  in  bed  but  can  move  some  muscles.  In  the 
more  severe  type  the  paralysis  is  complete. 

It  is  known  to  develop  usually  at  the  time  of  puberty  and  lasts 
indefinitely  throughout  life.  Goldflam  has  reported  several  cases  in 
families.  J.  K.  Mitchell  and  Flexner  have  made  numerous  observa- 
tions with  regard  to  the  analysis  of  the  blood,  urine  and  faeces,  but 
have  not  succeeded  in  isolating  the  poison  producing  the  disease. 
Several  poisonous  substances  have  been  isolated  from  the  faeces  in 
normal  individuals,  which,  when  injected  into  the  veins  of  animals, 
will  produce  temporary  paralysis  similar  to  that  produced  by  curare, 
but  in  these  cases  of  family  periodic  paralysis  no  one  of  these  intestinal 
toxines  has  been  as  yet  discovered.  The  only  treatment  that  can  be  sug- 
gested is  the  free  use  of  laxatives  and  purgatives  as  soon  as  the  attack 
begins  and  in  my  case  the  patient  had  come  to  the  conclusion  that  such 
treatment  if  begun  early  enough  would  limit  the  duration  and  severity 
of  the  attack.  In  a  few  cases  the  persistent  use  of  alkalies,  especially 
citrate  of  potash,  has  appeared  to  lessen  the  frequency  of  the  attacks. 

The  following  case  from  ray  clhiic  has  been  carefully  studied  by  Dr. 
C.  E.  Atwood,  to  whom  I  am  indebted  for  the  history.  It  is  a  typical 
case. 

The  patient  is  a  young  man,  aged  eighteen,  a  Russian  Hebrew. 
There  were  nine  cases  in  patient's  family,  in  four  generations,  all  on  his 
*  Journal  of  Nervous  and  Mental  Disease,  1898,  vol.  xxv.,  p.  637. 


776 


FUNCTIONAL   PAHALYSIS. 


Fig.  305. 


mother's  side,  and  transmitted  through  both  males  and  females.  These 
comprise  brother,  mother,  mother's  brother,  two  of  grandmother's 
sister's  grandchildren,  two  of  great-grandmother's  sister's  children,  and 
great-grandmother's  brother ;  all  being  typical  cases.  Maternal  grand- 
father had  asthma  ;  mother  migraine.  Second  cousin  choked  to  death 
during  an  attack,  from  vomited  matter  which  he  was  unable  to  clear 
from  the  throat.     Another  died  in  an  attack  when  a  vein  was  opened 

to  obtain  a  specimen  of  blood.  The  brother , 
21,  has  had  but  one  attack,  at  the  age  of  19. 
He  awoke  with  complete  flaccid  motor  paral- 
ysis lasting  about  an  hour ;  did  not  fully  re- 
cover all  day.  The  mother,  41,  has  had  four 
severe  attacks,  viz.,  at  the  age  of  17,  19,  32 
and  37.  Each  attack  lasted  24  hours,  and 
aifected  all  four  extremities,  trunk  and  neck. 
She  awakened  each  time  completely  paralyzed. 
Has  had  milder  incomplete  attacks,  once  to 
four  times  a  year.  In  these,  legs  are  usu- 
ally affected ;  sometimes  also  the  arms  ;  some- 
times only  the  fingers.  All  attacks  have  fol- 
lowed errors  in  diet.  The  affected  uncle,  45, 
has  had  three  attacks  at  intervals  of  about 
five  years.  A  second  cousin  has  attacks 
about  once  or  twice  a  year,  following  over- 
eating on  certain  holidays.  Another  distant 
cousin  had  attacks  for  ten  years  at  intervals, 
early  in  life,  but  lived  to  be  seventy.  For  a 
year  he  was  under  continuous  observation  in 
Vienna.  Attacks  of  all  these  relatives  began 
early  in  life  and  ceased  at  or  before  the  cli- 
macteric. 

The  patient  had  measles  only ;  no  convul^ 
sions  ;  no  hysterical  stigmata ;  is  bright  and 
active.  Health  good  except  for  constipation, 
large  tonsils,  and  adenoids.  Muscular  build.  Large  quadriceps,  es- 
pecially vasti  externi,  suggesting  an  associated  dystrophy.  (See  Fig. 
305.)  This  has  been  found  in  a  few  cases  by  others.  Dystrophies  in 
these  cases  must  be  associated  conditions,  as  the  paralysis  is  never  per- 
manent. There  was  no  dystrophy  in  the  other  cases  of  paralysis  ex- 
amined in  this  family.  That  it  should  appear  at  all  in  a  disease  at- 
tended by  flaccid  paralysis  is  interesting.  First  attack  of  paralysis  at 
4;  second  at  12;  the  third  at  14.  Since  14,  severe  attacks  at  one 
time  once  a  week,  every  Thursday ;  later,  once  in  three  to  six  weeks  ; 
minor  attacks  frequently. 

Severe  attacks  are  typical,  comprising  a  flaccid  motor  paralysis  of 
the  legs,  arms,  trunk  and  neck,  without  apparent  involvement  of  the 
cranial  nerves.  Special  features  in  this  case :  chronic  constipation. 
Attacks  follow  errors  in  diet  and  emotional  excitement.     Attacks  are 


Case  of  family  periodic  pa- 
ralysis. Notice  "hypertropliy  of 
thigh  muscles.  (Kindness  of 
Dr.  C.  E.  Atwood.) 


FAMILY   PEBIODIC    PARALYSIS.  777 

more  frequent  in  winter.  Severe  exercise  does  not  produce  attacks. 
Prodromal  symptoms  often  occur,  e.  g.,  feeling  of  heaviness  and  weak- 
ness in  the  legs.  Onset  of  attacks  is  always  between  midnight  and 
dawn ;  patient  awakens  paralyzed.  If  paralysis  is  at  first  incomplete, 
proximal  muscles  of  legs  are  first  paralyzed,  then  arms,  then  trunk  and 
neck.  Muscle  and  nerve  irritability  is  lost )  reflexes,  deep  and  super- 
ficial, are  lost.  These  all  return  with  return  of  power.  The  heart  has 
a  temporary  mitral  systolic  bruit.  Rectal  sphincter  is  involved  only 
in  severest  attacks.  Then  micturition  is  difficult,  and  the  voice  is  less 
strong.  Swallowing  is  accomplished ;  but  everything  taken  on  first 
day  is  vomited.  Vomiting,  coughing  and  sneezing  are  difficult.  There 
is  no  pain  ;  no  disturbance  of  consciousness  ;  no  parsesthesise ;  no  dis- 
turbance of  sensation  or  muscular  sense.  The  intensity  of  paralysis 
varies.  Improvement  may  occur  with  subsequent  greater  onset  arid 
return  to  complete  paralysis.  Duration  of  severe  attacks,  from  a  few 
hours  to  three  and  a  half  days.  Erections  lasting  about  five  minutes 
occur  in  severe  attacks,  sometimes  two  or  three  times,  causing  em- 
barrassment when  turned  in  bed.  Moderate  sweating  occurs,  but  there 
is  no  thirst.  If  placed  in  erect  or  sitting  posture  patient  faints.  Urine 
analysis  during  attacks  showed  increased  acidity,  increased  indican,  a 
little  albumin,  increased  sulphate  excretion.  Bacteriological  study  of 
faeces  showed  marked  intestinal  infection  and  fermento-putrefaction  due 
to  a  clean-cut  infection  of  the  bacillus  aerogenes  capsulatus.  Same  was 
found  in  aifected  mother  and  brother,  in  lesser  degree ;  and  also,  to  a 
slight  extent,  in  father,  unaffected  by  the  disease.  In  the  intervals 
between  the  attacks  there  are  no  symptoms  of  any  kind.  Treatment 
has  been  by  elimination.  Mild  diuresis  by  potassium  citrate,  mild 
catharsis,  hygiene,  and  increased  meat  diet,  have  apparently  rendered 
attacks  milder,  less  prolonged,  and  less  frequent.  Attacks  are  prob- 
ably due  to  circulation  of  some  toxic  agent  acting  upon  the  muscles. 


CHAPTER  XLV. 

THE  SPASMODIC   NEUROSES.     MYOCLONIA. 
Chorea.     Huntington's  Chorea.     Nodding  Spasm. 

There  are  many  forms  of  fuuctional  nervous  disease  in  which 
spasmodic  contractions  of  the  muscles  form  the  prominent  symptom. 
These  have  been  grouped  together  and  termed  the  myoclonia,  myotilonus 
meaning  a  muscular  twitch.  Fibrillary  twitching  in  a  muscle  has  been 
called  myokimia.  A  sharp  distinction  has  been  drawn  between  twitch- 
ings  which  occur  in  a  single  muscle  or  group  of  muscles  causing  a 
motion  which  cannot  be  voluntarily  produced ;  and  twitchings  which 
cause  a  movement  which  appears  to  have  a  voluntary  conscious  char- 
acter with  an  object  and  involves  a  certain  co5rdinatcd  action  in  the 
muscles  affected.  To  the  former  the  term  spasm  has  been  limited. 
The  latter  are  called  ties.  This  distinction  will  be  observed  in  con- 
sidering the  various  types  of  myoclonia. 

CHOREA. 

Chorea  minor,  Chorea  of  Sydenham,  or  St.  Vitus'  Dance  is  a  func- 
tional nervous  disease,  characterized  by  sudden  rapid  twitchings  of 
any  or  all  of  the  muscles,  by  some  deficiency  in  the  control  of  the 
muscles  which  twitch,  and  by  mental  irritability. 

Etiology. — Out  of  2,239  cases  treated  in  my  clinic,  1,492  were 
females  and  747  were  males.'  In  the  following  table  the  age  at  the 
cime  of  onset  is  shown  : 

Table.  —  Age  of  Onset  of  Chorea. 


3  or  under       4               5              6 

7                8 

9 

10 

11 

11            26            60          111 

134        190 

201 

197 

167 

12              13              14                15 

16              17 

18 

19 

20            20-30 

148        171        135          86 

67          47 

34 

16 

19          34 

It  will  be  seen  that  the  period  from  six  to  fourteen  is  that  of  maxi- 
mum liability,  and  that  at  the  time  of  puberty  (thirteen)  there  are 
more  cases  than  just  before  or  after.  These  statistics  agree  with  those 
of  other  authors.  No  age,  however,  is  exempt,  as  chorea  does  occur 
in  adult  life,  especially  in  women  during  or  after  pregnancy. 

While  children  in  all  classes  of  the  community  are  subject  to  chorea, 
a  large  majority  of  the  cases  are  found  among  the  lower  classes,  espe- 
cially among  children  living  in  tenement  houses,  under  bad  hygienic 

^The  statistics  in  this  article  have  been  collected  by  my  clinical  assistant,  Dr.  S.  P. 
Goodhart. 

778 


CSOHEA.  779 

surroundings  and  eating  poor  and  badly  cooked  food.  When  well- 
nourished  and  well-fed  children  are  affected  it  is  usual  to  find  some 
hereditary  factor  or  some  fright  as  the  cause. 

Anaemia  is  a  most  frequent  cause  of  chorea,  many  of  the  patients 
being  pale  and  languid,  and  having  functional  or  blood  murmurs. 
The  blood  count  usually  shows  a  deficient  number  of  red  corpuscles 
and  a  low  per  cent,  of  hemoglobin,  but  this  is  not  always  present,  even 
in  patients  who  look  pale.^ 

There  is  some  evidence  of  heredity  to  be  found  in  the  histories,  334 
of  the  children's  parents  having, had  the  disease  in  early  life  among  my 
cases,  i.  e.,  7  per  cent.  In  93  cases  a  brother  or  sister  was  simulta- 
neously affected. 

There  is  no  definite  relation  between  the  infectious  diseases  of  child- 
hood and  chorea,  and  it  rarely  occurs  as  a  direct  sequel  of  these  diseases. 

The  relation  of  chorea  to  rheumatism  has  been  studied  by  many 
writers.  Many  cases  of  chorea  develop  after  an  attack  of  acute  rheu- 
matism, with  or  without  endocardial  complications.  In  some  cases  the 
diseases  appear  together ;  in  some  they  occur  alternately.  In  a  collec- 
tion of  2,500  cases  made  from  various  authors,^  I  found  that  26  per  cent, 
had  had  an  attack  of  rheumatism  just  preceding  the  chorea,  and  that 
23  per  cent,  had  a  cardiac  murmur.  Among  my  own  cases  372  had 
an  attack  of  rheumatism  preceding  chorea.  I  do  not  consider  the  in- 
definite muscular  pains,  called  "  growing  pains,"  of  cliildren  a  positive 
evidence  of  rheumatism.  Many  patients  complained  of  such  pains 
before  or  during  the  attack.  The  statement  may  be  made  that  a  certain 
poison  or  microorganism  in  the  blood  under  certain  conditions  produces 
either  rheumatism  or  endocarditis  or  chorea,  according  to  the  varying 
susceptibility  of  the  joints,  heart,  or  brain  in  different  persons. 

In  430  of  my  cases  a  cardiac  murmur  was  found  at  the  base  of  the 
heart  in  systole,  or  at  the  apex  in  systole.  This  was  in  sonle  cases  a 
blood  murmur,  and  passed  away  when  the  disease  subsided.  In  the 
majority  of  my  cases  (251),  however,  it  has  been  a  true  endocardial 
murmur  and  has  remained,  leaving  the  heart  permanently  defective. 
It  is  this  class  of  cases  which  has  established  the  relation  between  endo- 
carditis and  chorea.  In  a  very  large  proportion  of  my  cases  no  car- 
diac murmur  was  found. 

Chorea  is  a  disease  of  the  temperate  climates  and  is  almost  unknown 
in  the  tropics.     Negroes  are  rarely  affected,  but  are  not  exempt. 

The  occurrence  of  chorea  chiefly  in  the  spring  months  between 
March  and  July,  which  has  been  noticed  by  many  authors  and  which 
my  records  confirm,  is  probably  due  to  the  fact  that  at  this  time  the 
confinement  of  the  children  in  the  house  in  bad  air,  the  inability  to 
obtain  out-of-door  exercise  in  winter,  and  the  mental  strain  of  school 
result  in  a  culmination  of  the  malnutrition  which  is  the  chief  predis- 
posing cause  of  the  disease ;  while  the  lesser  number  in  summer  and 
fall  indicates  a  better  nutrition    of  children  who  have  had  an  out-of- 

'  TJniverHity  Medical  Maj^azine,  December,  189f>. 

^See  article,  Chorea,  American  Texthoolc  of  Disca.ses  of  Children,  pp.  481-2. 


780  THE  SPimODlC  NEUnOSES. 

door  life.  This  is  confirmed  by  the  fact  that  such  life  has  a  great  cura- 
tive influence  on  the  affection. 

Fright  is  an  exciting  cause  of  chorea  in  many  cases.  Any  mental 
excitement,  such  as  long  continued  strain  at  school,  sudden  disappoint- 
ment or  grief,  may  also  produce  the  disease.  The  fright  or  shock  may 
precede  the  disease  a  few  days,  or  even  a  week. 

While  it  is  possible  that  eye-strain  from  unbalanced  muscles,  or  irri- 
tation of  the  naso-pharynx  by  adenoids  or  polypi  may  cause  local 
twitching  of  the  eyelids  or  face,  they  never  cause  true  chorea,  and 
treatment  of  the  disease  by  the  relief  of  muscular  insufficiency  of  the 
eyes  is  useless  in  my  experience. 

One  attack  of  chorea  predisposes  to  another,  and  in  a  large  number 
of  cases  relapses  occur.  This  is  probably  merely  because  the  cause 
which  produces  the  first  attack  is  renewed  after  recovery.  In  one 
quarter  of  my  cases  relapses  were  recorded. 

Symptoms.  —  The  movements  occurring  in  chorea  are  spasmodic, 
unexpected,  and  inimitable.  They  cannot  be  arrested  by  the  will  for 
any  length  of  time,  but  are  much  increased  by  attention,  by  excitement, 
or  by  any  effort  to  restrain  them  or  to  use  the  muscles  involved.  In 
the  majority  of  cases  the  twitching  is  momentary  and  slight,  and  does 
not  exhaust  the  patient.  In  a  few  cases  they  are  extended,  violent  and 
continuous,  endangering  the  patient's  safety  and  even  his  life.  These 
movements  interfere  with  voluntary  acts,  rendering  them  imperfect, 
awkward,  excessive,  or  even  impossible.  When  chorea  is  slight,  such 
acts  as  dressing,  writing,  or  playing  the  piano  may  reveal  irregular 
motions  not  noticeable  in  a  state  of  rest ;  and  often  it  is  this  unusual 
awkwardness  in  the  performance  of  these  acts,  or  nervousness,  which 
attracts  attention  to  the  condition.  When  the  disease  is  fully  devel- 
oped, any  movement  involving  fine  coordination  is  impossible.  While 
any  muscle  of  the  body  may  be  involved  in  choreic  movements,  it  is 
more  common  to  notice  them  in  the  face  and  extremities  than  in  the 
trunk.  The  child  makes  queer  grimaces,  especially  when  talking.  The 
eyes  are  suddenly  closed  or  opened,  the  mouth  pouts,  the  cheeks  twitch, 
the  tongue  if  protruded  is  seen  to  be  affected,  and  may  be  suddenly 
withdrawn,  or  even  be  cut  by  an  unexpected  snapping  of  the  jaws 
together ;  occasionally  the  laryngeal  and  respiratory  muscles  are 
affected,  breathing  is  irregular,  and  noises  are  made  in  the  throat.  The 
neck  is  not  as  frequently  affected  as  the  shoulders,  but  the  arms  below 
the  elbows  are  almost  always  involved,  and  irregular  awkward  motions 
of  the  fingers  are  always  seen  or  felt  if  the  hands  are  held.  While  the 
trunk  muscles  do  not  often  appear  to  swell  out  in  contraction,  yet  the 
entire  body  is  uneasy  and  frequent  changes  of  position  are  seen.  The 
legs  below  the  knees  are  in  constant  motion,  but  the  thighs  do  not 
often  twitch  and  the  patient  rarely  falls,  though  he  may  stagger  in 
walking.     These  motions  cease  during  sleep. 

The  iveakness  in  the  muscles  affected  occasionally  becomes  so  ex- 
treme that  forcible  movement  is  impossible,  and  paralysis  may  be  sus- 
pected.    The  hands  may  be  not  only  awkward  but  so  weak  that  the 


CHOBEA.  781 

acts  of  feeding  or  of  dressing  may  be  impossible,  and  the  legs  may  give 
out  so  that  the  child  may  fall  in  walking ;  the  child  may  even  be 
unable  to  turn  over  in  bed.  The  weakness  subsides  as  the  child 
recovers  and  permanent  paralysis  is  never  observed.  But  when  great 
feebleness  develops  and  difficulty  in  talking  and  in  swallowing  occurs 
the  prognosis  is  serious.  I  have  known  the  inability  to  swallow  to 
result  in  such  a  condition  of  malnutrition  that  the  child  died.  Awk- 
wardness and  ataxia  are  always  noticed.  The  disease  might  be  con- 
sidered as  muscular  in  origin  were  it  not  that  it  is  always  attended  by 
mental  irritability  and  is  often  unilateral.  Among  2,239  cases  770 
were  unilateral,  the  right  side  being  affected  a  little  more  frequently 
than  the  left.  If  the  disease  begins  as  hemichorea  it  rarely  becomes 
general.  If  it  has  once  occurred  as  a  hemichorea,  it  usually  recurs  as 
such. 

The  mental  irritability  is  usually  noticeable  early  in  the  disease.  It 
may  be  accompanied  by  inability  to  exert  the  mind  continuously,  and 
by  depression  of  spirits.  The  child  frets,  is  easily  irritated,  is  quarrel- 
some, when  previously  he  has  been  good  tempered,  cannot  be  amused, 
and  is  said  to  be  naughty  when  in  reality  he  is  unable  to  exercise  self- 
control.  He  may  even  act  in  a  semi-imbecile  manner,  laughing  too 
easily.  These  children  are  always  incapacitated  for  study  and  should  be 
taken  out  of  school.     The  mental  excitement  may  interfere  with  sleep. 

A  child  who  is  suffering  from  chorea  is  usually  pale,  badly  nourished, 
has  little  appetite,  is  languid,  is  constipated,  passes  but  little  urine, 
and  that  of  a  high  specific  gravity,  loaded  with  phosphates  and  urates. 
Examination  often  shows  a  loud  systolic  heart  murmur  which  may  be 
either  functional  and  due  to  anaemia,  or  organic  and  due  to  endo- 
carditis. There  is  often  obtained  a  history  of  muscular  pains  or  of  an 
attack  of  rheumatism,  preceding  or  coincident  with  the  onset  of  chorea. 
Headache  is  often  complained  of.  There  is  usually  a  diminution  of 
the  tendon  reflexes  and  a  hyperexcitability  of  the  muscles  to  electrical 
stimulation.      Temperature,  pulse  and  respiration  are  natural. 

The  disease  appears  suddenly  after  a  fright  or  after  some  mental 
strain  or  from  general  malnutrition,  increases  during  the  first  two 
weeks,  lasts  for  several  weeks  (ten  is  the  average)  and  gradually  sub- 
sides, but  may  recur  a  year  later  at  the  same  season  at  which  it  first 
appeared. 

This  description  applies  to  the  majority  of  cases  of  chorea.  There 
are  exceptional  cases  which  require  notice. 

In  a  few  instances  the  motions  are  constant,  excessive  and  violent, 
so  that  the  patient  will  be  thrown  off  a  chair  or  out  of  a  bed,  and  is 
liable  to  incur  injuries.  Unless  these  patients  are  protected  by  lying 
in  bed  and  guarded  by  mattresses  at  both  sides  of  the  bed,  and  are 
kept  asleep  by  narcotics,  they  are  soon  worn  out  and  die  of  exhaustion. 

In  a  few  cases  the  mental  irritation  rises  to  the  pitch  of  acute  mania, 
and  acute  delirium  occasionally  occurs  in  this  form  of  the  disease. 

In  some  cases  the  weakness  is  so  much  more  apjiarent  than  the 
twitching  that  the  case  may  be  considered  as  one  of  paralysis.     Usu- 


782  TRE   SPASMODIC   NEVB0SE8. 

ally  ataxia  attends  this  weakness.  I  have  known  chorea  to  be  diag- 
nosticated as  infantile  paralysis,  and  as  locomotor  ataxia,  though  other 
characteristics  of  these  diseases  were  absent. 

In  a  few  instances  speech  becomes  affected  early,  and  may  be  so 
indistinct  as  to  be  scarcely  understood ;  and  in  these  cases  grunting 
noises  may  be  made  in  the  throat.  Sometimes  nervous  patients  affected 
with  twitching  motions  give  vent  to  loud  words  unexpectedly,  usually 
of  a  profane  or  obscene  kind.  This  condition,  known  as  coprolalia,  is 
not  choreic,  but  hysterical.  So  too  is  echolalia,  in  which  the  patient 
repeats  the  last  word  heard.  Such  patients  often  mimic  motions  and 
show  other  signs  of  hysteria. 

The  joints  may  be  inflamed,  swollen  and  tender,  when  rheumatism 
attends  chorea.  In  a  few  cases  subcutaneous  nodes,  which  are  small, 
round,  hard  nodules  appearing  in  many  parts  of  the  body,  notably  on 
the  back  and  along  the  flexor  surfaces  of  the  extremities,  are  found. 
They  are  regarded  as  evidence  of  rheumatism,  and  have  no  relation  to 
the  chorea. 

Chorea  occurring  in  adult  life  presents  the  same  symptoms  as  in 
childhood,  but  is  usually  attended  by  much  greater  mental  irritability 
and  by  marked  depression  of  spirits ;  in  fact,  a  true  melancholia  not 
infrequently  develops  and  there  is  danger  of  suicide.  It  may  last  three 
or  four  months  and  occasionally  becomes  chronic.  Chorea  is  some- 
times seen  during  pregnancy.  It  develops  in  neurotic  women  during 
the  third  or  fourth  month  and  may  be  very  severe  and  last  for  several 
weeks.  It  also  occurs  just  after  labor  and  is  then  attended  by 
melancholia. 

Duration.  —  The  duration  of  the  disease  varies  greatly  in  different 
cases.  In  some  it  runs  a  rapid  course  and  the  child  is  well  in  a 
month.  In  the  majority  of  cases  it  lasts  about  three  months,  passing 
away  slowly.  In  some  cases  it  lasts  a  year  or  even  more,  with  vary- 
ing degrees  of  severity.  Occasionally  it  becomes  chronic.  The  large 
majority  of  the  cases  terminate  in  recovery,  but  there  is  always  danger 
of  a  relapse,  and  many  patients  suffer  from  a  third  or  even  fourth 
attack,  which  usually  occurs  at  the  same  period  of  the  year  at  which 
the  first  seizure  occurred.  When  a  child  dies  of  chorea  it  is  because 
of  exhaustion  on  account  of  the  severity  of  the  spasms.  In  the  cases 
appearing  during  pregnancy,  it  may  cease  after  the  third  month,  or  it 
may  last  throughout  the  pregnancy.  In  cases  developing  just  after 
labor  it  is  often  severe,  but  not  of  long  duration.  In  a  few  of  these 
severe  cases  it  is  attended  by  mental  symptoms,  mania  or  melancholia, 
and  may  be  fatal.  In  adult  life  chorea  is  apt  to  last  two  or  three 
months,  and  if  attended  by  melancholia,  as  it  often  is,  may  prove  fatal. 

Pathology. — There  is  little  ground  for  positive  statements  regard- 
ing the  pathology  of  chorea  or  regarding  the  situation  of  the  lesion. 
The  lesions  found  by  various  authors ;  minute  hemorrhages  or  capil- 
lary emboli  of  the  basal  ganglia  of  the  brain;  vacuolization  of  the 
nervous  tissue  due  to  distension  of  lymphatic  spaces  around  blood- 
vessels or  nerve  cells ;  various  types  of  degeneration  in  the  cells  of  the 


CHOBEA.  783 

cortex  or  spinal  cord  ;  hyperplasia  of  neuroglia,  are  probably  due  t»  the 
exhaustion  produced  by  the  disease.  The  unilateral  character  of  the 
symptoms  points  to  the  brain  as  the  site  of  the  functional  disturbance, 
and  this  is  confirmed  by  the  mental  symptoms  usually  present. 

Prognosis.  —  This  should  be  favorable,  but  guarded  as  to  duration. 
The  chances  all  favor  recovery  within  three  months,  but  it  is  to  be 
remembered  that  some  cases  last  much  longer,  and  that  many  relapses 
occur.  Another  fact  makes  a  guarded  prognosis  necessary.  Some 
children  are  exceedingly  susceptible  to  arsenic  and  hence  cannot  take 
the  chief  remedy ;  or  if  it  is  given  they  develop  arsenical  poisoning,  or 
even  arsenical  multiple  neuritis.  The  more  anaemic  the  child,  the  worse 
the  prognosis.  The  more  the  mental  excitement,  the  worse  the  prognosis. 

The  development  of  rheumatism  during  the  disease  does  not  render 
the  prognosis  very  grave,  as  few  children  die  of  rheumatism.  It 
lengthens  the  duration  of  the  chorea.  The  occurrence  of  endocarditis 
is  more  serious,  as  it  may  last  through  life  and  finally  lead  to  serious 
cardiac  disease.  During  the  early  stages,  however,  the  cardiac  insuf- 
ficiency rarely  causes  symptoms,  and  I  have  rarely  seen  a  child  with 
chorea  suifer  from  oedema  or  dropsy.  Nor  have  I  seen  a  child  with 
chorea  develop  hemiplegia  from  cerebral  embolism. 

Diagnosis.  —  There  are  many  diseases  in  which  the  chief  and  most 
prominent  symptom  is  a  twitching  of  the  muscles.  These  should  not 
be  mistaken  for  chorea. 

Tic  convulsif,  which  is  a  unilateral  twitching  of  the  face,  is  not 
chorea,  as  shown  by  its  local  limitation.      (See  Chapter  XLVI.) 

Other  forms  of  tic,  such  as  tic  of  the  head  or  shoulder  or  arm,  are 
not  to  be  mistaken  for  chorea,  because  they  are  strictly  limited  in  the 
extent  of  the  twitching,  and  have  in  distinction  from  choreic  move- 
ments the  characteristic  of  a  tic,  viz.,  that  it  is  a  simple  act  originating 
in  an  idea  and  coSrdinated  for  a  special  end,  but  controllable  momen- 
tarily by  eiFort. 

The  maladie  des  tics  convulsives  of  Gilles  de  la  Tourette,  consisting 
of  a  sudden  convulsive  twitching  of  any  or  all  of  the  muscles  of  the 
body,  resembles  chorea,  but  may  be  distinguished  from  it  by  the  facts 
that  it  is  not  attended  by  weakness  or  awkwardness  in  voluntary 
movements,  the  twitching  is  not  increased  or  set  up  by  voluntary 
motion,  and  only  appears  during  rest ;  there  is  no  mental  irritability ; 
the  disease  does  not  usually  begin  till  puberty ;  it  is  chronic  and  does 
not  yield  to  arsenic. 

Habit  tics  should  not  be  confounded  with  chorea.  All  children 
have  a  tendency  to  mimicry,  and  a  child  who  is  afflicted  with  habit 
tic  makes  motions  which  have  the  character  of  voluntary  move- 
ments such  as  winking,  pouting  the  lips,  frowning,  raising  the  eye- 
brows, turning  the  head,  shrugging  the  shoulders,  or  moving  hands  and 
feet.  These  habit  tics  are  not  as  quick  and  jerky  as  the  twitchings 
of  chorea,  but  after  a  time  they  arc  not  easily  controlled,  as  they  are 
at  first,  and  hence  resemble  chorea.  Voluntary  control  can,  however, 
be  exercised  if  the  child's  attention  is  forced  to  the  necessity  of  it,  and 


784  TEE    SPASMODIC   NEUBOSES. 

in  this  condition  moral  treatment  and  general  hygienic  measures,  such 
as  baths,  exercises  and  massage  are  of  more  service  than  medicine. 

Myoclonus  multiplex  is  a  spasmodic  affection  resembling  chorea. 
But  in  this  disease  it  is  the  muscles  of  the  body  and  of  the  proximal 
portions  of  the  limbs  which  are  affected ;  the  face,  hands,  and  feet  are 
quiet.  The  spasms  are  bilateral  and  symmetrical ;  they  occur  at  intervals 
and  are  rapidly  repeated,  as  many  as  ninety  contractions  of  the  muscles 
in  a  minute  may  occur.  The  movements  are  very  violent,  so  as  to 
throw  the  patient  down  if  walking,  or  to  hurl  him  off  of  a  chair. 
The  spasm  can  be  brought  on  by  tapping  the  tendons.  During  the 
interval  between  the  spasms,  a  fibrillary  tremor  of  the  muscles  may  be 
seen.  The  disease  may  occur  at  any  age,  but  is  usually  seen  in  adults 
after  some  fright  or  anxiety,  in  persons  of  a  hysterical  type. 

The  hemichorea  following  hemiplegia  is  characterized  by  slow, 
irregular  ataxic  motions,  excited  only  by  voluntary  effort  or  by  emotion, 
and  is  rather  a  hemiathetosis  than  a  true  chorea.  The  history  of  its 
onset,  following  an  attack  of  hemiplegia  or  some  form  of  apoplectic 
stroke,  distinguishes  it  clearly  from  ordinary  hemichorea. 

Treatment.  —  Remove  the  patient  from  the  bad  hygienic  surround- 
ings which  have  caused  the  disease,  and  send  him  to  the  seashore,  if 
possible,  for  a  life  out  of  doors.  Keep  him  quiet  and  without  excite- 
ment in  any  case,  and  always  take  him  out  of  school.  Give  him  a 
nutritious  diet  of  varied  character,  without  restrictions,  and  aid 
digestion  by  a  bitter  tonic,  by  iron,  and  by  laxatives.  Long  continued 
(half-hour)  baths  at  temperature  of  95°  are  to  be  given  twice  a  day, 
the  child  being  allowed  to  play  in  the  water.  No  cold  shocks  are  to 
be  given.  Rest  in  bed  or  on  a  couch  is  a  good  thing  with  older 
children,  but  a  young  child  who  will  not  rest  should  be  dressed  in 
merino  and  allowed  to  play  on  a  bed,  but  not  to  run  on  the  floor. 
Gentle  massage  of  the  entire  body  may  be  given  daily  for  an  hour. 
The  child  should  see  only  one  or  two  members  of  the  family,  so  as  to 
be  kept  free  from  excitement.  It  may  be  amused  by  reading.  These 
means  alone  will  result  in  improvement  in  two  weeks. 

The  improvement  can  be  hastened  by  medicines.  If  rheumatism 
has  preceded  or  attended  the  chorea,  it  is  best  to  use  salicylate  of  soda, 
salicin,  aspirin,  or  oil  of  wintergreen  freely.  They  may  be  combined 
with  antipyrin  or  exalgin  in  small  doses.  If  heart  tonics  are  needed, 
camphor  or  caffeine  are  the  best.  The  doses  of  all  these  drugs  must 
be  determined  by  the  age  and  susceptibility  of  the  child. 

If  there  be  no  history  of  rheimaatism,  it  is  well  to  think  of  the  pos- 
sibility of  malarial  infection  as  a  cause  of  chorea,  and  periodical  rises 
of  temperature  will  indicate  a  mercurial  purge,  followed  by  the  use  of 
quinine  or  of  Warburg's  extract  for  at  least  a  week.  This  often  cuts 
short  an  attack  of  chorea. 

In  all  other  cases  arsenic  is  the  chief  remedy.  I  prefer  Fowler's 
solution*  It  is  to  be  begun  in  3-drop  doses  three  times  a  day,  and  in- 
creased 1  drop  daily  until  a  puffiness  of  the  eyelids,  nausea,  or  pain  in 
the  stomach  are  produced.     Some  children  will  stand  only  10  drops  a 


EEBEDITABY    CHOREA.  785 

day,  others  will  take  40  drops  a  day  without  discomfort.  It  is  my 
rule  to  increase  the  dose  till  physiological  effects  are  produced,  then 
to  stop  it  for  twenty-four  hours,  and  then  to  give  the  dose  just  below 
the  maximum  for  some  days  or  even  weeks,  always  watching  for 
poisoning,  and  stopping  if  this  appears.  It  should  be  begun  well 
diluted  and  be  given  after  meals  only. 

If  a  child  cannot  take  arsenic,  chloral  hydrate  in  2  or  3-grain  dose 
for  a  child  of  six  may  be  given  3  or  4  times  in  24  hours.  This  may  be 
combined  with  bromide  of  sodium  in  5  or  10-grain  dose,  and  given 
after  meals.     The  condition  of  the  heart  is  to  be  watched  carefully. 

Chloretone  in  dose  of  5  grains  three  times  a  day  for  a  child  of  six 
has  been  of  service  in  some  cases.  The  dose  may  be  increased  in  fre- 
quency in  older  children. 

In  some  cases  tincture  of  cimicifuga  in  half  drachm  dose  repeated 
and  increased  5  minims  daily  may  do  good.  This  is  also  of  service  in 
adults. 

In  very  severe  cases  where  the  motions  are  violent,  the  patient 
should  be  guarded  from  injuries  by  being  kept  in  bed,  mattresses  being 
placed  at  the  sides  of  the  bed.  In  these  cases  begin  with  5  or  6  drops 
of  arsenic  in  milk  every  6  hours,  and  increase  the  dose  each  time  1 
drop,     At  the  same  time  give  bromide  and  chloral  freely  by  rectum. 

It  may  be  necessary  to  give  inhalations  of  chloroform  in  these  cases, 
to  induce  sleep.  Sulphonal  or  chloralamide  or  veronal  are  also  of  ser- 
vice in  these  cases.  Hypodermic  use  of  morphine  usually  increases 
the  spasms,  but  hydrobromate  of  hyoscine  in  -^^  grain,  or  less  for 
a  small  child,  may  do  good. 

In  addition  to  these  direct  remedies  the  condition  of  anaemia  must 
be  combated  by  some  form  of  iron.  The  albumenate  of  iron  is  the 
best  form  for  children,  as  it  is  tasteless  and  can  be  given  in  milk. 
Chocolate  lozenges  containing  iron  may  also  be  given. 

When  all  medicinal  and  other  treatment  fails,  and  the  case  seems 
chronic,  a  change  of  climate,  especially  to  the  seashore,  is  often  of  great 
benefit,  but  sea  bathing  is  not  to  be  recommended.  High  altitudes  are 
not  favorable  to  chorea. 

HEREDITARY  CHOREA  OR  HUNTINGTON'S  CHOREA. 

A  form  of  chorea,  developing  in  adult  life,  and  distinctly  hereditary 
in  origin,  was  first  described  by  Dr.  Huntington,  of  Easthampton, 
L.  I.,  after  whom  it  was  for  a  time  named.  He  observed  a  family  in 
which  cases  had  developed  in  four  successive  generations.  Others  have 
recorded  the  history  of  other  families  similarly  afflicted  and  now  the 
disease  is  recognized  all  over  the  world. ^ 

The  chorea  begins  about  the  age  of  forty,  though  a  case  has  been 
seen  as  early  as  twenty -three.     The  motions  begin  in  the  hands  and 

'A.  S.  Hamilton  has  described  twenty-seven  cases,  Amer.  Journ.  of  Tnsan.,  Jan., 
1908,  and  Brown inf<  has  collected  the  records  of  over  one  hundred  cases  from  literature  ; 
Neurographs,  vol.  i.,  No.  2,  May,  1908.  See  also  Curschmann,  Deut.  Zeitschr.  f. 
Nervenheil.,  Oct.  15,  1908. 

60 


786  TEE    SPASMODIC   NEUROSES. 

feet  and  extend  until  the  entire  body  is  affected.  They  are  excessive, 
so  that  speech,  coordinated  actions  and  gait  are  involved  and  the 
muscles  are  all  in  violent  movement,  rather  more  intense  than  in  ordi- 
nary chorea.  The  spasms  are  increased  by  mental  or  physical  exertion, 
but  occur  when  the  body  is  at  rest.  They  cease  during  sleep.  They 
are  never  unilateral  only. 

After  a  time  mental  symptoms  uniformly  develop,  and  many  of  the 
cases  have  been  observed  in  asylums.  In  some  patients  there  is  evi- 
dently a  congenitally  defective  mind.  In  others  a  progressive  dementia 
develops.  The  mind  slowly  fails,  there  is  a  loss  of  power  of  applica- 
tion and  of  judgment  and  of  memory,  and  there  is  despondency  and 
emotional  irritability.  In  some  cases  suicidal  impulses  are  felt.  In 
quite  a  number  of  cases  a  more  active  type  of  insanity  appears,  delusions 
of  persecution  or  of  an  exciting  or  anxious  type,  self-accusations  and 
maniacal  outbursts.  In  some  the  hebephrenic  type  of  dementia  prsecox 
has  been  observed.  In  the  latest  stage  a  gradually  increasing  spastic 
paraplegia  develops,  both  legs  and  arms  becoming  rigid  with  increased 
reflexes  and  imperfect  control  of  the  sphincters.  The  disease  may  last 
many  years.  Certain  associated  symptoms  have  been  noted  by  various 
observers,  such  as  ptosis,  an  increase  in  the  muscular  tonicity,  a  slow 
response  to  painful  sensations,  and  bulimia ;  but  they  are  not  particu- 
larly characteristic. 

Males  are  more  frequently  affected  than  females.  It  may  be  trans- 
mitted through  either  side  of  the  family,  but  Dana  reports  a  family, 
where  the  transmission  was  wholly  through  the  females.  While  it 
appears  as  a  rule  in  adult  life,  there  is  a  tendency  for  the  disease  to 
appear  at  progressively  earlier  ages  in  succeeding  generations,  and 
Sinkler  has  reported  a  congenital  case  of  hereditary  chorea.  Hamilton 
observed  in  his  cases  many  signs  of  degeneracy  and  of  early  senility. 
There  are  cases  of  senile  chorea  with  dementia  in  which  no  inheritance 
can  be  traced. 

The  lesion  found  is  a  sclerosis  of  the  cortex  of  the  brain  scattered 
widely  in  patches  especially  about  the  blood  vessels,  and  secondary  to 
changes  in  the  vessel  walls.  Sclerotic  patches  have  also  been  found  in 
the  basal  ganglia. 

The  prognosis  is  bad  and  no  treatment  has  succeeded  in  stopping 
the  progress  to  dementia. 

NODDING  SPASM. 

In  some  children  soon  after  birth  or  within  the  first  six  months  of 
life  certain  oscillatory  motions  of  the  head  develop,  usually  lateral, 
sometimes  vertical,  and  occasionally  a  mixture  of  both.  These  are 
very  commonly  associated  with  a  condition  of  rotary  or  lateral  nys- 
tagmus which  appears  when  any  attempt  is  made  to  turn  either  head 
or  eyes.  These  nodding  movements  do  not  seem  to  incommode  the 
child  and  are  certainly  not  attended  by  pain.  They  are  not  unlike 
the  nodding  movements  and   slight  rotary  movements  occurring  in 


NODDING    SPASM.  787 

very  old  persons,  though  these  are  not  associated  with  nystagmus. 
Nodding  spasm  is  more  common  in  children  of  neurotic  inheritance 
and  children  who  are  badly  nourished  and  the  subject  of  rickets.  It 
develops  not  infrequently  in  cases  of  hydrocephalus  in  mild  degree  in 
the  early  stage.  It  also  occurs  in  tumors  of  the  brain  in  children. 
The  origin  of  the  condition  is  wholly  unknown,  though  in  several  cases 
when  children  subject  to  nodding  spasm  have  grown  up  and  have  out- 
grown the  spasm  defects  of  vision  of  the  nature  of  extreme  astigmatism 
have  been  discovered.  It  seems,  therefore,  not  unlikely  that  eyestrain 
may  be  the  origin  of  these  conditions.  Little  is  known  of  the  causa- 
tion. The  spasm  usually  persists  until  the  child  gets  to  be  a  year  old, 
when  it  is  outgrown.  I  have  rarely  seen  it  last  through  the  third  year. 
As  the  disease  is  rarely  seen  in  adult  life  it  is  evident  that  the  majority 
of  patients  recover.  There  is  no  known  form  of  treatment,  but  in 
these  children  the  general  health  should  be  carefully  attended  to. 
Proper  nutrition  should  be  secured ;  good  air  and  good  food,  frequent 
bathing,  and  tonics,  especially  cod-liver  oil  and  arsenic,  are  distinctly 
beneficial. 


CHAPTER  XLVI. 

THE  SPASMODIC  NEUROSES. 
The  Tics.     Facial  Spasm.     Torticollis.     Tortipelvis.     Myoclonus.     Myotonia. 

The  term  tic,  which  was  at  first  used  to  indicate  a  lightning-like 
twitching  of  the  face,  and  later  was  employed  to  describe  all  sorts  of 
sudden  spasmodic  contractions  of  the  muscles  of  the  forehead,  neck, 
shoulders  or  extremities,  has  of  late  been  defined  by  Brissaud^  and 
Meige^  as  "a  primary  act  caused  by  an  external  irritation  or  by  an 
idea  and  codrdinated  for  a  special  end."  By  repetition  this  act  becomes 
a  habit  and  finally  occurs  involuntarily  without  apparent  cause  or 
purpose.  It  may  then  increase  in  intensity  and  frequency  and  be 
associated  with  other  acts.  The  movement  is  preceded  by  a  sense  of 
need  and  by  an  uncontrollable  impulse  ;  to  control  it  causes  a  sense  of 
distress  or  anxiety,  but  it  can  be  controlled  momentarily  by  an  effort. 
It  ceases  when  the  mind  is  strongly  diverted  from  it  and  it  is  suspended 
during  sleep.  Charcot  taught  that  it  was  a  psychic  disease.  Friedreich 
called  it  a  co5rdinated  spasm  of  a  remembered  movement.  The  French 
School  considers  it  a  psychomotor  affection. 

Tics  are  movements  originally  voluntary  and  hence  initiated  by  a 
conscious  impulse  sent  from  the  cerebral  cortex ;  and  therefore  have 
all  the  characteristics  of  a  true  intended  coordinated  action  as  distin- 
guished from  a  reflex  or  involuntary  spasm.  They  are  convulsive  in 
character,  because  in  the  end  they  are  wholly  beyond  conscious  control 
and  have  no  obvious  purpose,  but  in  fact  become  an  annoyance  to  the 
patient.     They  have  been  called  by  some  stereotyped  movements. 

Children  between  the  ages  of  six  and  thirteen  are  not  infrequently 
found  to  acquire  certain  abnormal  movements ;  either  by  imitation  of 
other  children  or  because  of  some  slight  sensation  which  has  been 
unpleasant.  This  movement  is  always  voluntary  in  its  character  at 
first.  But  after  a  time  it  may  be  repeated  so  often  as  to  become  auto- 
matic and  the  child  may  be  unconscious  of  it.  Then  it  may  become 
frequent,  occurring  every  few  minutes  or  almost  constantly,  and  pro- 
duce unpleasant  effects. 

The  tic  may  consist  of  a  winking  of  one  or  both  eyelids,  of  wrinkling 
the  forehead,  of  pouting  the  lips,  of  twitching  the  head,  of  shrugging 
the  shoulders,  of  throwing  the  hands  up  or  out,  of  twitching  the  fingers, 
of  snapping  the  fingers,  of  catching  the  breath,  of  sighing  deeply,  of 
twitching  the  body,  of  kicking  the  legs,  or  of  any  other  simple  volun- 
tary movement.     Some  children  make  noises  in  the  mouth  or  throat, 

^  Brissaud.     Tics  et  spasms  de  la  face.     Jour.  deMed.  et  Chur.  pract.,  Jan.  24,  1894. 
^  Meige  et  I'eindel.     Les  Tics  et  leur  traitment.     Manon  et  Cie,  Paris,  1902.     Meige. 
Art.  Tics.     Traite  de  Medicine,  tome  x,  p.  330,  Paris,  1905. 

788 


Thb!  Spasmodic  nEUROSE^.  789 

Of  eVeH  l-epeat  words.  Some  children  touch  objects  as  they  pass  them. 
Some  have  impulses  to  count  or  to  repeat  phrases.  In  fact  any  mental 
or  motor  impulse  of  a  morbid  kind  may  pass  into  a  habit. 

It  is  chiefly  among  neurotic  children  whose  inheritance  is  bad  and 
whose  training  has  been  imperfect  and  whose  nutrition  is  not  good 
that  these  habit  tics  develop.  The  exciting  cause  may  be  some  slight 
irritation  from  imperfect  eyesight,  from  nasal  obstruction,  from  uncom- 
fortable clothing,  from  too  great  restraint  at  school,  or  some  fright  or 
emotional  shock.  It  sometimes  develops  when  the  child  first  goes  to 
school. 

The  epidemic  forms  of  chorea  described  as  occurring  in  the  middle 
ages  were  really  tics  from  imitation,  in  persons  of  hysterical  type. 

Tics  are  not  confined  to  children.  They  often  develop  in  adults. 
A  tic  being  a  physiological  movement,  it  is  evident  that  a  coordinated 
group  of  muscles  is  usually  involved.  Every  possible  variety  of  move- 
ment has  been  observed,  and  to  describe  the  forms  of  tic  it  would  be 
necessary  to  mention  almost  every  known  act.  Some  special  forms 
may  be  considered  because  of  their  frequency. 

Tic  of  the  eyelids  consists  of  a  spasmodic  closure  of  the  eyes,  attended 
by  wrinkling  of  the  forehead ;  the  eyes  cannot  be  held  open  for  any 
length  of  time  and  this  gives  the  patient  much  inconvenience.  This  is 
not  to  be  confounded  with  true  blepharospasm  (see  page  791). 

Tic  of  the  face  is  usually  seen  in  the  form  of  a  grimace  at  first  uni- 
lateral, later  bilateral.  There  may  be  movements  of  the  lips,  of 
pouting,  sucking,  pursing,  and  all  sorts  of  expressions  of  the  face 
ensue,  giving  the  appearance  of  depression  or  of  mirth. 

Sometimes  a  local  spasm  in  the  risorii  may  occur.  Thus  a  well- 
nourished  young  woman,  not  anaemic,  suffered  from  a  bilateral  tic 
of  the  middle  muscles  of  the  face,  so  that  she  looked  all  the  time  as  if 
smiling,  and  the  upper  lip  was  stretched  tightly  back  across  the  upper 
teeth,  and  this  impeded  her  speech,  her  eating,  and  gave  her  the  appear- 
ance of  laughing  all  the  time,  when  she  had  no  such  desire.  This  con- 
dition came  on  very  suddenly  after  a  great  mental  shock,  soon  after  the 
birth  of  her  first  baby,  and  persisted  for  six  months.  The  chief  dis- 
comfort lay  in  the  great  limitation  of  talking.  There  was  no  affection 
of  the  tongue  or  of  the  larynx,  but  all  letters  involving  the  use  of  the 
lips  were  imperfectly  pronounced,  especially  when  her  attention  was 
directed  toward  it.  When  her  attention  was  diverted  by  an  examina- 
tion of  her  eyes  and  throat  the  spasm  relaxed  and  the  lip  assumed  its 
normal  appearance,  but  the  moment  she  began  to  speak,  or  the  moment 
that  she  tried  to  smile,  the  sudden  contracture  of  the  muscles  resulted 
in  a  flattening  of  the  upper  lip  against  the  teeth,  where  it  was  held 
rigid.  She  appeared  to  be  perfectly  well  in  every  other  respect ;  there 
was  a  slight  tendency  to  divergence  of  the  eyes ;  there  had  never  been 
any  pain  or  difficulty  in  chewing.  There  was  no  pain  on  pressure  along 
the  fifth  or  seventh  nerves ;  there  was  no  apparent  obstruction  in  the 
nose  or  throat. 

Sometimes  it  is  only  the  corner  of  the  mouth  or  side  of  the  nose 


790  TEE    SPASMODIC    NEUEOSES. 

which  is  raised.  Sometimes  the  chin  is  drawn  down.  This  is  to  be 
sharply  distinguished  from  facial  spasm  (see  page  791). 

Tic  of  the  tongue  may  cause  sucking  or  clicking  sounds. 

Tic  of  the  masseters  may  cause  grating  of  the  teeth. 

Habitual  or  spasmodic  yawning  is  a  form  of  tic.  It  is  often  uncon- 
sciously associated  with  certain  places  or  with  certain  acts ;  thus  a 
person  may  yawn  in  church  constantly,  but  not  elsewhere  ;  he  may 
always  yawn  when  reading  aloud,  but  not  when  reading  to  himself ;  he 
may  yawn  in  the  presence  of  certain  persons  only ;  he  may  yawn  con- 
stantly if  he  is  seized  with  the  fear  that  he  may  yawn ;  like  all  tics, 
yawning  is  produced  by  seeing  another  person  yawn. 

Tic  of  the  neck  causes  a  shrugging  of  the  shoulders  or  a  turning  of 
the  head^  This  simulates  torticollis,  and  in  fact  Brissaud  describes  a 
mental  torticollis  which  is  a  psychical  form  of  wry-neck,  differing  in  some 
respects  from  the  other  forms.  In  these  cases  the  persuasion  that  some 
act  will  stop  the  spasm  is  followed  by  relief.  Thus  the  pressure  of  a 
finger  on  the  chin  or  on  the  side  of  the  face,  resting  the  head  on  the 
hand  or  on  a  chair  will  stop  such  a  tic.  The  majority  of  cases  of 
wry-neck  are  of  this  variety.     (See  p.  794.) 

Tics  of  the  extremities  are  rare,  the  habit  of  tapping  objects  with  the 
fingers,  or  of  kicking  with  the  feet  being  rather  of  the  nature  of  bad 
habits  than  of  true  tic. 

Tic  of  respiration,  of  cough,  and  of  swallowing,  have  been  recorded, 
and  I  have  recently  seen  a  tic  of  the  abdominal  muscles,  the  young 
woman  making  movements  of  the  hypogastric  region  constantly. 

Tics  of  speech  (echolalia)  in  which  the  patient  repeats  the  word  last 
heard,  or  repeats  some  word  over  and  over,  are  rather  to  be  classed 
with  hysterical  phenomena. 

In  all  cases  of  tic  the  mental  characteristics  of  the  patient  are  to  be 
studied,  it  being  kept  in  mind  that  there  is  a  psychical  element  in  these 
cases.  Fixed  ideas,  abnormal  impulses,  and  peculiar  ideas  are  often  to 
be  detected  by  questioning.  This  has  to  be  particularly  regarded  in 
the  treatment,  suggestion  and  persuasion  of  possible  relief  being  essen- 
tial to  success. 

Course.  —  The  usual  history  obtained  is  that  of  a  slow  onset  of  the 
tic  following  some  mental  shock  or  anxiety  —  its  gradual  development 
and  then  a  long  period  without  relief.  During  this  period  variations 
in  severity  from  time  to  time  occur.  Sometimes  there  is  spontaneous 
recovery.  Sometimes  treatment  cures.  In  a  few  cases  described  by 
Gilles  de  la  Tourette  there  is  a  gradual  extension  of  the  tic  from  one 
part  to  another.  The  disease  begins  at  the  age  of  six  or  eight  by 
tic  of  the  face ;  this  extends  to  the  neck  and  head  and  shoulders,  then 
to  the  hands  and  finally  to  the  trunk  and  legs.  Echolalia  and  respira- 
tory tic  are  added.  As  the  disease  goes  on  mental  failure  becomes 
apparent,  the  patients  show  queer  obsessions,  develop  delusions  and 
finally  go  on  to  dementia. 

The  prognosis  is  fairly  good  when  the  disease  begins  in  early  life, 
as  proper  mental  and  physical  training  usually  enables  the  child  to 
overcome  the  habit.     In  adults  the  prognosis  is  not  as  good,  for  tern- 


FACIAL    SPASM.  791 

porary  reqpvery  is  usually  followed  by  relapses,  and  finally  the  disease 
becomes  chronic.     The  disease  does  not  shorten  life. 

Etiology.  —  A  neuropathic  or  psychopathic  heredity  can  be  ascer- 
tained in  the  majority  of  cases  of  tic.  The  disease  is  often  directly 
inherited,  several  members  of  a  family  being  aifected  in  different  genera- 
tions. If  it  is  not,  a  history  of  epilepsy,  hysteria,  neurasthenic  im- 
pulses, phobias,  migraine,  or  diabetes  may  be  obtained,  or  some  organic 
nervous  disease  in  the  parent  may  be  found  to  exist.  Imitation  plays 
a  part  in  the  origin  of  the  disease,  as  many  children  copy  the  motions 
of  others.  Imperfect  training  is  also  responsible  for  some  cases. 
Children  are  very  liable  to  begin  spontaneously  some  bizarre  motion 
and  unless  firmly  corrected  or  punished  for  it  will  keep  it  up  till  it 
runs  into  a  tic. 

Tics  develop  chiefly  in  children  between  the  ages  of  five  and  ten. 
This  is  the  age  of  greatest  imitation  and  when  habits  are  easily  formed. 
Both  sexes  are  liable.  Some  cases  develop  at  puberty.  Earely  the 
disease  appears  in  old  age  and  then  it  is  usually  a  part  of  a  senile 
dementia. 

Treatment.  —  The  recognition  that  tics  are  psychomotor  in  origin 
has  led  to  the  only  satisfactory  method  of  treatment.  This  is  a  method 
of  self-control  and  self-education  or  psychomotor  discipline  devised  by 
Brissaud  and  Meige.  The  patient  must  keep  his  mind  on  his  spasm, 
must  attempt  to  control  it  voluntarily,  and  in  order  to  do  this  must 
go  through  regular  exercises  several  times  daily  before  a  mirror,  con- 
tracting the  muscles  which  twitch  and  their  opponents,  so  as  to  secure 
voluntary  control  of  the  muscles.  This  education  of  the  muscles  must 
be  insisted  upon  and  is  best  done  in  company  with  some  one  who  has 
a  firm  control  of  the  patient.  Sometimes  isolation  from  the  family  has 
a  good  effect  on  children.  A  system  of  rewards  for  success  aids  the 
treatment.  It  is  useless  to  give  medicine  (e.  g.,  arsenic),  or  to  a°ttempt 
surgical  treatment  by  division  of  nerves  or  tendons.  But  in  view  of 
the  neurotic  constitution  of  the  majority  of  patients  it  is  necessary  to 
advise  that  form  of  life  which  will  conduce  to  general  improvement  in 
health. 

FACIAL  SPASM. 

A  spasmodic  twitching  of  the  muscles  supplied  by  the  facial  nerve 
is  not  an  uncommon  affection.  It  is  usually  primary,  and  not  secon- 
dary to  facial  paralysis.  All  the  muscles  may  be  affected  by  the 
spasm,  or  it  may  be  limited  to  the  muscles  about  the  eye,  the  orbi- 
cularis palpebrarum,  when  it  is  called  "  blepharospasm."  Usually  the 
zygomatic  muscles  and  the  levator  auguli  oris  are  affected  with  the 
orbicularis  palpebrarum.  When  this  is  the  case  the  spasm  usually 
extends  to  the  muscles  of  the  chin.  It  is  a  unilateral  affection  at  first, 
but  may  become  bilateral.  The  slight  bilateral  twitching  of  the  face 
sometimes  seen  in  childhood  or  in  hysterical  individuals,  the  result  of 
voluntary  grimaces  which  have  become  automatic,  is  not  included  in 
facial  spasm. 

Facial  spasm  is  a  disease  of  adult  life,  not  usually  developing  until 


792  TEE    SPASMODIC   NEUBOSES. 

after  the  age  of  forty  years.  It  occurs  in  women  more  frequently  than 
in  men  and  much  more  commonly  in  persons  of  a  neuropathic  consti- 
tution or  in  those  who  are  subject  to  migraine,  anaemia,  or  cachectic 
conditions.     A  sudden  mental  shock  has  been  known  to  produce  it. 

A  spasmodic  contraction  of  the  muscles  anywhere  in  the  body  is 
rarely  evidence  of  primary  motor  irritation.  It  is  usually  secondary 
to  a  sensory  impulse,  the  contraction  of  the  muscles  being  a  response 
to  such  an  impulse.  Thus  the  act  of  winking  is  primarily  due  to  an 
irritation  of  the  conjunctiva,  and  blepharospasm  can  be  usually  traced 
either  to  some  local  disease  in  the  eyelids,  to  some  strain  of  the  ocular 
muscles,  or  to  some  defect  of  vision.  The  most  sudden  or  lightning- 
like contractions  in  the  facial  muscles  are  seen  in  connection  with  facial 
neuralgia,  and  it  is  probable  that  in  the  majority  of  cases  of  facial 
spasm  the  cause  is  to  be  found  in  some  irritation  in  the  domain  of  the 
trigeminal  nerve.  Thus  irritation  in  the  nose,  or  about  the  teeth,  or 
even  in  the  mouth  or  throat,  or  in  the  scalp,  is  sufficient  to  produce  a 
facial  spasm,  and  many  cases  can  only  be  relieved  by  the  removal  of 
the  source  of  irritation.  It  is  not  impossible  that  small  sensory  fila- 
ments of  the  trigeminal  nerve  may  be  the  seat  of  neuritis  without  any 
attendant  pain,  and  that  the  result  may  be  a  facial  tic.  In  the  severe 
spasm  of  torticollis  it  is  possible  for  an  extension  of  the  spasm  to  the 
facial  muscles  to  occur,  so  that  the  spasm  not  only  turns  the  head,  but 
twists  the  face.  Cases  have  been  recorded  of  facial  spasm  due  to  a 
pressure  of  tumors  on  the  facial  nerve  within  the  skull. ^  Facial  spasm 
has  also  been  observed  in  irritating  lesions  of  the  facial  area  in  the 
motor  cortex  of  the  brain,  but  these  spasms  are  rarely  as  constant,  as 
frequent,  as  sudden,  or  as  extreme  as  those  in  true  facial  spasm.  They 
are  usually  attended  by  spasms  in  the  neck  and  arm  and  by  other 
symptoms  of  cortical  disease.  Nevertheless,  it  is  well  to  remember 
that  a  facial  spasm  may  be  the  first  sign  of  cortical  epilepsy.     (See  page 

Symptoms.  —  The  chief  symptom  of  the  affection  is  the  sudden, 
lightning-like  twitching  of  the  muscles  of  the  face.  Such  twitching 
occurs  at  intervals,  or  a  series  of  twitches  may  occur  rapidly,  one  after 
another,  each  paroxysm  lasting  several  seconds,  or  even  a  minute. 
The  final  contraction  may  be  a  tonic  one,  so  that  the  face  is  drawn  up 
and  is  motionless  for  a  few  moments.  Any  attempt  at  looking  fixedly 
at  an  object,  or  moving  the  face,  or  talking,  or  chewing  is  liable  to  be 
followed  by  a  spasm,  and  sometimes  a  cold  draught  is  sufficient  to  bring 
it  on.  Some  patients  complain  that  the  spasm  is  set  up  by  a  strong 
light,  under  which  circumstances  it  is  possible  that  the  irritation  of  the 
optic  nerve  is  sufficient  to  set  up  a  contraction.  For  this  reason  many 
patients  protect  the  side  of  the  face  by  a  thick  woollen  pad,  which  keeps 
out  light  and  cold.  The  spasm  is  never  painful,  and  as  there  is  no 
paralysis,  voluntary  motion  is  perfectly  possible.  There  is  no  change 
in  the  electrical  contractility  of  the  facial  muscles.  In  one  case  re- 
corded by  Keen  ^  the  spasm  extended  from  the  facial  nerve  to  the  mus- 

^Schultze,  Virchow's  Archiv,  vol.  Ixv.,  p.  385.     Vuss,  Neurol.  Centralbl.,  1886. 
*  Transactions  of  the  American  Surgical  Association,  1886. 


FACIAL    SPASM.  793 

cles  of  mastication,  the  tongue,  and  muscles  of  the  neck.  Occasionally 
a  tender  spot  in  the  region  of  the  fifth  nerve  or  slight  areas  of  anses- 
thesia  may  be  discovered  by  careful  examination,  a  fact  which  sup- 
ports the  theory  that  the  facial  spasm  is  secondary  to  disease  in  the 
trigeminal  nerve. 

The  course  of  the  case  is  usually  a  very  slow  one.  The  spasm  begins 
in  a  few  muscles,  and  extends  to  others  until  the  entire  face  is  involved, 
and  the  disease  may  remain  for  many  weeks  or  months,  and  then  sud- 
denly cease.  It  is  particularly  liable  to  return.  Not  infrequently 
there  is  no  permanent  recovery.  The  patient  is  liable  to  the  spasm  all 
his  life.  Hence  the  prognosis  is  unfavorable,  especially  if  the  disease 
has  lasted  more  than  a  month. 

Diagnosis.  —  Facial  spasm  is  not  to  be  confounded  with  facial  tic, 
for  it  does  not  have  the  characteristics  of  a  tic.  It  is  a  sudden  reflex 
act,  chiefly  in  one  or  a  few  muscles,  unconscious  and  not  under  volun- 
tary control.  A  tic  is  a  complex  act  apparently  with  a  conscious  end  in 
view  and  involves  a  co5rdinated  movement  which  can  be  temporarily 
arrested.  The  facial  spasm  is  usually  a  surprise  to  the  patient.  Prior 
to  a  tic  the  patient  has  an  impulse  to  make  the  motion  which  gathers 
strength  until  he  can  no  longer  resist,  and  then  he  feels  relief  when 
the  motion  is  made. 

Treatment.  —  Treatment  should  consist  in  a  very  careful  attempt  to 
discover  some  source  of  reflex  irritation  by  examination  of  the  func- 
tions of  the  eye,  careful  investigation  of  the  throat  and  nose  and  of 
the  teeth  ;  and  the  removal  of  such  irritation  if  possible.  In  one  patient 
whose  entire  left  side  of  the  face  was  in  constant  spasm  examination 
showed  that  a  marked  defective  visual  power  in  the  right  eye  had 
thrown  all  the  work  of  vision  on  the  left  eye.  This,  too,  was  astigmatic 
and  myopic.  The  proper  adjustment  of  glasses  resulted  in  a  progres- 
sive cure,  complete  within  a  month,  although  the  condition  had  been 
present  for  over  a  year.  If  no  cause  is  found  the  disease  must  be 
treated  symptomatically  by  hot  applications  to  the  face  and  by  the  free 
use  of  sedatives,  of  which  bromide,  cannabis  indica,  morphine,  gelse- 
mium,  and  conium  are  the  most  useful.  Electricity  is  of  no  use. 
Counter-irritation  by  blisters  or  cautery  also  fails  to  relieve.  Stretch- 
ing of  the  facial  nerve,  which  has  been  attempted,  is  usually  followed 
by  paralysis,  but  when  this  subsides  the  twitching  returns. 

Some  cases  of  facial  spasm  are  of  interest  because  of  the  possibility 
of  locating  the  seat  of  irritation  by  means  of  hypodermic  injections  of 
cocaine.  In  two  cases  under  my  care  the  spasm  had  been  of  long  dura- 
tion and  had  given  the  patients  much  discomfort  and  had  failed  to  yield 
to  any  remedies.  In  neither  case  was  there  any  localizable  pain.  Inas- 
much as  the  majority  of  such  cases  of  facial  spasm  are  due  to  irritation 
in  the  course  of  the  trigeminal  nerve,  it  was  necessary  to  determine, 
first,  whether  the  spasms  were  reflex,  and,  second,  ifso,  in  which  branch 
of  the  trigeminal  nerve  the  irritation  began.  This  was  arrived  at  by 
injecting  cocaine  beneath  the  skin  of  the  face  successively  at  the  point 
of  emergence  of  the  supraorbital,  infraorbital,  and  dental  branches  of 
the  trigeminal  nerve.     In  the  first  case  the  injections  had  no  effect  on 


794  TEE    SPASMODIC    NEUROSES. 

the  spasm  when  the  upper  two  branches  were  anaesthetized,  but  the 
injection  at  the  mental  foramen  in  the  lower  jaw  was  succeeded  by 
immediate  stoppage  of  the  spasm  for  one-half  hour,  or  until  the  local 
effect  of  the  cocaine  had  subsided.  This  patient  was  then  operated 
upon  by  Hartley,  who  exsected  this  branch  of  the  trigeminal  nerve  at 
its  exit  from  the  jaw  with  the  effect  of  permanent  cure  of  the  spasm. 

In  the  second  case  injection  in  the  infraorbital  branch  of  the  nerve 
stopped  the  spasm,  and  here  division  of  this  branch  was  followed  by 
permanent  cure  of  the  spasm. 

SPASMODIC  TORTICOLLIS. 

Spasmodic  torticollis  -is  a  spasmodic  contraction  of  any  or  all  of  the 
muscles  moving  the  head  upon  the  trunk.  It  consists  of  a  quick,  sharp 
contraction  of  the  sternomastoid  or  of  the  trapezius  muscles,  causing  a 
sharp,  quick  turning  of  the  head  toward  one  side,  as  if  the  patient  were 
looking  over  his  shoulder.  In  some  cases  this  contraction  is  so  sudden 
as  to  cause  an  almost  lightning-like  twitch  of  the  head.  In  other  cases 
the  contraction  is  slower  when  the  head  is  turned  slowly,  but  none  the 
less  firmly  to  the  side.  Twitching  occurs  at  intervals,  may  take  but  a 
second,  and  be  succeeded  by  many  other  twitchings,  so  that  the  head 
turns  eight  or  ten  times  in  a  minute,  or  it  may  be  much  slower,  and  then 
the  head  is  frequently  held  for  a  minute  or  more  in  a  fixed  position. 
As  soon  as  a  relaxation  of  the  muscle  occurs  the  patient  voluntarily 
turns  the  head  back,  only  to  find  it  jerked  about  again  with  much 
force  and  persistency.  As  time  goes  on  in  this  affection  the  twitch- 
ings become  more  severe  and  either  more  rapid  or  more  prolonged  in 
duration.  The  latter  is  very  common,  so  that  these  patients  after  a 
time  find  the  head  permanently  fixed  in  the  abnormal  position,  though 
the  occasional  relaxation  of  the  spasm  allows  them  to  turn  the  head 
back  occasionally.  The  usual  form  of  torticollis  is  the  one  in  which 
the  sternomastoid  muscle  alone,  or  the  upper  fibres  of  the  trapezius 
alone,  or  these  two  muscles  together  are  affected.  If  the  sternomastoid 
alone  is  involved  the  chin  is  elevated  and  drawn  toward  the  opposite 
shoulder.  If  the  trapezius  alone  is  involved  the  chin  is  elevated  to  a 
greater  degree  and  the  head  is  thrown  backward.  If  both  muscles  act 
together  the  entire  head  is  turned  over  toward  the  affected  side  and 
rotated  with  the  chin  toward  the  opposite  shoulder.  The  affection  is 
liable  to  be  of  long  duration,  the  spasm  continuing  for  many  weeks, 
with  varying  degrees  of  intensity.      It  usually  relaxes  during  sleep. 

It  may  be  produced  by  any  voluntary  effort,  especially  the  acts  of 
reading  or  looking  fixedly  at  any  object  which  involves  the  voluntary 
fixation  of  the  head  or  eyes.  As  such  a  fixation  may  be  a  necessary 
act  in  the  pursuit  of  a  profession  or  work,  Cruchet,^  whose  studies  of 
torticollis  are  the  most  complete,  has  classified  these  cases  together  and 
calls  them  the  "professional  type."  He  thus  likens  them  to  writers' 
cramp  and  other  occupation  neuroses.  (See  page  829.)  He  affirms 
that  in  these  cases  the  spasm  occurs  only  when  the  muscles  of  the  neck 
*  Cruchet.     Traite  des  Torticolis.     Masson  &  Cie,  Paris,  1907. 


SPASMODIC    TOBTICOLLiS.  1^5 

are  called  ipto  action  by  the  effort  to  hold  the  head  steady,  as  in  fixing 
head  and  eyes  on  any  fine  manual  work  or  writing.  In  this  class  of 
cases  he  says  there  is  no  spasm  when  the  head  is  at  rest,  and  he  thus 
distinguishes  this  type  from  others. 

Patients  sometimes  find  that  there  is  some  point  upon  the  neck  or 
head,  pressure  upon  which  will  temporarily  arrest  the  spasm,  and  they 
are  inclined  to  produce  pressure  upon  this  point  either  by  resting  the 
head  upon  the  back  of  the  chair  or  lying  with  the  support  of  a  pillow 
or  compressing  this  point  by  the  hand.  Cruchet  holds  that  when  this 
is  so,  the  case  must  be  classed  with  the  tics,  and  he  attempts  to  estab- 
lish a  "  mental  type "  of  torticollis,  in  which  the  spasm  is  rather  a 
psycho-motor  act.  Brissaud  had  previously  distinguished  a  form  of 
torticollis  which  he  termed  hysterical  or  mental  because  of  the  fact  that 
some  insignificant  act  would  arrest  the  spasm  by  persuasion,  and  in  such 
cases  he  found  hypnotic  suggestion  of  use.  For  one  patient  who  had 
a  serious  form  of  spasmodic  torticollis  which  failed  to  respond  to  any 
treatment  I  devised  a  mechanical  apparatus  containing  a  lever  which 
pushed  a  pad  against  the  lower  part  of  the  occiput  upon  the  left  side, 
which  pad  could  be  made  to  press  more  firmly  by  a  movement  of 
adduction  of  his  arm,  and  this  apparatus  was  worn  for  many  months 
with  great  relief. 

The  spasm  is  not,  as  a  rule,  accompanied  by  any  pain  or  by  any  sen- 
sory disturbances,  though  the  muscles  may  become  wearied  by  their 
constant  contraction  and  may  feel  tired  and  stiff.  Occasionally  there 
is  acute  neuralgia  in  the  back  of  the  neck  or  in  the  occipital  nerve. 
Cruchet  considers  these  cases  as  reflex  in  origin  and  cites  instances  in 
which  relief  of  the  neuralgia  by  division  of  some  branch  of  the  occipital 
nerve  has  cured  the  spasm.  Curshmann  has  recorded  a  case  due  to 
irritation  of  the  auditory  nerve. 

The  act  of  walking  or  talking  may  intensify  the  spasm,  and  any 
attempt  at  effort  of  the  arms  occasionally  is  attended  by  an  increase  in 
the  contractions.  Thus  one  patient  at  present  under  my  observation 
is  prevented  from  writing  by  the  occurrence  of  the  spasm.  The  muscles 
that  are  affected  may  be  seen  and  felt  to  contract,  but  show  no  changes 
to  the  electrical  reaction. 

The  spasm  may  extend  from  the  sternomastoid  and  trapezius  muscles 
to  any  or  all  of  the  deeper  muscles  moving  the  head.  The  splenius 
is  affected  in  many  cases,  and  in  a  few  cases  the  deeper  muscles  of  the 
back  of  the  neck  are  all  involved  in  the  spasm.  Under  these  circum- 
stances it  is  not  unusual  for  the  spasm  to  extend  to  the  opposite  side 
or  to  affect  the  superficial  muscles  on  one  side  and  the  deeper  muscles 
on  the  other,  which  act  conjointly  in  turning  the  head.  Symmetrical 
muscles  are  rarely  affected.  If  they  are  they  usually  act  in  harmony, 
producing  a  throwing  of  the  head  forward  upon  the  chest  or  a  turn- 
ing of  it  backward  and  an  elevation  of  the  chin.  Sometimes  in  seri- 
ous cases  of  long  duration  the  spasm  extends  to  the  muscles  moving 
the  slioulder,  the  rhomboids,  the  levator  anguli  scapulae,  or  the  ser- 
ratus  or  pectorals;  and  in  these  cases  movements  of  the  arm  are  fre- 
quently associated  with  those  of  the  head.     (See  Fig.  28G.)     The  arm 


796 


TEE    SPASMODIC    tJEUEOSE^. 


may  be  adducted  and  flexed,  wrist  and  fingers  flexed,  or  it  may  be 
thrown  backward  behind  the  body.  Occasionally  these  spasms  be- 
come so  intense  that  the  muscles  of  the  back  are  also  implicated,  and 
the  patient  is  no  longer  able  to  walk  in  an  upright  position  and  even 
while  lying  down  is  drawn  around  to  one  side,  the  curve  of  the  spine 
being  convex  toward  the  unaifected  side.  This  I  have  seen  in  one 
chronic  case  which  lasted  for  five  years  and  resisted  all  forms  of  treat- 
ment including  division  of  the  eleventh  nerve.  In  cases  where  the 
head  is  thrown  backward  by  contraction  of  the  trapezii  there  is  an 
associated  movement  of  the  frontalis  muscle,  and  then  the  patient  not 
only  elevates  the  chin  but  has  the  appearance  of  looking  upward 
forcibly,  the  eyebrows  being  raised.  As  time  goes  on  the  muscles 
that  are  afiected  increase  in  size  on  account  of  their  constant  exercise, 
so  that  the  neck  becomes  perceptibly  larger. 

The  course  of  the  disease  is  a  chronic  one.  The  spasm  begins  in 
slight  degree,  increases  in  intensity,  in  frequency  of  attack  and  in 

duration  of  attack,  so  that  within 
Fig.  306.  three    months    of    the    onset    the 

patient's  life  becomes  a  burden  and 
all  occupation  has  to  be  abandoned. 
In  a  few  cases  after  a  period  of 
two  or  three  mouths  the  spasms 
gradually  diminish  in  frequency 
and  cease,  but  they  are  particu- 
larly liable  to  recur  from  time  to 
time,  and  these  patients  suffer  from 
attacks  of  spasmodic  torticollis 
throughout  their  lives.  The  dis- 
ease may  occur  after  any  illness. 
In  many  cases  it  continues  for  over 
a  year  with  little  intermission,  some- 
times being  moderate  in  degree,  at 
other  times  increasing  in  intensity. 
Even  the  more  severe  cases  occa- 
sionally recover,  the  spasms  gradu- 
ally subsiding  and  finally  disap- 
pearing. It  is  not  attended  by 
convulsions  or  by  cortical  epilepsy, 
though  I  have  occasionally  seen  it 
accompanied  by  considerable  men- 
tal agitation,  a  distinctly  hysterical  mental  condition  developing  as  a 
result  of  the  irritation  of  the  continuous  spasm. 

Cases  recorded  by  Brodie  and  Gowers  in  which  the  spasm  ceased  dur- 
ing an  attack  of  insanity  are  interesting. 

There  are  no  pathological  observations  to  determine  the  exact 
cause  of  torticollis.  The  disease  is  not  primarily  muscular  nor  is  it 
to  be  ascribed  to  any  lesion  of  the  eleventh  nerve  or  motor  nerves  to 
the  muscles,  inasmuch  as  irritation  of  the  motor  nerves  does  not 
cause  spasm.     It  may  be  regarded  as  reflex  in  some  cases,  like  facial 


Tonic  spasm  of  right  trapezius,  rhomboid  and 
levator  auguli  scapulae,  of  two  years'  duration, 
following  whooping-cough. 


SPASMODIC    TORTICOLLIS.  797 

spasm  and  blepharospasm,  as  the  result  of  a  sensory  irritation  in  some 
one  of  the  sensory  nerves  of  the  neck.  In  these  cases  neuralgia  may 
precede  the  attack.  It  has  been  known  to  occur  as  the  early  symptom 
in  spinal  caries  and  in  meningitis  or  tumors  of  the  upper  part  of  the 
spinal  cord.  In  the  vast  majority  of  the  cases  no  cause  can  be  ascer- 
tained. The  hypothesis  of  a  cortical  origin  of  this  spasm  receives 
a  certain  amount  of  support  from  the  fact  that  the  movements  are  quite 
similar  to  those  produced  by  volitional  effort,  and  in  many  cases  mus- 
cles which  lie  upon  opposite  sides  of  the  body,  and  are  supplied  by 
various  nerves,  join  to  produce  the  movement.  The  fact  that  the  eyes 
are  not  uncommonly  turned  involuntarily  to  the  side  toward  which 
the  face  looks  and  that  the  eyebrows  are  elevated  when  the  head  is 
turned  upward  supports  this  hypothesis.  When  the  arm  is  involved 
the  motions  produced  are  such  as  would  be  produced  in  a  normal  in- 
dividual straining  to  move  the  head  in  an  extreme  position  of  rotation. 
Anyone  can  substantiate  this  fact  who  makes  extreme  voluntary  eiforts 
to  turn  the  head  from  side  to  side. 

Etiology.  —  The  disease  is  more  common  in  women  than  in  men. 
It  occurs  in  adult  life  between  thirty  and  fifty  years,  though  no  age  is 
entirely  exempt.  It  usually  occurs  in  persons  predisposed  to  nervous 
disease  by  inheritance  or  subject  to  other  forms  of  nervous  affection, 
such  as  neurasthenia,  migraine,  or  psychosis.  It  is  not  analogous  to 
chorea  nor  does  it  follow  attacks  of  chorea  or  facial  tic.  Occasionally 
it  has  been  known  to  develop  after  a  fall  or  an  injury  in  which  the 
head  was  forcibly  twisted.  It  has  been  known  to  occur  after  exposure 
to  cold  and  after  an  attack  of  rheumatism  in  the  muscles  of  the  neck. 
It  has  been  known  to  follow  otitis  media  and  disease  of  the  vestibule 
and  labyrinth,  and  in  very  many  cases  some  defect  of  vision  has  been 
discovered  which  indicates  that  eyestrain  may  be  a  factor.  It  has  been 
ascribed  to  insufficiency  of  the  ocular  muscles  and  also  to  myopia  and 
hypermetropia.  Both  these  conditions  undoubtedly  cause  an  involun- 
tary straining  of  the  muscles  of  the  neck  in  the  act  of  vision,  and  occip- 
ital headache.  None  of  these  causes,  however,  seems  to  me  of  sufficient 
moment  to  originate  the  disease.  I  am  disposed  to  regard  it  as  usually 
a  cortical  affection  dependent  upon  some  irritating  cause  acting  upon 
the  cortical  centres  which  move  the  head.  This  irritating  cause  may 
be  exhaustion  from  voluntary  overaction  of  the  muscles  consequent 
upon  defects  of  vision,  and  it  may  be  considered  as  an  affection  anal- 
ogous to  the  many  forms  of  occupation  neuroses.  If  this  theory  is 
accepted  the  functional  disturbance  is  to  be  located  in  the  entire  ner- 
vous mechanism  involved  in  the  movement  of  the  head,  the  sensory 
elements  and  the  motor  elements  being  equally  involved  and  thrown 
into  a  state  of  hypersensitiveness  which  results  in  slight  sensations  pro- 
ducing undue  movements. 

Prognosis.  —  The  prognosis  in  wryneck  must  be  given  with  great 
caution,  as  the  history  of  various  cases  shows  such  varied  course. 
The  more  sudden  the  onset,  the  more  intense  the  spasm,  the  more 
widespread  the  distribution,  and  the  longer  its  duration  the  worse  the 


798  '  THE    SPASMODIC   NEUROSES. 

prognosis.  The  younger  the  patient,  the  less  severe  the  affection,  the 
greater  the  intervals  between  attacks,  and  the  less  the  discomfort 
appreciated  the  more  likely  is  recovery. 

Treatment.  —  The  treatment  of  torticollis  is  unsatisfactory  because 
we  are  ignorant  of  the  cause  of  the  disease.  Various  forms  of  nerve 
sedatives  have  been  used.  I  have -found  but  little  relief  from  large 
doses  of  bromide  or  from  bromide  and  chloral.  Valerianate  of  zinc 
and  asafoetida  have  given  somewhat  better  results,  but  the  best  effects 
have  been  obtained  from  large  doses  of  tincture  of  belladonna,  which 
may  be  gradually  increased  up  to  full  physiological  effect  and  usually 
cause  a  distinct  improvement  in  the  condition.  Cannabis  indica  has 
also  been  of  some  service  in  the  treatment  of  this  affection.  When 
the  spasm  is  very  severe  and  causes  great  distress  opium  in  some  form 
may  be  used,  in  the  extreme  cases  by  hypodermic  injections  of  mor- 
phine. This  usually  gives  considerable  relief,  but  unfortunately  leads 
to  the  morphine  habit,  to  which  several  of  my  patients  have  succumbed. 
Hypodermic  injections  of  atropine,  ^w  g^'^in  increased  slowly  up  to 
-^  grain  once  daily,  into  the  muscles  affected  have  given  relief  in  some 
cases,  but  have  failed  in  others.  Electricity  has  no  effect  whatever. 
If  it  is  tried  it  should  be  in  the  form  of  a  mild,  continuous  galvanic 
current,  as  it  is  futile  to  produce  contractions  in  muscles  already  in  a 
state  of  spasm.  Attempts  to  strengthen  the  opposing  muscles  by  elec- 
trical exercise  should  not  be  made,  for  even  the  greatest  voluntary  con- 
traction in  the  healthy  muscles  is  incapable  of  overcoming  the  involun- 
tary spasm.  Counter-irritation  to  the  back  of  the  neck  by  means  of 
blisters  and  by  the  actual  cautery  not  uncommonly  gives  a  certain 
amount  of  temporary  relief  and  may  be  used  frequently  if  the  skin  be 
not  injured.  Mechanical  supports  of  all  sorts  are  involuntarily  sought 
for  by  the  patient,  and  apparatus  may  be  devised  for  producing  a  con- 
stant support  of  the  head,  a  rod  carrying  a  pad  being  attached  to  a 
corset  and  thus  held  in  position. 

Torticollis  has  been  treated  by  surgeons  by  division  of  the  tendons 
of  the  muscles.  This  affords  temporary  relief,  but  does  not  stop  the 
spasm,  which  returns  as  soon  as  the  tendons  have  united.  It  has  also 
been  treated  by  division  or  by  cutting  out  a  portion  of  the  eleventh 
nerve  where  it  enters  the  sternomastoid  muscle,  or  deep  in  the  neck 
just  at  its  exit  from  the  skull.  These  measures  certainly  stop  the 
spasm  in  the  muscles  supplied  by  the  nerve  divided,  but  very  often 
after  the  division  of  the  nerve  the  motion  continues,  and  then  it  is  dis- 
covered that  the  muscles  lying  deep  in  the  neck  upon  the  same  side  or 
upon  the  opposite  side  have  originally  been  affected  by  the  spasm. 
Keen  has  divided  the  upper  four  cervical  nerves  in  efforts  to  paralyze 
these  deeper  muscles,  an  operation  involving  considerable  difficulty  on 
account  of  hemorrhage  and  on  account  of  the  depth  to  which  the  wound 
must  be  carried.  In  some  cases  this  has  given  relief,  but  after  the 
nerves  have  regenerated  the  spasm  has  returned.  In  a  discussion 
before  the  American  Neurological  Association  in  1897  the  consensus 
of  opinion  was  against  surgical  treatment,  yet  all  agreed  that  it  afforded 
the  only  prospect  of  temporary  relief  in  severe  cases. 


PABAMYOCLONUS   MULTIPLEX.  799 

SPASMODIC  TORTIPELVIS. 

A  spasm  of  the  muscles  attached  to  the  pelvis,  causing  peculiar 
postures  on  standing  and  a  very  irregular  gait,  with  twisting  of  the 
trunk,  bending  of  the  body  and  in  extreme  cases  a  tendency  to  walk 
on  "  all  fours "  has  been  described  by  various  writers  ^  under  the 
names  of  tortipelvis,  dysbasia  lordotica,  and  dystoma  musculorum 
deformans.  When  recumbent  the  spasms  cease.  On  attempting  to 
walk  they  appear,  throwing  the  balance  out  and  leading  to  remarkable 
positions  and  actions,  which  are  so  extraordinary  as  to  lead  to  the  idea 
of  intentional  simulation  of  disease.  Several  such  cases  have  been 
considered  hysterical,  a  suspicion  confirmed  by  the  success  of  treatment 
by  suggestion.  The  symptom  is  not  allied  to  athetosis,  because  it 
ceases  with  voluntary  effort.  It  is  not  attended  by  paralysis,  electrical 
change,  anaesthesia  or  permanent  deformity.  Occasionally  the  muscles 
of  the  arms  and  trunk  are  also  involved  in  the  spasm.  In  Oppen- 
heim's  cases  the  reflexes  were  diminished  and  the  muscles  seemed  to 
lack  a  proper  tone.  The  condition  is  to  be  grouped  with  the  tics,  but 
because  of  its  peculiar  type  has  received  a  special  name. 

PARAMYOCLONUS  MULTIPLEX. 

Paramyoclonus  multiplex  is  a  spasmodic  affection  of  the  muscular 
system,  occurring  bilaterally  in  symmetrically  situated  muscles  attached 
at  one  or  both  ends  to  the  trunk,  and  in  muscles  whose  function  is  as- 
sociated with  these,  consisting  of  a  series  of  violent  clonic  spasms  of  con- 
siderable rapidity  and  severity,  occurring  only  at  intervals ;  and  associ- 
ated with  fascicular  tremors  of  the  affected  muscles,  persisting  during  the 
interval  between  the  spasms.  It  is  not  accompanied  by  any  distur- 
bance of  sensory  or  motor  functions,  excepting  by  an  increase  of  the 
superficial  and  deep  reflexes.  The  spasms  can  be  excited  by  irritation 
of  the  skin  or  tendons. 

The  causation  is  uncertain ;  fright  and  anxiety  or  muscular  strain 
in  lifting  weights  or  walking  have  preceded  the  onset  in  some  cases. 
Males  in  adult  life  are  more  commonly  affected. 

Symptoms.  —  The  symptoms  are  quite  characteristic.  The  spasms 
are  bilateral  and  symmetrical.  They  are  limited  to  certain  muscles. 
In  many  cases  the  quadriceps  femoris  and  flexors  of  the  leg,  and  the 
so-called  upper  arm  group  of  muscles,  the  deltoid,  biceps,  and  supinator 
longus,  were  affected.  In  some  cases  the  muscles  of  the  back  were 
involved,  and  the  muscles  of  the  neck  contracted.  In  a  few  cases  the 
glutei  and  the  face  and  the  diaphragm  were  involved.  In  no  case 
have  the  muscles  of  the  hands  or  forearms,  of  the  feet  or  legs  been 
affected.  The  usual  limitation  of  the  spasm  to  the  body  muscles  with 
those  of  the  thighs  and  arms  is  very  noticeable. 

The  character  of  the  spasm  is  also  characteristic.     It  is  a  rapidly 

^  Leszinsky,  Jour.  Nerv.  and  Ment.  Disease,  1904,  p.  33,  Oppenheim,  Neurol.  Cen- 
tralbl.,  1911,  p.  1090. 


800  THE    SPASMODIC    NEUBOSES. 

repeated  clonic  spasm  occurring  at  intervals.  In  six  cases,  the  rate 
of  contraction  has  been  counted.  It  has  varied  from  50  per  minute 
to  180  per  minute.  In  one  of  my  cases  it  was  about  90.  It  is  not  a 
sudden,  single  irregular  muscular  contraction,  like  that  of  chorea,  but 
appears  to  be  always  bilateral  and  to  involve  several  muscles  of  a 
physiological  group  at  once,  thus  resulting  in  a  series  of  movements, 
any  one  of  which  can  be  voluntarily  made.  In  several  cases  a  tonic 
contraction  has  occurred  in  one  or  more  of  the  muscles  affected,  before 
or  during  the  clonic  seizure.  In  one  of  my  cases  the  spasm  of  the 
diaphragm  was  tonic  for  one-eighth  to  one-quarter  of  a  minute  during 
each  attack,  and  in  the  early  attacks,  the  spasms  of  the  back  were  tonic 
for  some  seconds.  The  clonic  contractions  continue,  when  once  set  up, 
for  a  varying  time,  from  half  a  minute  to  ten  minutes,  and  are  suc- 
ceeded by  a  complete  interval  of  freedom  from  spasm.  This  interval 
varies  from  half  an  hour  to  about  one  week.  During  the  spasm  itself, 
the  resulting  movements  are  of  a  very  violent  nature.  The  head  is 
thrown  about  by  the  movements  of  the  body,  rendering  the  patient 
dizzy.  The  body  is  tossed  about  in  the  chair,  so  that  there  seems  to 
be  danger  of  the  patient  being  thrown  out  upon  the  floor.  If  the 
spasm  occurs  while  he  is  walking,  he  is  quite  liable  to  be  thrown  down. 
But  this  violence  is  not  always  present  —  for  in  two  cases  the  spasms 
were  never  severe  enough  to  cause  a  movement  of  the  joints,  and  were 
only  observed  when  the  patient  was  stripped  —  being  then  of  the  nature 
of  a  fascicular  twitching.  In  one  case,  such  a  fascicular  muscular 
twitching  was  occasionally  seen  during  the  intervals  in  the  muscles  of 
the  back  and  the  pectorals. 

In  the  majority  of  the  cases,  any  tapping  of  the  tendons  or  any  irri- 
tation of  the  skin  was  sufficient  to  produce  a  spasm.  This  seems  to  be 
an  important  point,  for  I  am  not  aware  that  it  has  been  observed  in 
hysterical  or  choreic  spasms.  It  is  true  that,  in  hysterical  cases,  cer- 
tain zones  or  areas  can  occasionally  be  found  on  the  body,  irritation  of 
which  may  cause  or  may  arrest  the  attack.  But  in  this  condition  the 
spasm  is  produced  by  irritation  anywhere  on  the  skin  —  or  by  tapping 
the  tendons  at  the  knee  and  ankle  —  and  was  not  associated  with  dis- 
turbances of  sensation,  which  are  characteristic  of  hysterical  zones. 
The  knee-jerk  has  been  increased  in  about  one-half  of  the  cases.  The 
skin  reflexes  were  also  increased  in  some  cases.  Mental  excitement 
seems  to  have  predisposed  to  the  onset  of  the  spasm  in  three  cases. 
Had  the  disease  been  hysterical  in  nature,  this  would  probably  have 
been  observed  in  a  larger  proportion.  Voluntary  effort  stopped  the 
spasm  in  four  cases  and  made  it  worse  in  three  cases.  Had  the  dis- 
ease been  hysterical,  volition  would  probably  not  have  influenced  it 
favorably  in  the  majority  of  cases.  The  spasm  has  ceased  during  sleep 
in  four  cases,  but  has  continued  in  one  case. 

In  none  of  the  cases  have  consciousness,  motion,  sensation,  coordi- 
nation, or  electric  excitability  been  in  any  way  affected  —  an  important 
negative  fact,  since  it  proves  at  once  that  the  condition  is  a  functional 
neurosis,  and  makes  it  very  unlikely  that  it  is  of  an  epileptic  or  an 


PAEAMYOCLONUS    MULTIPLEX.  801 

hysterical  nature.  In  one  case,  which  died  of  phthisis,  a  careful  exami- 
nation by  Professor  Schultze,  of  Heidelberg,  failed  to  reveal  any  lesion 
of  the  nervous  system. 

It  is  evident  from  this  review  of  the  symptomatology  that  the  char- 
acteristics of  the  disease  are  quite  distinct ;  that  it  can  be  diiferentiated 
from  chorea,  from  hysteria,  and  from  epilepsy.  Is  there  any  disease 
known  which  it  at  all  simulates  ?  In  tic  convulsif,  we  have  an  affec- 
tion consisting  of  spasmodic  contractions  of  irregular  intensity  and  fre- 
quency, often  attended  by  intervals  of  freedom.  The  resemblance  to 
paramyoclonus  multiplex  is  more  than  superficial,  and  has  been  noticed 
by  several  writers.  But  all  seem  to  agree  that  in  tie  convulsif  the  face 
is  usually  chiefly,  if  not  exclusively,  aifected ;  that  the  contractions  are 
often  single  and  unilateral,  are  liable  to  occur  during  voluntary  motion ; 
that  their  intensity  is  not  varied,  but  is  quite  uniform ;  that  the  spasm 
is  not  produced  or  increased  by  external  influences ;  and  that  it  is 
always  a  coordinate  volitional  motion  which  is  produced.  Gilles  de  la 
Tourette,  it  is  true,  has  described  a  maladie  des  ties  convulsif s,  in  which 
title  he  wishes  to  include  those  spasmodic  affections  described  by  various 
authors  as  jumping,  coprolalia,  myriachit.  But  here  again  there  is  a 
wide  difference  from  paramyoclonus  multiplex.  It  seems,  therefore,  as 
though  the  disease  must  be  regarded  as  distinct  from  tic  convulsif  and 
as  having  a  character  of  its  own. 

There  is  a  condition  described  by  Henoch  as  chorea  electrica  which 
in  some  respects  resembles  paramyoclonus.  Henoch  says  that  in 
chorea  electrica  we  have  a  combination  of  true  choreic  movements 
with  clonic  twitchiugs.  The  patients  are  quiet,  and  lightning-lijce 
twitches  occur  from  time  to  time,  perhaps  every  five  minutes  or  more 
frequently,  especially  on  the  muscles  of  the  neck  and  shoulders. 
There  is  also  seen  a  twitching  of  individual  muscles  when  the  body  is 
naked,  not  sufficient  to  move  the  limbs.  These  continue  during 
sleep.  The  disease  occurs  between  the  ages  of  nine  and  fifteen,  and  is 
a  manifestation  of  direct  or  reflex  irritation  of  the  nervous  centres. 
The  spasms  affect  the  head,  hands  and  feet,  and  implicate  all  the 
voluntary  muscles,  including  the  diaphragm  and  larynx,  so  that  the 
patient  may  growl  and  bark.  It  is  evident  that  while  the  character 
of  the  spasm  resembles  that  in  paramyoclonus,  the  distribution  of  the 
spasm  is  more  extensive  in  chorea  electrica. 

Prognosis.  —  In  regard  to  the  prognosis,  it  may  be  said  that  this  is 
favorable.  The  majority  of  the  cases  have  recovered  quite  rapidly 
under  treatment.     In  some  cases,  however,  relapses  have  occurred. 

Treatment.  —  The  treatment  which  has  been  of  most  service  has 
been  the  application  of  strong  galvanic  currents  to  the  spine  and  neck, 
and  the  application  of  the  anode  to  sensitive  points  in  case  these  exist. 
Many  nerve  sedatives  have  been  used,  and  also  nerve  tonics.  The 
exact  effect  of  these  seems  to  be  doubtful.  In  my  own  case,  sedatives, 
tonics,  and  electrical  applications  had  all  been  equally  futile  to  arrest 
the  attacks,  but  the  patient  had  improved  to  a  considerable  degree 
under  the  varied  treatment.  Under  galvanism  to  the  spine,  arsenic 
61 


802  THE    SPASMODIC    NEUE08ES. 

and  chloral,  recovery  occurred.     The  hypodermatic  use  of  arsenic  in 
the  form  of  cacodylate  of  soda  deserves  a  trial. 

It  is  useless  to  discuss  the  nature  of  the  disease  from  so  few  cases 
as  are  at  our  disposal.  It  has  been  regarded  as  a  functional  neurosis, 
and  to  this  all  must  agree,  both  on  account  of  the  absence  of  any 
lesion,  in  one  case  examined  by  the  most  competent  neuro-pathologist 
in  Germany,  and  on  account  of  the  absence  of  symptoms  of  organic 
disease  and  the  recovery  of  the  cases.  Whether  it  has  a  central  origin 
and  is  produced  by  a  hyper-excitability  of  the  brain  or  spinal  cord, 
induced  by  the  sudden  vaso-motor  spasm  accompanying  fright  or 
mental  or  physical  strain,  as  Friedreich  believed,  or  whether  it  may  be 
a  reflex  spasm  due  to  some  peripheral  irritation  which,  being  conveyed 
to  the  spinal  and  medullary  centres,  produces  the  spasm  reflexly,  as 
another  author  has  suggested,  remains  for  the  future  to  decide. 

MYOCLONUS  EPILEPSY. 

This  disease,  first  described  by  Unverricht,  and  lately  studied  by 
Lundborg,  is  an  exceedingly  rare  affection  combining  some  of  the 
features  of  myoclonus  with  the  condition  of  epilepsy.  It  is  usually 
found  in  several  members  of  the  same  family,  though  direct  inheritance 
has  not  been  observed.  It  begins  in  childhood  and  is  chronic  in  its 
course.  It  begins  as  a  nocturnal  epilepsy  the  attacks  at  first  occur- 
ring but  seldom,  but  later  increasing  in  frequency.  The  patient  does 
not  always  lose  consciousness ;  but  often  awakens  with  a  painful  tonic 
or  clonic  and  tonic  cramp  in  different  muscles.  Sometimes  rapid  alter- 
nate muscular  contractions  occur  in  the  extremities.  Sometimes  the 
contractions  are  so  continuous  as  to  make  the  limb  rigid  as  in  tetanus. 
Any  attempt  to  move  the  limb  increases  the  spasm  and  is  painful.  As 
these  attacks  increase  in  frequency  a  second  stage  of  the  disease  is 
reached  in  which  symptoms  persist  between  the  attacks.  A  tremor 
is  always  present,  with  fibrillary  fascicular  contraction  in  the  muscles, 
beginning  in  the  hands  and  gradually  extending  to  involve  all  the 
voluntary  muscles  of  the  body.  This  tremor  varies  somewhat  from 
day  to  day,  and  alternates  with  quick  lightning-like  twitches  in  the 
various  muscles.  The  tremor  stops  during  sleep.  It  is  increased  by 
effort.  A  general  stiffness  and  rigidity  of  the  muscles  finally  develops. 
The  knee-jerks  are  exaggerated.  An  increase  of  saliva  and  of  perspi- 
ration has  been  noted.  Sensory  symptoms  are  absent.  After  some 
years  a  gradual  failure  of  mental  power  is  observed,  and  hallucinations 
or  states  of  exaltation  or  depression  have  occurred  in  some  cases.  The 
patients  are  finally  confined  to  bed  and  die  of  cachexia. 

MYOTONIA  CONGENITA.     THOMSENS  DISEASE. 

This  disease,  described  in  1876  by  Thomsen,^  who  was  himself 
affected,  should  hardly  be  included  in  a  treatise  on  nervous  diseases, 

1  Archiv  f.  Psych.,  Bd.  vi.,  s.  702 ;  also  Bd.  xxiv.,  s.  918. 


MYOTONIA    CONGENITA.  803 

but  may  be  mentioned,  inasmuch  as  it  produces  a  certain  amount  of 
paralysis.  It  is  a  congenital  and  inherited  disease  of  the  muscles, 
characterized  by  a  marked  hypertrophy  of  muscular  fibres  and  a 
proliferation  of  the  muscular  nuclei.  The  muscular  fibres  diifer  in 
shape  from  the  normal,  being  more  circular  and  not  polygonal  in 
transverse  section.^ 

Symptoms.  —  The  symptoms  of  the  affection  are  a  peculiar  rigidity 
of  the  muscles  occurring  after  they  have  been  at  rest  for  some  time. 
The  muscle  is  so  firmly  contractured  that  the  patient  cannot  overcome 
the  tonic  spasm,  and  hence  moves  with  great  difficulty  until  the  spasm 
relaxes.  All  the  muscles  of  the  body  may  be  affected,  and  hence 
walking,  running,  and  the  use  of  the  hands  are  very  much  hampered. 
The  quick  compensatory  and  automatic  movements  necessary  in  order 
to  preserve  the  balance  are  so  markedly  interfered  with  that  these 
patients  constantly  lose  their  equilibrium  and  are  liable  to  falls.  By 
repeated  attempts  at  movement  and  by  constant  exercise  these  muscles 
may  be  limbered  up,  so  that  at  the  end  of  a  long  walk  these  patients 
may  walk  in  a  normal  manner,  but  any  period  of  rest  or  the  ordinary 
rest  of  night  is  always  followed  by  a  renewal  of  the  hard  contraction 
of  the  muscles.  Patients  are  hampered  all  their  lives  by  this  abnormal 
rigidity.  Cold  or  damp  weather,  cold  baths,  great  mental  excitement, 
and  sudden  sensory  impressions  are  said  to  increase  the  degree  of  the 
spasm.  Active  exercises  decrease  it.  The  patients  have  no  other 
symptoms  whatever,  and  hence  the  disease  cannot  be  mistaken  for 
anything  else.  The  electrical  contractility  in  the  muscles  is  distinctly 
altered,  a  tonic  contraction  being  produced  by  faradic  excitement,  and 
the  reaction  of  degeneration  being  discovered  on  galvanic  examination. 
Erb  describes  undulating  rhythmical  contractions  starting  from  the 
cathode  and  passing  to  the  anode.  Mechanical  excitability  is  increased, 
percussion  of  the  muscle  producing  a  sharp  contraction  of  the  muscle 
which  remains  for  some  time  (fifteen  to  twenty  seconds),  producing  a 
groove  upon  the  skin.  Pressure  upon  the  muscles  will  also  produce  a 
firm  contraction.  As  but  thirty  cases  have  been  recorded  in  literature 
up  to  the  present  time,  the  affection  may  be  considered  rather  a 
curiosity.     There  is  no  known  treatment. 

^  Jacoby,  Journal  of  Nervous  and  Mental  Disease,  March,  1887. 


CHAPTER   XLVII. 

TETANY. 

Tetany  is  a  spasmodic  affection  of  the  muscles  of  the  extremities, 
attended  by  a  characteristic  and  abnormal  excitability  of  both  muscles 
and  nerves  to  mechanical  and  electrical  stimulation. 

Etiology.  —  It  is  a  disease  usually  observed  in  children,  and  boys 
are  more  subject  to  it  than  girls.  It  may  occur,  however,  in  adult 
life,  rarely,  if  ever,  in  old  age.  It  is  probably  a  toxic  neurosis,  due 
to  the  presence  in  the  blood  of  some  product  of  gastric  or  intestinal 
indigestion.  It  is  most  common  in  children  suffering  from  rickets, 
and  usually  develops  in  the  spring  months,  in  children  who  have  a 
poor  digestion.  It  may  occur  in  connection  with  severe  diarrhoea ; 
with  gastric  dilatation  and  gastro-enteritis  ;  with  the  existence  of  intes- 
tinal worms ;  and  sometimes  after  typhoid  and  other  infectious  fevers. 
In  adults,  it  may  occur  as  a  complication  of  pregnancy,  or  with  lacta- 
tion. It  has  been  seen  after  poisoning  with  chloroform,  morphine, 
ergot  and  lead.  It  may  develop  in  patients  who  have  had  the  thyroid 
gland  removed.  It  occurs  as  an  epidemic  in  the  spring  in  Austria, 
and  chiefly  among  shoemakers  and  tailors.  A  similar  epidemic  has 
been  observed  in  New  York.  This  is  probably  due  to  a  poison  taken 
into  the  system  from  some  article  used  in  their  trades. 

Symptoms.  —  The  disease  begins  with  numbness  and  parsesthesise 
of  the  hands  and  feet,  attended  by  burning  sensations.  Then  suddenly 
the  spasmodic  contractions  of  the  hands  and  feet  appear.  The  thumb 
and  fingers  are  closely  pressed  together  at  their  tips,  making  the  hand 
assume  a  cone  shape,  though  sometimes  the  thumb  is  flexed  in  the  palm. 
Or  the  hand  may  assume  the  claw  shape  seen  in  progressive  muscular 
atrophy.  The  wrist  is  flexed  and  adducted,  and  the  forearm  is  held 
across  the  chest  by  the  flexion  of  the  elbow  and  adduction  of  the  arm. 
Both  arms  are  equally  affected.  The  feet  share  in  the  spasm,  being 
arched  and  inverted,  the  ankles  extended  and  the  legs  held  in  rigid 
extension.  In  a  few  cases  the  trunk  is  affected,  the  body  being  bent 
forward,  as  a  rule.  Occasionally  the  jaw  is  fixed,  and  the  facial  mus- 
cles become  rigid :  the  muscles  of  the  pharynx,  larynx  and  tongue 
may  also  be  thrown  into  a  spasm.  Convergent  strabismus  has  been 
observed.  The  muscles  of  respiration  are  seldom  involved.  The 
extent  of  the  spasm  differs  in  different  cases ;  in  some  it  is  limited  to 
the  hands ;  in  others  it  involves  many  muscles.  The  intensity  of  the 
spasm  varies.  It  may  last  only  a  few  minutes,  and  be  so  slight  that 
voluntary  effort  can  overcome  it.     In  other  cases  it  lasts  longer,  even 

804 


TREATMENT.  805 

several  ds^s,  and  the  spasm  is  so  severe  that  no  force  from  without 
can  stop  it.  It  comes  on  suddenly  and  subsides  gradually,  but  recurs 
in  a  series  of  attacks.     The  spasm  is  attended  by  pain  in  the  muscles. 

In  a  patient  suffering  from  the  disease,  pressure  on  any  nerve  or 
plexus  will  produce  at  once  a  spasm  (Trousseau's  phenomenon).  A 
galvanic  current  passed  through  the  nerve  will  cause  tetanus  of  the 
muscles  instead  of  a  single  contraction,  and  the  electric  reaction  is 
altered,  anodal  opening  contraction  being  greater  than  cathodal  closure 
contraction  (Erb's  phenomenon),  percussion  of  the  motor  nerves  will 
start  a  spasm  (Chvosteck's  symptom),  and  either  percussion  or  elec- 
trical stimulation  of  the  sensory  nerves  may  start  a  spasm  (Hoffman's 
symptom).  There  is  no  loss  of  sensation.  In  some  cases  redness  and 
cedema  of  the  hands  and  feet,  an  excessive  sweating,  or  a  loss  of  the 
nails  has  been  observed.  The  attack  may  be  attended  by  a  rise  of 
temperature  and  a  rapid  pulse. 

The  duration  of  an  attack  varies  from  one  day  to  several  months ; 
the  spasms  may  occur  only  at  long  intervals,  but  the  peculiar  sensitive- 
ness of  the  nerves  may  last  for  a  long  time. 

Prognosis.  —  The  prognosis  is  good  in  the  majority  of  cases.  When 
it  occurs  after  thyroidectomy,  or  when  the  grastro-enteritis  is  compli- 
cated with  dilatation  of  the  stomach,  the  case  may  be  prolonged  and 
may  be  fatal.  It  is  occasionally  fatal  in  children,  but  usually  because 
of  the  condition  of  the  digestive  tract. 

Treatment.  —  As  the  disease  is  due  to  a  poison,  it  is  wise  to  begin 
treatment  by  free  purgation  with  castor  oil.  This  is  to  be  followed 
by  the  use  of  intestinal  antiseptics  of  which  salol  and  salicin  are  the 
best.  The  nutrition  is  to  be  kept  up  by  careful  feeding,  and  large 
amounts  of  water  are  to  be  given.  Lavage  of  the  stomach  and  high 
enemata  are  of  much  service. 

For  the  spasms,  large  doses  of  bromide  with  small  doses  of  chloral 
are  the  best  remedies.  Opium  may  also  be  given.  All  effort  should 
be  avoided.  Friction  of  the  limbs  with  oil  or  liniments  may  relieve 
the  spasm ;  or  the  limbs  may  be  wrapped  in  hot  cloths.  When  the 
disease  is  very  severe  during  pregnancy,  it  may  be  necessary  to  induce 
premature  labor.  When  it  occurs  during  lactation,  the  child  should 
be  weaned.  When  it  occurs  after  thyroidectomy,  thyroid  extract 
should  be  given  at  once,  and  continued  for  months. 

Success  has  lately  been  attained  by  the  use  of  calcium  lactate  in 
15  grain  dose  three  times  daily. ^ 

'Kinnicutt,  Amer.  Jour.  Med.  Sci.,  July,  1909. 


CHAPTER  XLVIII. 

PARALYSIS  AGITANS  AND   TREMOR. 

Paralysis  agitans,  shaking  palsy,  or  Parkinson's  disease^  is  a 
functional  disease  of  the  nervous  and  muscular  systems,  characterized 
by  tremor  of  the  extremities,  by  rigidity  of  the  muscles  which  pro- 
duces slow  movements,  abnormal  postures  and  an  unsteady  gait  with 
tendency  to  fall,  and  by  abnormal  sensations  of  varying  intensity. 

Etiology. — The  disease  is  more  common  among  men  than  women.^ 
While  occasionally  appearing  in  youth  it  is  rarely  seen  before  the 
thirtieth  year,  and  is  far  more  common  in  the  presenile  period,  as 
shown  in  this  table. 

Table  XIX.  —  Age  of  Onset. 

20-30.  30-40.  40-50.  50-60.  60-70.  70-80.  Total. 

Male 2            10  38  50  32            5  139 

Female 0  _9  i^  ^2  i^  -i  _§? 

2           19  54  90  45           9  219 

Occupation  appears  to  have  no  influence  on  the  development  of  the 
disease.  Direct  inheritance  can  rarely  be  traced,  though  in  the  few 
cases  occurring  in  youth,  collected  by  Willige,^  a  distinct  inheritance 
could  be  traced  in  the  majority.  In  16  only  of  our  cases,  some 
relative  had  suffered  from  the  disease.  Gowers  found  an  heredi- 
tary tendency  in  15  per  cent,  of  his  cases,  and  WoUenberg  con- 
sidered 26  per  cent,  of  his  cases  due  to  the  inheritance  of  a  defective 
nervous  system.  A  number  of  cases  in  one  family  have  been  observed 
by  Borgherini  and  by  others.  But  in  view  of  the  other  etiological 
factors  these  conclusions  seem  to  me  questionable. 

The  disease  is  usually  attended  by  so  many  other  evidences  of  old 
age  that  all  authors  regard  it  as  a  condition  consequent  upon  senile 
changes.  A  tendency  to  tremor  exists  in  all  old  people,  even  in  those 
who  do  not  have  the  disease,  and  some  stiffness  and  slowness  of  motion 
often  develops;  hence  it  seems  as  if  the  symptoms  were  largely  an 
exaggeration  of  the  evidences  of  senile  atrophy. 

The  exciting  causes  of  the  disease  appears  to  be  emotional  shocks, 
fright,  worry,  or  anxiety ;  traumatism,  overwork,  exposure  to  cold  and 
hardship.  The  disease  sometimes  follows  acute  diseases,  grippe,  ma- 
laria, typhoid  and  pneumonia.  Any  or  all  of  these  causes  produce  a 
general  weakening  of  the  nervous  system  and  thus  start  or  hasten  any 

'  Parkinson  described  it  first  in  1817. 

2  The  statistics  in  this  article  were  prepared  by  my  assistant,  Dr.  T.  Stuart  Hart, 
from  the  records  of  the  Vanderbilt  Clinic  between  1888  and  1904. 

^H.  WiUige.  Ueberparalysisagitansimjugendlichen  Alter.  Zeitsch.  f.  Neurologie, 
iv.,  520,  1911. 

806 


SYMPTOMS.  607 

process  of  retrograde  evolution.  I  have  seen  many  cases  where  it  has 
followed  soon  upon  a  fright  or  a  period  of  great  anxiety.  I  have  also 
known  it  to  develop  soon  after  a  fall  or  blow.  Thus  a  fall  on  the 
shoulder  or  an  injury  of  one  arm  was  soon  followed  in  31  cases  by 
tremor  in  that  arm.  Sometimes  the  tremor  develops  in  a  part  not  the 
subject  of  direct  injury.  I  have  known  it  to  occur  as  a  sequel  of 
neuritis,  both  in  the  arm  and  in  the  leg. 

Symptoms.  —  Tremor  is  the  symptom  most  constant,  and  usually 
the  one  first  observed.  It  is  a  rather  coarse  tremor,  of  variable  inten- 
sity, occurring  continuously.  It  consists  of  alternate  contractions  of 
flexor  or  extensor  muscles,  so  that  the  joints  are  in  a  constant  state  of 
rhythmical  motion.  It  can  be  arrested  for  a  few  moments  by  an  effort 
of  the  will,  but  is  equally  severe  in  effort  or  in  rest.  Occasionally  it 
ceases  for  a  moment  but  is  quickly  resumed.  The  rate  is  about  five 
or  six  vibrations  to  the  second.  It  ceases  during  sleep.  The  origin 
of  the  tremor  is  an  interference  in  the  constant  tone  of  the  muscles, 
which  is  maintained  by  impulses  going  out  from  the  motor  centres. 
These  impulses  are  so  frequent  as  to  be  practically  continuous  in  their 
effect  on  the  muscles,  but  if  they  are  impeded  in  their  projection  the 
effect  becomes  intermittent,  and  then  in  place  of  a  normal  muscular 
tone  an  intermittent  tremor  develops. 

The  tremor  begins,  as  a  rule,  in  the  hands  or  arms,  one  being  affected 
for  some  months  before  the  other  (157  cases  in  207).  Occasionally  it 
begins-in  the  feet  or  legs  (23  cases  in  207).  Sometimes  both  arm  and 
leg  on  one  side  are  affected  together  (8  cases),  and  if  the  rigidity  comes 
on  rapidly  a  hemiplegia  may  be  suspected.  In  rare  cases  the  tremor 
begins  in  the  head  (3  cases),  or  is  general  from  the  outset  (5  cases). 
When  it  begins  in  the  hands,  the  index  finger  and  thumb  are  usually 
affected  before  the  other  fingers.  The  affection  is  always  noticeable 
in  the  handwriting,  and  this  may  first  call  attention  to  the  disease. 
In  90  cases  the  tremor  began  on  the  right  side  of  the  body ;  in  73 
cases  on  the  left  side.  There  is  always  an  extension  of  the  tremor 
from  the  part  first  affected  to  other  parts,  until  in  the  end  all  extremi- 
ties are  affected.  It  is  usual  for  the  tremor  to  extend  from  an  arm  to 
the  leg  of  the  same  side,  but  sometimes  it  appears  in  the  other  arm  be- 
fore the  leg  is  affected.  If  it  begins  in  the  leg,  it  extends  to  the  arm 
of  the  same  side  sooner  than  to  the  other  leg.  The  rapidity  of  exten- 
sion varies  greatly.  In  the  majority  of  cases  one  or  two  years  elapsed 
before  an  extension  to  a  second  limb  was  noticed,  but  in  a  few  cases 
such  extension  occurred  within  a  few  weeks.  In  one  case  no  extension 
had  taken  place,  although  the  right  hand  had  been  tremulous  for  twelve 
years  and  rigidity  with  lateropulsion  was  present.  In  another  case 
all  the  extremities  were  involved  within  a  year  of  the  onset. 

Tremor  of  the  head  develops  late  in  the  disease  in  many  cases.  This 
is  not  to  be  confounded  with  the  general  oscillation  caused  by  the 
tremor  in  the  arms.  In  not  a  few  a  tremor  of  the  jaw  or  of  the  lips 
and  tongue  was  noticed,  and  in  rare  instances  of  the  eyelids.  The 
eyel)alls  never  oscillate. 

The  patients  often  attempt  to  arrest  the  tremor  by  holding  on  to 


o06  PABALYSIS    AGITANS    AND    TBEMOR. 

objects.  The  habit  of  these  patients,  of  pressing  the  thumb  against 
the  first  finger  or  of  holding  the  fingers  together  to  stop  the  tremor, 
has  made  the  pen  posture  of  the  hand  quite  characteristic  of  the  dis- 
ease. Active  exercise  or  passive  movements  often  mitigate  the  tremor 
for  a  time.  The  vibration  of  a  railway  train  has  the  same  effect. 
Any  emotional  strain,  or  the  fact  of  being  noticed  increases  it. 

Rigidity  of  the  muscles  is  a  symptom  which  follows  the  tremor  after 
some  years.  Occasionally  it  is  the  first  symptom  to  aj^pear  and 
may  exist  for  a  long  time  without  any  tremor.  It  is  felt  in  the 
muscles  of  the  neck  and  back,  at  first,  but  soon  extends  to  the  ex- 
tremities, and  finally  to  every  muscle  of  the  body.  The  stiffness  of 
the  neck  and  back  lead  to  a  peculiar  attitude  characteristic  of  the  dis- 
ease. The  head  is  bent  forward,  the  shoulders  are  rounded,  the  body 
inclines  forward,  and  all  freedom  of  movement  is  lost.  The  arms  are 
usually  held  to  the  sides,  the  elbows  being  flexed,  the  wrists  extended 
and  the  first  phalanges  flexed.  The  other  phalanges  are  extended. 
Abduction  of  the  thighs  is  hampered  and  in  walking  the  knees  are 
approximated  and  the  steps  are  short. 

The  facial  muscles  are  also  stiff,  so  that  there  is  little  play  of  expres- 
sion and  the  face  is  like  a  mask.  In  fact  it  is  often  possible  to  recog- 
nize the  disease  from  the  peculiar  facial  expression,  which  shows  none 
of  the  natural  mobility  in  conversation  and  gives  the  idea  of  a  lack  of 
interest. 

The  vocal  cords  may  be  so  rigid  that  the  voice  is  reduced  to  a 
whisper.  There  were  changes  in  the  voice  noticed  in  120  of  my  cases. 
In  59  the  speech  was  monotonous  ;  in  31  it  was  slow;  in  12  it  was 
piping ;  in  15  it  was  weak  and  feeble ;  in  10  it  was  thick;  in  others  it 
was  jerky  or  tremulous. 

This  rigidity  hampers  all  movements,  but  it  is  not  attended  by 
paralysis  in  the  early  stages. 

If  asked  to  look  around  the  patient  does  not  turn  his  head,  but 
slowly  moves  his  entire  body,  holding  his  neck  rigid. 

In  the  last  stage,  however,  the  rigidity  may  become  so  extreme  that 
the  patients  are  confined  to  a  chair  or  to  the  bed,  and  thus  are  virtually 
incapable  of  voluntary  movement.  Passive  motions  are  then  met  by 
resistance,  which  is  not  true  in  the  early  stage.  The  rigidity  leads  to 
a  slowness  of  action  from  the  first,  and  this  is  noticed  both  in  fine 
movements,  such  as  writing  or  dressing,  or  in  the  larger  ones  of  rising 
from  a  chair  or  walking  or  turning  the  head  or  body  around. 

The  stiffness  of  movement  and  the  peculiar  posture  in  walking  lead 
to  a  characteristic  gait.  This  is  at  first  stiff  and  slow,  as  instinctive 
balancing  movements  are  hampered  and  as  the  body  is  thrown  forward 
the  centre  of  gravity  is  really  in  front.  Hence  the  patient  often  feels 
as  if  about  to  fall  forward,  and  instead  of  standing  erect  hastens  his 
steps  almost  to  a  run  and  finally  stops  himself  by  seizing  some  object 
to  keep  him  from  falling.  This  is  termed  propulsion  or  festination. 
Occasionally  a  tendency  to  step  backward  is  noticed.  Thus  if  a 
patient  attempts  to  reach  up  for  an  object,  throwing  the  body  back- 
ward, he  loses  his  balance,  and  steps  back  several  steps  till  he  runs 


SYMPTOMS. 


809 


against  soirfething.  This  retropulsion  may  be  set  up  by  pushing  the 
patient  when  he  is  standing  still.  Propulsion  was  present  in  77  cases, 
retropulsion  in  19  cases,  and  lateropulsion — a  tendency  to  fall  to  one 
side — in  9  cases  in  my  series.  It  is  not  a  constant  symptom.  It  may 
occur  when  there  is  no  tremor.  It  never  occurs  without  rigidity. 
The  difficulty  in  balancing  sometimes  prevents  patients  from  starting 
to  walk.  X  have  known  several  patients  who,  after  being  assisted  to 
rise  from  a  chair,  had  to  be  swayed  forward  and  backward  and  given 
a  little  shove  in  order  to  enable  them  to  walk.  The  power  to  begin 
stepping  seemed  lacking. 


Fig.  307. 


Fig.  308. 


Position  assumed  by  patient  juiFerlng 
from  paralysis  agitans. 


Rigidity  of  back  in  paralysis 
agitans. 


In  spite  of  the  rigidity,  the  muscles  remain  in  good  condition,  and 
do  not  atrophy  or  show  any  electric  changes.  In  the  last  stage  an 
atrophy  from  disuse  is  quite  common.  In  188  cases  the  deep  reflexes 
were  normal  in  90,  were  exaggerated  in  68,  and  were  diminished  in 
38.  The  knee-jerks  were  often  unequal  on  the  two  sides,  but  this  had 
no  relation  to  the  tremor. 

Contractions  of  the  hands  and  feet  occasionally  develop  in  the  later 
stages  of  the  disease.  The  hands  and  fingers  are  then  firmly  closed 
and  the  feet  in  the  posture  of  talipes  equinus.  The  toes  are  occasionally 
drawn  under  the  foot.  When  the  face  becomes  rigid,  the  saliva  and 
tears  may  not  be  retained. 


810  PARALYSIS   AGlTANS   AND    TEEMOH. 

The  rigidity  of  the  muscles  is  often  accompanied  by  an  aching  feel- 
ing or  a  dull  pain.  There  is  also  a  great  sense  of  restlessness,  and  the 
patients  feel  that  they  may  get  ease  by  a  change  of  posture.  Patients 
who  walk  with  difficulty  have  to  be  helped  out  of  the  chair  and  helped 
about  the  room  every  little  while,  to  obtain  any  relief.  It  is  rare, 
however,  for  pain  to  be  sharp  or  to  keep  a  patient  awake.  Sometimes 
pain  precedes  the  onset  of  tremor. 

Parsesthesise  are  very  commonly  complained  of.  Tickling,  tingling, 
numbness,  flushing,  and  sensations  of  heat  and  of  cold  are  felt,  usually 
in  the  tremulous  limb,  often  all  over  the  body.  The  most  common 
sensation  is  a  burning  feeling  in  the  skin,  and  the  skin  may  feel  hot 
to  the  touch  even  when  there  is  no  internal  rise  of  temperature.  There 
is  never  any  anaesthesia.  Hyperidrosis  was  observed  in  one  quarter 
of  my  cases,  but  the  sweating  was  not  limited  to  the  tremulous  limbs. 

A  rapid  pulse  was  occasionally  noticed. 

Insomnia  is  a  frequent  symptom,  and  often  requires  treatment. 

The  functions  of  the  bladder  and  rectum  are  never  abnormal  unless 
some  complication  (such  as  enlarged  prostate  or  hemorrhoids)  is  present. 

The  mind  is  never  affected,  but  patients  often  become  impatient,  or 
even  morbidly  depressed  at  their  helpless  state,  and  crave  sympathy 
and  encouragement.  A  certain  portion  of  the  cases  develop  hemiplegia, 
but  not  more  than  is  to  be  expected  at  the  age  affected. 

Course.  —  The  disease  is  a  slow  one  in  its  progress.  It  begins  as 
a  rule  gradually  and  the  tremor  may  last  for  many  months  before  it 
becomes  extreme  or  before  rigidity  develops.  The  posture  and  gait 
become  characteristic  only  after  several  years.  In  any  stage  a  remis- 
sion in  symptoms  may  occur,  and  I  have  seen  patients  who  had  suf- 
fered for  years  and  who  were  unable  to  walk  without  help  or  to  talk 
above  a  whisper,  restored  to  comparative  health  for  some  time.  Any 
emotional  strain  may  make  the  symptoms  much  more  intense,  or  increase 
the  rapidity  of  their  development. 

Occasionally  the  disease  begins  suddenly  after  a  fright  or  an  emo- 
tional strain.  A  number  of  cases  of  sudden  onset  were  recorded  in 
France  after  the  sieges  of  Strassbourg  and  Paris,  and  due  to  fright  or 
anxiety.     I  have  known  several  such  cases. 

As  a  rule,  the  disease  gets  gradually  worse,  and  after  several  years 
the  patient  is  finally  obliged  to  remain  in  a  chair,  and  moves  with 
great  effort. 

In  some  cases  the  rigidity  is  the  first  symptom,  and  tremor  does  not  ap- 
pear for  several  years,  after  the  face  and  voice  and  gait  are  characteristic. 

The  duration  is  indefinite,  as  patients  never  die  of  the  disease,  but 
they  never  fully  recover. 

Prognosis.  —  This  is  unfavorable  for  recovery,  but  good  for  life. 

Diagnosis.  —  The  symptoms  are  so  characteristic  that  there  should 
rarely  be  any  difficulty  in  the  diagnosis.  It  can  be  easily  distinguished 
from  multiple  sclerosis  by  the  facts  that  effort  stops  instead  of  increas- 
ing the  tremor ;  that  the  speech,  though  weak,  is  never  scanning ; 
and  by  the  fact  that  nystagmus  does  not  develop.     It  cannot  be  mis- 


TREATMENT.  811 

taken  for  hemiplegia  because  of  its  gradual  onset,  slow  extension  of 
symptoms,  and  the  absence  of  any  true  paralysis. 

The  tremor  of  paresis  is  finer,  is  not  continuous  while  the  hands 
are  at  rest,  and  there  is  no  rigidity  or  characteristic  posture  and  gait 
in  paresis. 

Senile  tremor  usually  begins  in  the  head,  and  while  it  may  invade 
the  hands  does  not  aifect  the  legs  and  is  not  attended  by  rigidity,  or 
other  symptoms  of  the  disease.  Senile  paraplegia  from  arterial  scle- 
rosis and  secondary  sclerosis  in  the  spinal  cord,  is  accompanied  by 
actual  paralysis  and  anaesthesia,  with  disturbance  of  the  bladder  and 
rectum  and  a  spastic  gait  which  is  not  like  that  of  paralysis  agitans. 
The  posture  of  hands  and  of  the  body  in  standing,  the  mask-like  face, 
the  gait,  and  the  tendency  to  festination  are  not  seen  in  any  other  disease. 

Theories  of  the  Disease. — The  lesions  described  by  various  ob- 
servers, such  as  degeneration  of  the  motor  cells  of  the  spinal  cord; 
arterial  sclerosis  in  the  finer  spinal  vessels  and  a  perivascular  sclerosis 
of  the  anterior  horns ;  and  degeneration  of  the  motor  cells  of  the  cor- 
tex of  the  brain,  appear  to  be  lesions  of  old  age  and  not  characteristic 
of  the  disease.  It  is  possible  that  an  affection  of  the  terminal  plates 
of  the  muscular  nerves  may  be  the  true  lesion  of  the  disease,  but  this 
is  as  yet  only  a  theory.  Gauthier  believes  the  disease  to  be  primarily 
muscular  in  origin,  and  there  is  much  to  be  said  in  favor  of  this  hypoth- 
esis Camp,^  in  a  careful  review  of  the  various  theories  and  on  the 
basis  of  fourteen  cases  with  autopsies,  reaches  the  same  conclusion. 
Moebius  believes  it  to  be  due  to  autointoxication  from  some  error  in 
the  metabolism,  due  in  some  cases  to  parathyroid  disease,  and  this 
theory  has  been  urged  by  Lundeburg  ^  and  Berkeley.^ 

Gowers  believes  that  the  disease  is  of  cortical  origin,  basing  his  state- 
ment on  the  fact  that  in  his  observation  the  onset  of  hemiplegia  caused 
a  cessation  of  the  tremor.  But  I  have  seen  two  cases  in  which  the 
onset  of  hemiplegia  has  had  no  effect  on  the  tremor  whatever. 

The  pathology  of  the  affection  must  still  be  considered  as  unknown. 

Treatment.  —  A  life  of  congenial  occupation  without  annoying  care, 
varied  by  travel  and  by  visits  to  health  resorts,  and  under  the  wise 
direction  of  a  good  nurse  who  can  give  massage,  is  the  best  regime  for 
the  patient.  Diet  has  no  influence  on  the  disease.  A  warm  climate  is 
to  be  sought,  as  patients  suffer  more  and  are  more  rigid  in  their  move- 
ments in  cold  weather.  In  a  chronic  affection  it  is  not  wise  to  stop 
the  patient's  work,  as  he  is  then  more  likely  to  worry  over  himself. 

Warm  or  tepid  baths  of  twenty  minutes  duration  either  morning  or 
evening,  not  followed  by  cold  shocks,  afford  relief  to  the  rigidity. 
Massage  is  also  of  great  service  and  may  be  kept  up  indefinitely.  The 
lomi  lomi  method  of  Hawaii  is  of  especial  use.  Passive  motions  often 
give  relief  to  the  feeling  of  fatigue  and  may  lessen  the  tremor  and 
rigidity.     Vibration  is  also  of  benefit,  especially  the  forms  obtainable 

'Jour.  Amer.  Med.  Assoc,  April  13,  1907. 
^Deut.  Zeitschr.  f.  Nervenheilkunde,  1904-5. 
*W.  L.  Berkeley,  Medical  News,  Deceniljer,  1905. 


812  PABALTSIS   AGITANS   AND    TBEMOB. 

in  the  Zander  institutes,  where  the  entire  body  can  be  shaken,  or 
vibratory  pads  can  be  applied  to  the  spine.      Electricity  is  of  no  use. 

Though  the  course  of  the  disease  cannot  be  affected  by  drugs,  some 
of  the  symptoms  can  be  mitigated. 

The  tremor  may  be  diminished,  and  even  arrested  for  a  time  by  the 
use  of  hyoscyamin.  A  tablet  of  -g-i-g-  grain  may  be  given  at  first  every 
6  hours,  then  more  frequently,  the  dose  being  increased  till  the  tremor 
is  diminished,  and  then  kept  at  that  point.  Thus  in  some  patients  3 
such  doses  daily  are  sufficient.  In  others  twice  this  amount  may  be 
required.  The  dryness  of  the  mouth  and  the  dilatation  of  the  pupil 
are  evidence  that  enough  is  being  taken,  and  the  dose  should  not  be 
pushed  as  it  is  a  poison.  The  use  of  the  drug  may  be  kept  up  for 
years  with  benefit.  It  is  aided  by  the  simultaneous  use  of  codeine  in 
^  grain  dose.  This  is  not  to  be  increased  beyond  three  doses  daily. 
Duboisin  sulphate  in  -^-^  grain  dose  may  be  used  if  hyoscyamin  fails. 
I  have  not  found  it  as  efficacious.  Cannabis  indica  in  pills  of  ^  grain 
each,  three  or  four  times  a  day,  may  be  of  service.  Small  doses  of 
tincture  of  veratrum  viride  (2-3  drops  in  water  3  times  a  day)  some- 
times diminish  the  tremor.  Tincture  of  gelsemium  is  often  of  service. 
Arsenic  is  a  remedy  which  has  some  reputation,  and  though  it  does 
not  affect  the  tremor  it  may  lessen  the  rigidity.  It  is  to  be  used  with 
caution,  never  continuously.  In  the  very  last  stage  of  the  disease 
when  the  rigidity  is  painful  and  the  patient  helpless,  the  codeine  may 
be  increased  in  amount  or  morphine  may  be  used  to  give  relief. 

The  use  of  parathyroid  yL  grain  in  tablet  3  or  4  times  daily  is  urged 
by  Berkeley,  but  is  not  successful  in  many  cases. 

The  rigidity  is  decidedly  benefited  by  warm  baths  and  by  massage. 

The  insomnia  may  require  treatment  by  trional,  veronal,  or  the 
bromides. 

Tremor.  —  Tremor  is  a  symptom  which  may  be  present  under 
various  conditions.  There  are  toxic  tremors  from  alcoholism  and 
lead  and  mercury  poisoning.  There  is  a  tobacco  tremor  often  asso- 
ciated with  tobacco  heart.  Neurasthenia  or  any  exhausting  disease 
may  lead  to  a  fine  tremor.  These  tremors  can  be  cured  by  removing 
the  cause. 

Hereditary  tremor  develops  in  some  families  in  middle  life  without 
known  cause.  It  affects  the  hands  chiefly  though  after  a  time  the 
legs,  the  head  and  the  chin  may  be  involved.  It  is  never  very  in- 
tense but  persists  through  life  —  the  chief  inconvenience  being  felt  in 
the  act  of  writing.  It  is  increased  by  mental  or  physical  effort,  also 
by  coffee  and  tobacco. 

Senile  tremor  appears  in  some  persons  after  the  age  of  fifty.  It 
usually  begins  with  a  slight  lateral  oscillation  of  the  head.  Later  the 
hands  may  be  affected.     It  also  persists  to  the  end  of  life. 


CHAPTER  XLIX. 

EPILEPSY. 

Epilepsy  is  a  disease  of  the  brain,  characterized  by  the  occurrence 
of  attacks  at  intervals,  there  being  no  symptoms  between  the  attacks. 
The  attacks  which  occur  may  be  of  three  kinds  —  slight  attacks,  or 
petit  mal;  severe  attacks  of  convulsive  nature,  grand  mal;  or  psy- 
chical attacks. 

The  jpetit  mal  attack  consists  of  a  sudden  loss  of  consciousness,  last- 
ing a  few  seconds  only,  and  often  not  appreciated  by  the  patient.  He 
may  turn  pale,  his  pupils  may  dilate,  he  may  gave  a  slight  start  or 
twitch  or  sigh,  or  he  may  turn  his  eyes  or  head,  or  close  his  eyelids. 
He  may  stop,  if  walking,  and  sway  as  if  about  to  fall.  He  may  make 
some  sound,  smacking  the  lips,  or  attempting  to  speak,  or  groaning  in 
a  low  tone,  or  merely  catching  his  breath.  If  talking  he  may  hesitate, 
stare  in  an  abstracted  absent-minded  manner  for  a  second,  and  then 
resume  the  conversation,  wholly  unaware  that  he  has  acted  in  a  pecu- 
liar way.  Some  patients  will  suddenly  run  forward,  or  in  a  circle,  for 
a  few  seconds,  rapidly,  without  any  apparent  object  and  be  unable  to 
explain  the  act.  This  has  been  termed  procursive  epilepsy.  It  occurs 
in  children  and  is  often  the  first  sign  of  the  disease.  It  continues  and 
is  usually  succeeded  by  attacks  of  grand  mal,  in  which  the  running 
may  constitute  the  aura.  I  regard  this  as  evidence  of  cerebellar  irrita- 
tion. Sometimes  in  infants  a  series  of  movements  of  the  head  and 
body,  a  swaying  forward  and  backward,  has  been  observed  attended 
by  a  vacant  stare.  This  has  been  called  a  tic  de  salaam.  It  is  a  form 
of  petit  mal  and  often  goes  on  to  grand  mal. 

In  some  cases  the  patient  becomes  suddenly  conscious  of  a  sensation 
which  is  forced  upon  his  attention  and  so  occupies  his  mind  that  he 
disregards  everything  else.  The  sensation  may  be  one  of  vertigo,  or 
a  sinking  feeling,  or  a  sense  of  distress  in  the  stomach,  or  a  palpitation 
of  the  heart,  or  an  indefinite  abdominal  feeling.  It  may  be  a  light, 
or  a  blurring  of  vision ;  it  may  be  a  sound  like  a  ringing  in  the  ears, 
or  a  single  tone,  or  a  crash ;  it  may  be  a  smell  or  a  taste ;  it  may  be 
a  tingling  feeling  in  the  skin  of  any  part  of  the  body,  usually  in  one 
limb.  It  may  be  the  sensation  of  a  twitching  of  some  muscles,  and 
the  twitching  may  actually  occur  for  a  few  seconds.  Consciousness  is 
lost  for  a  few  seconds  after  this  abnormal  sensation,  and  when  regained 
the  patient  has  a  knowledge  of  the  sensation  only,  which  has  then 
passed  away.  Some  patients  are  slightly  dazed  after  such  an  attack 
of  petit  mal ;  l^ome  may  even  go  to  sleep ;  but  the  majority  pay  no  at- 
tention U)  the  attack  and  resume  their  ordinary  condition  after  it  is 

813 


814        '  EPILEPSY. 

over.  Many  deny  the  occurrence  of  any  attack,  being  entirely  uncon- 
scious of  it,  and  hence  having  no  memory  of  it. 

Attacks  of  petit  mal  may  be  the  only  manifestation  of  epilepsy. 
As  a  rule  they  are  not. 

The  grand  mal,  or  severe  attack  of  epilepsy,  consists  of  a  sudden 
loss  of  consciousness,  followed  by  a  general  convulsion,  and  by  a 
period  of  stupor,  from  which  the  patient  recovers  with  no  memory 
of  what  has  occurred. 

Epileptic  convulsions  are  preceded  in  about  one  half  of  the  cases, 
by  a  conscious  sensation,  which  is  recognized  after  a  time  as  an  inevit- 
able precursor  and  warning  of  an  attack.  It  is  called  an  aura.  An 
aura  was  present  in  750  cases  out  of  2,000.^  Its  character  was  as  fol- 
lows, in  order  of  frequency  : 

Table  XX.     Kind  of  Aura. 

Epigastric  sensation 198 

Vertigo 158 

Numbness  in  one  or  both  arms 76 

Visual  hallucinations  or  blindness 68 

Mental  states  with  fear  or  depression 51 

Cardiac  sensations 34 

Auditory  hallucinations 30 

Abdominal  sensations 25 

Numbness  in  one  or  both  legs 27 

Numbness  in  the  face  22 

Respiratory  sensations  16 

Hallucinations  of  smell  8 

Feeling  of  cold  or  heat 8 

Nausea 6 

Hallucination  of  taste 5 

Numbness  in  the  trunk 4 

Sensation  of  thirst 4 

Eush  of  blood  to  the  head 4 

Sleepiness 4 

Hallucination  of  taste  and  smell  

Sensation  of  hunger 

Flow  of  saliva 

Diarrhoea 

Tremor 

A  variety  of  sensations  not  included  in  this  list  may  occur  as  aurse. 
Thus  tremor  of  one  extremity  or  an  indefinite  sense  of  tremor  through 
the  body,  a  twitching  of  some  muscles,  grinding  of  the  teeth,  a  sucking 
motion  or  a  clicking  made  by  the  tongue,  a  harsh  cough  or  a  sudden 
yawning  may  occur  at  the  beginning  of  the  attack  before  consciousness 
is  lost.  Some  epileptics  stop  suddenly  if  walking,  make  balancing 
movements  or  turn  about ;  others  start  up  and  walk  or  run  a  few 
steps ;  others  run  about  in  a  circle  and  then  fall  in  a  fit.  There  is  a 
dim  consciousness  of  such  acts  and  they  are  remembered  afterward  as 
the  invariable  precursor  of  an  attack.  Among  the  visual  aura  the 
seeing  of  colors,  especially  red,  or  of  figures  is  not  uncommon.     One 

1  The  statistics  in  this  chapter  are  based  upon  an  analysis  of  1,750  cases  from  my 
clinic,  prepared  for  me  by  Dr.  L.  Pierce  Clark,  and  on  250  consecutive  cases  in  my 
private  practice, 


EPILEPSY.  815 

patient  always  sees  a  figure  resembling  little  red  riding  hood,  another 
sees  a  drageto  head,  anotlier  the  form  of  a  new  moon.  To  others  the 
objects  about  them  seem  to  grow  smaller  or  larger  as  they  lose  con- 
sciousness. Hughlings  Jackson  has  described  an  intellectual  aura 
which  he  called  a  dreamy  state.  In  this  some  recent  events  of  the 
patient's  life  seem  to  pass  through  his  mind  rapidly,  a  sort  of  forcible 
reminiscence  of  experience  which  holds  his  attention.  In  a  patient  of 
F4r§  the  attack  began  with  a  doubt  of  the  existence  of  the  Deity.  It 
is  therefore  evident  that  in  every  form  of  mental  activity  of  which  we 
have  recollection  a  sudden  forcible  presentation  of  the  contents  of  the 
mind  without  volition  may  occur  as  an  aura  of  an  epileptic  attack. 

The  aura  is  uniform  in  each  patient,  and  does  not  vary  its  character 
in  different  attacks.  It  is.  more  likely  to  occur  in  light  cases.  It  is 
evident  that  the  aura  constitutes  the  attack  of  petit  mal  in  many  cases; 
and  patients  who  have  grand  mal  attacks  with  an  aura  often  perceive 
the  aura  without  having  a  convulsion.  The  duration  of  the  aura  is 
not  more  than  a  few  seconds,  but  it  leaves  a  memory,  which  is  usually 
the  only  means  the  patient  has  of  knowing  that  an  attack  an  occurred. 

After  the  aura,  or  without  any  warning,  there  is  a  sudden  loss  of 
consciousness  which  is  more  lasting  than  in  petit  mal.  A  deep  inspira- 
tion or  expiration  occurs,  and  if  the  glottis  is  fixed  at  the  time  this 
causes  a  groan  or  a  cry,  often  low,  sometimes  sharp  and  loud.  The 
head  and  eyes  turn  to  one  side,  or  upward  or  downward,  or  a  fixed 
stare  appears.  The  face  turns  pale.  The  pupils  dilate.  Equilibrium 
is  lost  and  the  patient  falls  heavily,  often  injuring  himself.  Then  all 
the  muscles  become  rigid,  in  a  state  of  tonic  spasm.  The  respiration 
ceases  and  cyanosis  follows.  The  eyeballs  bulge,  conjunctival  hemor- 
rhages occur.  The  jaw  closes  and  the  tongue  is  often  bitten.  The 
hands  are  closed,  the  thumb  being  shut  in  by  the  fingers,  the  limbs  are 
sometimes  flexed,  sometimes  extended.  It  is  more  usual  for  the  arms 
to  be  bent  and  the  legs  straight.  They  are  not  symmetrical  on  the 
two  sides.  The  stomach,  bladder,  seminal  vesicles  and  rectum,  if  full, 
are  sometimes  emptied  by  the  tonic  spasm  of  the  muscles.  This  tonic 
stage  lasts  from  5  to  40  seconds. 

A  general  convulsive  movement  of  the  face  and  limbs  ensues,  con- 
stituting the  clonic  stage  of  the  attack.  The  motions  are  at  first  small 
in  extent,  and  jerky  in  character,  but  soon  become  larger  and  faster 
and  more  violent,  until  the  entire  body  is  writhing  in  a  general  con- 
vulsion. The  head  is  turned  from  side  to  side,  the  jaws  snap,  the  eyes 
roll,  the  face  makes  grimaces,  of  a  horrible  kind,  the  tongue  is  thrust 
out,  saliva  flows,  and  the  arms  and  legs  are  twisted  in  all  possible 
positions  and  contortions.  Injuries  often  occur  in  consequence,  and 
dislocations  of  the  joints,  especially  of  the  shoulder  and  jaw,  may  result. 
If  the  tongue  has  not  been  bitten  in  the  tonic  stage  it  may  be  in  the 
clonic.  Respiratory  movements  relieve  the  cyanosis  but  are  irregular, 
often  deep  and  stertorous,  and  the  saliva  and  blood  flow  from  the  mouth 
and  are  churned  into  a  froth  which  covers  the  lips.  Every  possible 
attitude  may  be  assumed,  and  the  position  of  the  limbs  is  rarely  sym- 


816  EPILEPSY. 

metrical  on  the  two  sides.  After  a  continuance  of  these  convulsive 
movements  for  a  varying  period,  from  one  to  five  minutes,  the  spasms 
gradually  become  less  frequent  and  severe,  and  finally  cease.  Deep 
abdominal  respiratory  movements  continue  for  some  seconds  at  the  end. 
The  patient  does  not,  however,  recover  consciousness  immediately,  but 
gradually  comes  to  himself.  He  is  at  first  dazed,  does  not  know  what 
has  happened  or  where  he  is,  and  requires  some  time  to  return  to  his 
normal  state  of  mind.  His  last  memory  is  of  the  aura  with  which  the 
fit  began,  and  his  first  perception  is  usually  of  fatigue  in  the  muscles, 
or  of  some  injury  received  in  the  fit.  In  a  majority  of  cases  the 
patients  pass  directly  from  the  convulsion  into  a  comatose  state,  and 
cannot  be  aroused.  In  this  the  pupils  are  contracted,  the  body  is  cov- 
ered with  sweat,  and  the  odor  of  the  breath  is  very  oifensive.  Others 
regain  consciousness,  appreciate  that  an  attack  has  occurred,  and  then 
voluntarily  compose  themselves  to  sleep  from  which  they  may  be 
wakened.  Coma  or  sleep  may  last  several  hours.  On  waking,  or  on 
coming  out  of  the  coma,  the  patient  feels  dull,  is  much  fatigued,  has 
headache,  and  sometimes  vomits.  Occasionally  a  true  paralysis  of  the 
exhausted  muscles,  or  a  state  of  aphasia,  or  of  blindness  or  deafness  or 
of  impaired  sensation,  remains  for  a  few  hours.  Babinski  has  observed 
an  exaggeration  of  the  deep  reflexes  with  ankle  clonus  and  extension 
of  the  great  toe  on  scratching  the  foot  (Babinski's  reflex),  also  a  loss  of 
the  anal  reflex  during  and  after  a  grand  mal  attack,  and  these  physical 
signs  he  considers  of  great  importance  as  differentiating  an  epileptic 
from  an  hysterical  convulsion.  A  slight  rise  of  temperature  has  been 
observed  during  an  attack.  The  urine  after  an  attack  is  often  in- 
creased in  amount  and  contains  an  excess  of  phosphates  and  urea,  and 
sometimes  albumen.  Intestinal  disturbances  and  diarrhoea  occasionally 
ensue.  As  a  rule,  the  patient  has  fully  recovered  after  twenty-four 
hours  and  is  then  in  his  usual  state  of  health. 

Every  possible  degree  of  severity  of  attack  has  been  seen  in  epilepsy, 
from  the  slightest  form  of  petit  mal  attack,  up  to  a  severe  type  of 
grand  mal.  In  fact  it  is  often  impossible  to  determine  to  which  class 
the  attack  is  to  be  assigned,  some  authors  describing  as  petit  mal  a 
condition  which  others  consider  grand  mal.  In  many  patients  both 
types  of  attack  occur. 

If  the  attack  occurs  during  sleep  only  the  patient  may  for  years  have 
no  knowledge  of  his  disease  as  he  may  not  be  awakened  by  the  fit  and 
may  pass  from  it  into  a  deeper  sleep.  Unusual  fatigue  or  pain  felt  on 
waking,  at  intervals,  should  always  excite  suspicion  of  the  possibility  of 
nocturnal  epilepsy. 

A  convulsive  movement  of  one-half  of  the  body,  quite  similar  in  its 
characteristics  to  the  general  convulsion  of  epilepsy  but  not  often 
attended  by  a  loss  of  consciousness,  first  described  by  Hughlings  Jack- 
son and  hence  called  Jacksonian  epilepsy,  may  occur.  This  is  now 
regarded  as  a  positive  symptom  of  organic  focal  disease  of  the  brain,  and 
has  been  fully  described  on  page  91.  Jacksonian  attacks  sometimes 
occur  in  the  course  of  epilepsy,  but  should  not  be  classed  with  this  disease. 


EPILEPSY.  817 

Sometimes  one  graud  mal  attack  follows  another  in  rapid  succession, 
so  that  the  patient  passes  from  the  first  coma  into  another  convulsion 
and  these  alternate.  The  condition  is  then  known  as  status  epilepticus. 
As  many  as  a  hundred  convulsions  may  occur  in  a  day,  and  if  they 
continue  two  or  three  days  the  result  may  be  fatal.  In  this  condition 
the  temperature  gradually  rises  to  103°  or  105°,  the  pulse  becomes 
rapid  and  feeble,  the  respiration  rapid  and  irregular,  and  death  occurs 
from  exhaustion  or  heart  failure.  One  quarter  of  epileptics  die  in 
status  epilepticus. 

The  thh-d  type  of  epileptic  attack  has  been  termed  a  psychic  attack 
or  mental  epileptic  equivalent.  These  attacks  differ  from  major  and 
minor  attacks  in  the  fact  that  there  is  no  aura  and  no  apparent  loss  of 
consciousness.  While  the  patients  may  seem  dazed  and  peculiar,  they 
appreciate  their  surroundings,  and  answer  questions  properly.  On 
the  other  hand,  the  state  of  consciousness  is  not  normal,  for  after  the 
attack  is  over  there  is  no  memory  whatever  of  anything  done  or  said 
while  the  attack  is  in  progress.  This  form  of  attack  is  rare.  It  dif- 
fers in  each  patient,  and  is  not  uniform  in  the  same  patient.  Thus  one 
of  my  patients  who  is  fond  of  music  will  suddenly  abandon  anything 
he  is  doing,  go  in  search  of  his  violin,  play  it  for  a  few  minutes,  and 
then  carefully  hide  it  away  in  some  unusual  place.  He  then  lies  down 
on  any  convenient  bed  or  sofa,  or  on  the  floor,  and  appears  to  sleep  for 
a  few  seconds  or  a  minute,  and  on  waking  is  unaware  of  having  done 
anything  unusual.  If  asked  about  his  violin,  he  does  not  know  that 
he  has  touched  it,  and  is  unable  to  find  it.  In  two  attacks  it  has  been 
so  well  hidden  that  it  has  not  been  found  until  in  the  next  attack  he 
has  gone  to  its  hiding  place  to  get  it  in  order  to  play  it  again.  This 
young  man  has  never  had  a  major  or  a  minor  attack. 

Another  patient  becomes  suddenly  overcome  by  a  feeling  of  intense 
depression,  a  deep  melancholia ;  he  feels  that  he  has  sinned  ;  he  is 
inclined  to  commit  suicide,  but  is  oppressed  by  a  sense  of  physical 
weakness  and  soon  falls  asleep,  waking  in  a  few  minutes  perfectly  well 
and  happy. 

Many  patients  who  are  said  to  present  states  of  double  conscious- 
ness are  probably  suffering  from  psychic  epilepsy.  A  person  may  stop 
his  work,  or  leave  his  home  and  wander  away,  start  on  a  journey,  go 
to  some  other  place  and  be  gone  several  days,  finally  coming  to  himself 
in  a  strange  locality,  and  not  know  how  he  got  there  and  be  unable  on 
his  return  to  give  any  account  of  his  actions,  as  he  has  no  memory  of 
what  he  has  done  during  the  period  of  ambulatory  automatism,  as  it 
was  termed  by  Charcot.  Another  form  of  psychic  attack  is  less  harm- 
less. A  patient  will  suddenly  get  into  a  terrible  rage  without  cause, 
will  abuse  relatives  or  friends,  or  even  address  perfect  strangers 
with  insults,  will  attack  those  who  attempt  to  restrain  him  and  may 
commit  homicide  in  his  rage.  Others  attempt  to  set  fire  to  things 
about  them  or  commit  sexual  assaults.  The  attack  will  subside  as 
suddenly  as  it  begun,  and  the  patient  has  no  memory  of  it.  Thus 
one  of  my  patients^  a  devoted  mother,  has  sudden  attacks  of  this 
62 


818  EPILEPSY. 

kind  iu  which  she  has  attempted  to  kill  her  children.  Another,  a 
young  man  developed  such  attacks  after  a  fracture  of  the  skull,  and 
was  cured  by  trephining.  These  attacks  are  called  epileptic  mania. 
Some  psychic  attacks  are  less  severe,  a  sudden  intense  fear  and  dread 
of  impending  evil  or  danger  takes  possession  of  the  mind,  and  the 
patient  seems  to  suffer  intensely.  Such  a  patient  may  suddenly  start 
and  run  for  some  distance,  and  on  coming  back  to  normal  conscious- 
ness be  unable  to  account  for  his  conduct  and  have  no  memory  of  his 
mental  distress.  Some  patients  have  a  sudden  arrest  of  thought  and 
pass  at  once  into  a  dreamy  state  from  which  they  cannot  for  a  time  be 
aroused  to  full  consciousness.  When  they  awake  they  have  the  indis- 
tinct recollection  of  a  dream  but  no  acute  consciousness  of  anything 
said  or  done  in  their  presence  while  in  the  dreamy  state.  Some  patients 
experience  an  overwhelming  desire  to  sleep,  fall  asleep,  nod  or  snore 
once,  and  then  awake,  being  dazed  for  a  moment  afterward. 

Inasmuch  as  peculiar  mental  states  often  follow  attacks  of  both 
petit  mal  and  grand  mal,  it  has  been  held  by  some  authors  that  the 
psychic  attack  is  always  the  sequel  of  a  major  or  minor  attack  and 
should  not  be  considered  as  an  equivalent  of  an  attack,  but  as  a  post- 
epileptic phenomenon.  To  this  I  do  not  wholly  agree,  as  evidence  is 
wanting  of  any  form  of  minor  or  major  attack  preceding  the  mental 
state  in  many  cases. 

The  relative  frequency  of  the  different  types  of  epileptic  attack  is 
shown  in  the  subjoined  table. 

Table  XXI. — Character  of  Attacks  in  2,000  Cases. 

Cases.  Cases. 

a  Grand  mal  and  psychic 3 

Grand  mal   1,150  Grand  mal,  petit  mal  and  psychic...  6 

Grand  and  petit  mal  589  Petit  mal  and  psychic 2 

Petit  mal  179 

Jacksonian  37                                            b 

Psychic 16  Diurnal  and  nocturnal  882 

Grand  mal  and  Jacksonian  10  Diurnal  only  658 

Grand   and  petit  mal  and  Jack-  Nocturnal  only 380 


soman 


It  will  be  seen  that  every  combination  is  possible,  but  that  the 
psychic  type  is  quite  rare. 

The  second  part  of  this  table  shows  the  tendency  to  a  special  limi- 
tation of  the  attacks  in  some  cases  to  certain  times  of  the  day.  It  has 
long  been  noticed  that  some  epileptics  have  attacks  at  night  only  — 
others  at  certain  hours  of  the  day  only.  But  the  majority  of  cases 
have  attacks  both  by  day  and  by  night.  Dr.  L.  Pierce  Clark,  who 
has  made  a  special  study  of  the  Daily  Rhythm  of  Epilepsy,^  has  pre- 
pare the  annexed  chart  which  is  of  much  interest. 

The  condition  of  the  patient  between  the  attacks  may  be  that  of 
perfect  health  and  of  normal  mind.  This  is  the  rule  in  the  beginning 
of  the  disease,  or  when  the  intervals  between  the  attacks  are  long. 
1  Medical  News,  July  18,  1903, 


EPILEPSY. 


819 


But  the  tendency  is  for  these  intervals  to  become  shorter,  and  when 
attacks  are  occurring  with  considerable  frequency  the  majority  of 
patients  cannot  be  called  well  or  normal  between  the  attacks.  There 
is  a  gradual  development  of  dementia  in  the  chronic  severe  cases. 

The  usual  course  of  a  case  of  epilepsy  is  as  follows  :  The  disease 
begins  in  early  life.  One  convulsion  is  followed  after  a  year  or  more 
by  a  second,  and  this,  in  turn,  after  a  few  months  by  a  third.  Then 
the  attacks  become  more  frequent,  —  two  months  or  six  weeks  may  be 
the  usual  interval  in  some  cases ;  in  others,  attacks  occur  every  week ; 
in  very  severe  cases  two  or  three  times  a  week.  Not  uncommonly 
several  attacks  occur  within  a  few  days,  and  then  after  an  interval  of 
two  or  three  weeks  another  series  of  attacks  occur.  The  tendency  is 
for  the  attacks  to  increase  in  frequency,  until  a  regular  interval  is  es- 
tablished after  five  years,  and  at  this  interval  the  patient  continues  to 
have  attacks  all  his  life.  As  a  rule,  the  course  of  the  disease  is  modi- 
fied by  medicines.  These  reduce  the  frequency  of  the  attacks  just  in 
proportion  to  their  previous  frequency.  Thus  when  a  patient  has  had 
attacks  once  a  week,  treatment  may  prolong  the  interval  to  once  in  two 
months  ;  if  he  has  had  only  two  attacks  a  year,  he  may  go  three  or 
four  years  without  an  attack  if  his  treatment  is  kept  up.  When 
treatment  is  stopped  the  attacks  recur.  The  general  effect  of  the 
attacks  upon  the  physical  condition  is  not  serious.  Patients  often 
suffer  from  injuries  during  an  attack,  and  most  epileptics  can  show 
scars,  but  the  health  may  be  good.  The  more  serious  effect  of  the 
disease  is  upon  the  mind.     Children  who  suffer  from  epilepsy  cannot 


/J  ?n 

Table  XXII. 

(?Jn 

1 

, 

^ 

s 

V 

■i 

(, 

r 

f 

■?■ 

/,> 

„ 

/5 

/ 

3 

.5 

<, 

'<" 

c. 

7 

r    ,c 

„ 

/?. 

77>1 

W».' 

pS/) 

Kn 

Ult 

i-ttf 

^ao 

.'TCJIt 

i-3M 

.-rnn 

1 

- 

\ 

- 

Vttc 

1 

I 

1 

Wse 

1 

1 

1 

I 

1 

1 

1 

1 

1 

1 

1 

1 

■ 

■ 

• 

1 

1 

1 

1 

1 

1 

■ 

P 

I 

mm\ 

1        Yj'^3^'ftB-\-7^idff'r\filf^^<^'f;<p\?i'^\<l9^nrc\!^Of^f'^eYsyf^ 

Showing  the  hour  of  the  day  at  which  attacks  occurred  in  150,000  epileptic  seizures. 


go  to  school,  and  therefore  grow  up  with  an  imperfect  mental  training  ; 
they  are  difficult  to  control,  being  moody,  fretful,  ill-tempered  and 
undisciplined  ;  and  at  puberty  they  often  become  victims  of  self-abuse 
with  its  attendant  neurasthenic  symptoms.  Then  they  drift  into  a 
state  of  mental  weakness  and  require  the  same  care  which  is  given  to 
imbeciles.  Epileptics  lead  necessarily  a  semi-invalid  life,  cut  off  from 
occupation  and  society.  They  therefore  become  morbid,  hypersensi- 
tive, timid,  untrained  in  their  mode  of  thought  and  action,    have  few 


820  EPILEPSY. 

interests  outside  of  themselves,  and  are  difficult  to  manage.  Many 
gradually  develop  mental  peculiarities  ;  become  forgetful,  irritable  in 
temper,  incapable  of  concentration  of  mind  or  effort,  suspicious  of  their 
relatives,  and  show  evidences  of  a  weakened  mind.  A  small  per- 
centage develop  active  mental  symptoms  and  go  on  to  some  form  of 
insanity ;  either  temporary  attacks  of  mania  with  sudden  onset,  which 
necessitates  constant  restraint ;  or  epileptic  dementia,  in  which  a  pro- 
gressive loss  of  mind  is  observed.  The  same  mental  deterioration  is 
observed  in  the  cases  of  petit  mal  and  of  psychic  epilepsy  as  in  the 
cases  of  grand  mal.  A  few  patients  die  in  a  fit  from  suffocation  or 
from  cardiac  failure.  Many  go  into  the  status  epilepticus  and  die. 
But  the  large  majority  go  through  life  the  victims  of  the  affection,  and 
die  from  some  other  disease. 

There  are  many  exceptions  to  the  usual  course  described.  Thus 
some  patients  have  attacks  only  every  three  or  four  years,  show  no 
signs  of  mental  deterioration  and  no  ill  effects  of  the  affection.  Csesar 
and  Napolean  were  examples.  Others  have  a  series  of  attacks  in  youth, 
and  then  appear  to  outgrow  the  disease  and  have  no  further  attacks,  or 
only  rare  attacks  at  very  long  intervals.  Some  patients  have  attacks 
only  after  alcoholic  or  sexual  indulgence.  Some  women  have  attacks 
only  at  the  menstrual  period.  This  does  not,  however,  imply  any 
uterine  or  ovarian  disease,  and  attacks  may  not  cease  after  complete 
removal  of  the  organs.  Some  chronic  epileptic  women  are  entirely 
free  from  attacks  during  pregnancy,  but  this  is  not  the  rule.  Many 
epileptics  are  free  from  attacks  during  the  course  of  any  infectious 
disease,  such  as  typhoid  or  malarial  fever,  erysipelas  and  diphtheria, 
scarlet  fever  and  pneumonia,  and  in  some  cases  after  such  diseases  the 
patient  is  better  for  months.  Spratling^  states  that  when  pulmonary 
tuberculosis  develops  in  an  epileptic  and  is  active  from  the  onset,  the 
attacks  of  epilepsy  may  cease  for  some  months  or  even  years.  In  the 
majority  of  patients  physical  exertion,  dancing,  hard  riding,  or  fatigue, 
or  mental  strain,  anxiety  or  fear  or  fright,  will  induce  an  attack. 

While  many  epileptics  present  a  normal  appearance,  there  are  quite 
a  proportion  which  show  some  of  the  marks  of  degeneration.  Abnor- 
mal contour  of  the  skull,  a  microcephalic  head,  a  projection  of  the 
occipital  bone,  a  very  high  arched  palate,  irregular  teeth  from  narrow 
jaws,  peculiarly  shaped  ears,  and  unusually  large  hands  and  feet  are 
found,  especially  in  the  patients  with  bad  inheritance,  in  whom  the 
disease  has  developed  early.  It  is  in  these  patients  that  mental  pecu- 
liarities are  frequently  observed,  either  dulness,  stupidity,  and  ungov- 
ernable temper,  or  marked  talent  in  one  direction  with  incapacity  in 
others.  In  all  epileptics  the  pupils  are  constantly  dilated,  and  when 
they  contract  to  light  they  soon  dilate  again  and  vary  in  size.  This 
symptom  is  called  hippus.  During  an  attack  the  pupils  are  dilated. 
The  facial  expression  of  an  epileptic  is  usually  one  of  stupidity  or 
viciousness,  or  is  indicative  of  a  lack  of  mentality.  The  actions  may  be 
awkward  and  the  gait  slouching  and  irregular.     Clark  and  Scripture^ 

1  Jour.  Amer.  Med.  Assoc,  Nov.  7,  1908,  p.  1616. 

2  The  Epileptic  Voice  Sign,  N.  Y.  Med.  Record,  Oct,  31,  1908. 


EPILEPSY. 


821 


hold  that  l^e  voice  is  peculiar,  having  a  monotonous  character,  that  is, 
the  successive  sounds  do  not  vary  as  much  from  each  other  as  normal 
sounds  do  and  this  they  prove  by  the  Tambour  method. 

Etiology.  —  1.  Age  and  Sex.  —  The  following  tables  show  that  the 
disease  is  one  of  early  life,  68  per  cent,  of  the  cases  developing  before 
the  age  of  twenty-one,  and  comparatively  few  appearing  after  the  age 
of  twenty-five. 


Table  XXIII. 

Age. 

Cases. 

Age. 

Cases 

Age 

Cases. 

1 

yr. 

67 

11 

yrs. 

74 

21  yrs. 

66 

2 

vrs. 

93 

12 

62 

22 

61 

3 

80 

13 

77 

23 

53 

4 

55 

14 

74 

24 

37 

5 

58 

15 

114 

25 

28 

6 

49 

16 

85 

26 

26 

7 

53 

17 

92 

27 

28 

8 

60 

18 

71 

28 

29 

9 

47 

19 

64 

29 

17 

10 

56 
618 

20 

42 
755 

30 

23 
368 

Age. 

Cases. 

Age. 

Cases. 

■Ag 

Cases. 

Age. 

Cases 

31  yrs. 

42 

41 

yrs. 

12 

51 

yrs. 

3 

63  yrs. 

2 

32     " 

14 

42 

6 

52 

3 

66    " 

1 

33     " 

18 

43 

8 

53 

1 

34     " 

18 

44 

4 

54 

2 

35     " 

13 

4) 

7 

55 

1 

36     " 

14 

46 

6 

56 

0 

37     " 

11 

47 

9 

57 

1 

70     " 

1 

38     " 

11 

48 

4 

58 

2 

74     " 

1 

39     " 

13 

49 

5 

59 

2 

76     " 

1 

40     " 

6 
i60 

50 

2 
63 

60 

2 
18 

6 

Sex. 

Males  1163. 

Females  837. 

Among  my  own  cases  there  were  more  males  than  females,  but  other 
authors  have  found  little  diiference  between  the  sexes. 


Table  XXIV.  —  Hereditary  Factors  Noted  in  700  Cases. 

Cases. 

Epilepsy  in  a  near  relative 136 

Alcoholism  in  a  parent 120 

Nervous  diseases  118 

Rheumatism 108 

Tuberculosis. 76 

Alcoholism  and  rheumatism  38 

Rheumatism  and  nervous  diseases..  18 

Tuberculosis  and  nervous  diseases 19 

Tuberculosis  and  rheumatism 16 

Alcoholism  and  tuberculosis  16 

Alcoholism  and  epilepsy 7 

Alcoholism,  nervous  disease  and  tuberculosis 5 

Rheumatism  and  epilepsy  3 

Alcoholism,  rheumatism  and  tuberculosis 2 

2.  Heredity.  —  Wliile  a  direct  inheritance  of  epileptic  attacks  in  the 
children  of  epileptic  parents  is  quite  rare  in  my  experience  (17  cases  in 


822  ETIOLOGY. 

2,000  only)  it  is  admitted  by  all  writers  of  experience  that  the  most 
potent  predisposing  cause  of  epilepsy  is  a  neuropathic  inheritance.  A 
weak  nervous  system  is  a  sign  of  defective  development  of  the  most 
highly  specialized  organ  of  the  body.  And  any  factor  which  impairs 
the  health  of  a  parent  may  easily  aifect  the  development  of  a  child. 
This  may  be  a  constant  factor  like  a  bad  constitution  or  a  temporary 
factor  like  intoxication.  In  700  of  my  cases  some  such  factor  was 
noted  as  shown  in  table  XXIV. 

These  require  some  comment.  Epilepsy  is  often  present  in  a  number 
of  members  of  a  family.  Thus  it  is  not  at  all  uncommon  to  get  a  his- 
tory of  epilepsy  in  an  aunt,  uncle  or  cousin  of  a  patient,  even  if  the 
father  and  mother  are  free  from  the  disease.  And  if  inquiry  is  made 
in  regard  to  the  third  generation,  a  common  ancestor  is  often  found  to 
whom  all  the  cases  in  the  several  branches  can  be  traced.  In  my  list 
there  were  17  epileptics  whose  parents  had  epilepsy,  there  were  119 
who  had  aunts  or  uncles  suffering  from  the  disease. 

Alcoholism  in  a  parent  is  the  next  factor  which  causes  the  develop- 
ment of  a  weak  nervous  system.  While  the  statistics  show  only  120 
such  cases  of  epilepsy,  there  is  every  reason  to  believe  that  they  are 
more  common  than  is  stated.  In  my  private  practice  I  find  this  a 
most  common  predisposing  cause  of  nervous  disease  in  the  offspring. 
The  constant  use  of  alcohol  is  just  as  detrimental  to  the  nervous  system 
as  its  occasional  abuse,  and  just  as  likely  to  show  effects  in  the  next 
generation.  A  fact  which  appears  to  confirm  this  theory  is  the  rarity 
of  epilepsy  in  Mohammedan  countries  were  alcohol  is  forbidden. 

Actual  organic  nervous  diseases  in  a  parent,  such  as  locomotor  ataxia, 
multiple  sclerosis,  chronic  affections  of  the  brain  or  spinal  cord  were 
ascertained  to  exist  in  118  cases.  Here  again  the  defective  nervous 
system  of  the  child  could  be  ascribed  to  inheritance. 

It  seems  to  me  uncertain  that  rheumatism  and  tuberculosis  in  a 
parent  can  have  the  direct  effect  of  producing  epilepsy  in  the  child. 
But  in  the  statistical  investigation,  these  factors  were  inquired  into, 
and  a  history  of  such  a  condition  was  obtained  in  108  and  76  cases 
respectively.  These  are  conditions  which  impair  general  vitality,  and 
a  person  suffering  from  them  is  certainly  more  unlikely  to  produce 
healthy  offspring  than  one  who  is  in  a  state  of  health.  The  weakness 
in  the  child  may  show  itself  in  a  predisposition  to  rheumatism  or  to 
tuberculosis.  It  may  also  show  itself  in  an  imperfect  development  of 
the  nervous  system  and  this  may  lead  under  proper  exciting  causes  to 
epilepsy. 

It  is  natural  to  suppose  that  any  combination  of  these  factors  will 
exert  a  greater  influence  than  any  one  of  them  alone.  But  these  factors 
do  not  exhaust  the  list.  The  neurasthenia  of  modern  life  due  to  over 
anxiety  and  over  strain  of  a  mental  kind  reflects  itself  on  the  offspring 
and  is  a  cause  of  epilepsy  in  very  many  cases.  Chronic  ill  health  of 
a  kind  not  primarily  nervous,  such  as  the  chronic  toxaemia  of  digestive 
disturbances,  of  the  infectious  diseases,  especially  malaria,  must  be  con- 
sidered as  a  predisposing  cause  of  epilepsy. 


EPILEPSY.  823 

lnherite4'  syphilis  is  an  occasional  cause,  not  always  recognized.  In 
one  of  my  patients  the  appearance  of  Hutchinsonian  teeth  was  the  first 
suggestion  of  this  factor,  and  his  attacks,  which  no  other  form  of  treat- 
ment had  diminished,  have  ceased  entirely  on  mercurial  treatment,  and 
the  patient  has  grown  to  be  a  healthy  man.  He  has  now  been  free 
from  attacks  for  six  years. 

It  has  been  stated  that  the  children  of  workers  in  lead  are  likely  to 
be  epileptic.     Of  this  I  have  no  proof,  in  my  experience. 

3.  Exciting  Causes  of  the  Disease.  —  In  the  majority  of  cases  of  epi- 
lepsy no  cause  can  be  determined.  These  cases  are  termed  idiopathic 
for  want  of  an  explanation.  In  some  cases  the  disease  develops  in  con- 
nection with  or  after  some  event  suggestive  of  a  cause.  In  the  follow- 
ing table  the  cause  supposed  by  the  patient  or  by  his  family  to  be  re- 
sponsible for  the  disease  is  stated.  In  1,363  cases  out  of  2000  no  such 
cause  could  be  ascertained. 

Table  XXV.  —  Alleged  Cause  of  the  Epilepsy. 

C'^ses.  Cases. 

Trauma 229  Sunstroke 12 

Fright 119  Physical  strain 11 

Alcoholism 61  Measles 12 

Invalidism  49  Indigestion 10 

Mental  strain 36  Arterial  sclerosis 8 

Menstrual  disorder  24  Syphilis  of  the  brain 8 

Dentition  23  Whooping  cough 6 

Child-birth 24  Eickets 5 

Scarlet  fever 20  Excessive  heat 5 

Infantile  palsy _. 19  Trauma  to  mother 4 

Maldevelopment  of  brain 19  Diphtheria 4 

Masturbation 14  Typhoid  fever 4 

Menopause 11 

These  alleged  causes  of  epilepsy  require  some  comment. 

(a)  Injuries  of  the  brain,  causing  hemorrhages  either  gross  or  capil- 
lary, followed  by  the  development  of  cicatricial  tissue,  are  a  very  com- 
mon cause  of  epilepsy.  That  such  injuries  occur  in  the  process  of 
labor  by  undue  compression  of  the  head  or  by  a  delay  in  the  delivery, 
the  formation  of  a  large  caput  and  a  corresponding  congestion  of  the 
brain  is  admitted.  This  is  the  most  common  cause  of  the  cerebral 
atrophies  of  childhood  in  which  condition  epilepsy  develops  in  a  large 
percentage  of  the  cases.  ^  No  surprise  is  expressed  when  epilepsy  ap- 
pears in  these  cases,  where  a  gross  lesion  is  apparent.  But  many  cases 
of  epilepsy  undoubtedly  date  from  birth.  Here  the  hemorrhage  which 
has  occurred  is  so  small  as  to  leave  no  evidence  in  the  form  of  par- 
alysis or  of  sensory  defect  or  of  mental  deficiency,  but  has  been  suffi- 
cient to  form  a  focus  of  irritation  in  the  brain.  In  later  life  any  in- 
jury of  the  brain  may  cause  epilepsy.  A  severe  blow  on  the  head  or 
a  fall  which  causes  a  fracture  of  the  skull  is  often  followed  2  or  3 
years  later  by  the  appearance  of  epileptic  attacks.  In  many  cases 
there  is  no  fracture  but  the  brain  is  equally  injured.  And  since  sur- 
gical attacks  upon  the  brain  for  the  relief  of  abscesses  and  tumors  have 

'See  chapter  XXVIII. 


824  EPILEPSY. 

become  common  and  surgical  scars  have  been  produced  in  the  brain 
tissue,  it  has  been  recognized  that  epilepsy  is  a  not  infrequent  sequel 
of  the  operation.  Sometimes  these  cases  of  epilepsy  are  purely  Jack- 
sonian,  and  the  location  of  the  scar  can  be  ascertained  by  the  symptoms 
in  the  attack.  In  many  cases  the  fits  are  general,  either  of  the  minor 
or  major  variety,  with  or  without  localizing  aurse.  Any  scar  in  the 
brain  tissue  may  after  a  time  (usually  two  or  three  years)  become  a 
focus  of  irritation  sufficient  to  cause  attacks.  And  this  fact  awakens 
some  doubt  as  to  the  efficacy  of  surgical  operations  upon  the  brain  for 
the  relief  of  epilepsy;  for  even  where  a  true  focal  lesion  causing  the 
disease  can  be  found  and  removed  the  necessary  incisions  into  the  brain 
leave  cicatrices,  which  in  turn  may  produce  a  renewal  of  the  disease. 

The  influence  of  trauma  of  the  head  in  producing  epilepsy  is  shown 
by  the  statistics  of  the  Franco-Prussian  war.  Among  8,985  persons 
wounded  on  the  head  46  developed  epilepsy,  while  among  77,461  per- 
sons wounded  elsewhere  only  17  became  epileptic. 

In  my  own  cases  1 1  per  cent,  could  be  traced  to  traumatism.  When 
sunstroke  is  followed  by  epilepsy,  it  is  reasonable  to  suppose  that  either 
a  capillary  hemorrhage  or  a  toxic  action  on  the  cells  of  the  brain  has 
occurred,  which  has  resulted  in  the  formation  of  a  sclerotic  plaque. 

(6)  Fright  is  recognized  as  a  cause  of  epilepsy  by  all  authors.  It 
was  a  supposed  cause  in  119  of  my  cases.  In  one  case  a  young  girl 
was  suddenly  awakened  by  her  brother  who  had  concealed  himself  in 
her  room  at  night  and  covered  himself  with  a  white  sheet.  She  was 
much  alarmed,  had  a  major  attack  an  hour  after,  and  has  been  a  con- 
firmed epileptic  for  ten  years.  I  could  relate  many  such  cases  where 
the  cause  is  equally  clear. 

(c)  The  effect  of  alcoholism  in  the  production  of  epilepsy  is  also 
admitted.  Many  cases  come  on  during  a  spree  and  many  develop  in 
chronic  steady  drinkers.  Experiments  upon  animals  prove  that  alcohol 
in  the  blood  causes  degeneration  of  the  neurons  of  the  cord  and  brain. 
Observations  upon  alcoholic  brains  show  chromatolysis  and  disinte- 
gration of  the  cortical  cells.  It  is  not  unlikely  that  among  the  effects 
produced  by  these  changes  epilepsy  should  be  one,  especially  as  the 
pathological  findings  in  epilepsy  are  similar  in  character  and  in  loca- 
tion to  those  seen  in  alcoholic  brains. 

(d)  Epilepsy  may  develop  as  a  sequel  of  any  of  the  infectious  dis- 
eases either  in  childhood  or  in  adult  life.  In  the  list  of  supposed 
causes  tabulated  are  many  of  the  infectious  diseases — scarlet  fever, 
measles,  whooping-cough,  diphtheria,  typhoid  fever,  and  meningitis. 
Marie  has  shown  that  multiple  sclerosis  of  the  nervous  system  —  a 
disease  in  which  epileptiform  attacks  may  occur  as  a  symptom  —  is 
probably  a  sequel  of  these  diseases  and  due  to  the  infectious  agent, 
microorganism  or  toxin,  which  causes  them.  Is  it  not  possible  that  in 
the  epileptic  cases  developing  after  an  infectious  disease  some  similar 
sclerotic  plaque  may  have  been  produced  in  the  brain,  and  that  in  these 
cases  the  disease  has  an  organic  basis  ?  Among  my  2,000  cases,  50 
developed  as  a  sequel  of  such  diseases.  It  is  rarely  due  directly  to 
syphilis,  unless  the  syphilis  causes  a  gross  lesion  of  the  brain. 


EXCITING    CAUSES    OF    THE    DISEASE.  825 

(e)  Peripheral  irritation  has  been  supposed  to  be  a  possible  cause 
of  epilepsy.  There  are  a  few  cases  on  record  in  which  au  injury  to  a 
nerve  in  a  limb  has  been  followed  by  a  painful  scar  and  then  epilepsy 
has  developed  and  pressure  on  the  scar  has  produced  fits  and  excision 
of  the  scar  has  stopped  the  fits.  I  have  one  such  case  in  my  experi- 
ence. They  are  very  rare.  But  their  existence  has  led  to  the  idea 
that  irritation  arising  from  any  organ  or  any  part  of  the  body  may 
cause  epilepsy.  Thus  eye  strain  from  imperfect  muscular  action  or 
from  imperfect  refraction,  irritation  of  the  nasal  cavity  by  deflected 
bones  or  adenoids  or  polyps  ;  dentition,  chronic  stomach,  intestinal  and 
rectal  diseases,  uterine  and  ovarian  disease,  phimosis,  and  many  other 
affections  which  produce  pain  or  irritation  of  the  nervous  system  have 
been  cited  as  causes  of  epilepsy.  It  may  be  admitted  that  a  person 
who  has  epilepsy  is  more  susceptible  to  these  irritations  than  a  normal 
person.  And  in  an  epileptic  the  removal  of  such  irritation  often 
diminishes  for  a  time  the  number  of  attacks.  But  that  these  influ- 
ences can  cause  the  disease  in  an  otherwise  normal  person  I  do  not  be- 
lieve, and  I  have  never  known  a  cure  of  the  disease  by  their  removal. 
I  saw  a  little  boy  two  years  old  who  was  having  200  minor  attacks 
daily  which  ceased  in  3  days  after  circumcision  and  did  not  recur  for 
some  years.  Eight  years  afterwards  he  was  brought  to  me  suffering 
from  major  attacks.  I  had  published  his  case  as  one  of  reflex  epilepsy 
cured  by  circumcision.  I  now  believe  that  he  had  the  disease  from 
the  beginning  and  that  his  phimosis  was  an  exciting  cause  of  attacks 
but  not  of  the  disease.  I  could  cite  a  very  large  number  of  cases 
equally  conclusive  where  temporary  effects  have  been  produced  by 
removal  of  the  adenoids,  tonsils,  or  ovaries,  by  adjustment  of  glasses  or 
prisms,  but  no  permanent  cure  has  been  obtained.  The  recent  trial 
of  treatment  of  eye  strain  at  the  Craig  Colony  without  any  result 
has  proven  that  this  factor  has  been  overestimated  by  certain  writers. 

The  epilepsies  of  advanced  life,  which  are  happily  few  in  number 
(but  26  cases  in  2,000  developed  after  the  age  of  50)  can  almost  inva- 
riably be  traced  to  endarteritis  and  atheroma.  This  is  proven  by  the 
fact  that,  as  a  rule,  these  patients  have  attacks  of  apoplexy,  indicative 
of  cerebral  hemorrhage  or  thrombosis,  either  just  before  or  soon  after 
their  first  epileptic  fit.  Hence  we  may  believe  that  senile  epilepsy  is 
surely  of  organic  origin. 

(g)  Other  supposed  causes,  such  as  childbirth,  dentition,  masturba- 
tion, menopause,  overwork,  etc.,  seem  to  me  too  vague  and  uncertain 
to  be  accepted  as  true  etiological  factors. 

(/t)  Various  forms  of  toxaemia  must  be  admitted  as  causes  of  the 
individual  attacks,  though  it  is  questionable  if  this  is  a  cause  of  the 
disease.  Nothing  is  more  common  than  a  history  of  gastric  or  intesti- 
nal disturbance,  or  a  state  of  constipation  preceding  an  attack.  It  is 
well  known  that  in  infants  intestinal  diseases  produce  convulsions  which 
rarely  go  on  to  epilepsy.  Ursemic  and  alcoholic  convulsions  are  known 
to  occur.  Hence  it  is  probable  that  in  many  epileptics  the  existence 
of  toxic  states  is  the  exciting  cause  of  the  attack.     And  the  removal 


826  EPILEPSY. 

or  prevention  of  such  states  is  a  necessary  part  of  the  treatment.  But 
when  it  is  remembered  that  similar  toxic  states  occur  in  a  large  pro- 
portion of  mankind  without  causing  convulsions,  it  becomes  evident 
that  toxaemia  alone  is  not  a  cause  of  the  disease. 

Theories  of  the  Disease.  —  In  the  absence  of  a  positive  pathology 
of  epilepsy  it  is  necessary  to  consider  various  theories  of  the  disease, 
based  upon  our  present  knowledge  of  numerous  facts. 

The  Theory  of  Organic  Disease.  —  Every  motor  act  is  the  result  of  a 
discharge  of  energy  from  motor  cells  with  corresponding  using  up  of 
their  substance,  excretion  of  waste  products,  and  exhaustion.  Every 
conscious  sensory  act  is  attended  by  similar  activity  in  the  sensory 
cells.  In  ordinary  conditions  the  conscious  perceptions  originate  from 
impulses  outside  the  body  and  the  motor  acts  from  volitional  impulses 
within.  Certain  poisons  circulating  in  the  blood  can  cause  a  discharge 
of  the  inherent  energy  from  these  cells  producing  movements  or  sen- 
sations, —  e.  g.,  the  convulsions  of  strychnine,  the  hallucinations  of 
alcoholism  or  of  opium  poisoning.  These  being  spontaneous  and 
involuntary  are  not  orderly  and  rhythmical  and  under  control.  We 
have  seen  that  the  aura  in  epilepsy  is  unexpected  and  the  convulsion  is 
a  mass  and  series  of  disorderly  movements. 

In  a  state  of  health  the  tendency  of  nerve  cells  to  discharge  their 
energy  on  a  slight  provocation  is  restrained  by  the  inhibitory  power 
dwelling  in  the  highest  types  of  cells.  Anything  which  impairs  this 
inhibitory  power  increases  the  tendency  in  the  lower  levels  to  discharge 
irregularly.  It  is  a  fact  of  pathology  that  any  disorganizing  and  de- 
structive lesion  of  the  brain  may  be  productive  of  epilepsy.  And  this 
is  probably  so  because  such  lesions  impair  the  inhibitory  power. 

The  disease  is  one  which  appears  in  early  life  and  is  produced  by 
any  influence  which  impairs  the  full  and  complete  development  of  the 
nervous  system.  It  appears  with  great  frequency  in  children  whose 
nervous  system  is  maldeveloped.  It  is  admitted  that  the  inhibitory 
powers  are  the  culmination  of  functional  development.  It  seems 
likely  therefore  that  epilepsy  is  a  disease  which  in  a  large  majority 
of  cases  indicates  an  impairment  of  inhibitory  power  in  the  brain 
due  to  defective  development  or  to  actual  destructive  disease.  It 
is  a  disease  of  the  cortex  of  the  brain,  for  in  the  cortex  lie  the 
inhibitory  centres.  It  is  a  diffuse,  not  localizable  disease,  because  the 
variety  of  aura  indicate  widespread  starting  points  of  the  disease  in 
different  cases. 

When  convulsions  occur  in  the  course  of  cerebro-spinal  meningitis, 
or  of  pachymeningitis,  or  of  tumors,  abscesses  or  hemorrhages  in  the 
brain  they  are  not  considered  epileptic.  They  are  merely  symptomatic 
of  another  disease  which  is  recognized.  But  such  convulsions  are  in 
no  way  different  from  those  of  epilepsy.  The  presumption,  therefore, 
that  when  a  convulsion  occurs  it  may  be  due  first,  to  some  poison  in 
the  blood,  second,  to  some  infection,  third,  to  some  local  inflammatory 
deposit  in  the  meninges,  or  fourth,  to  some  organic  destructive  or  irri- 
tating lesion  in  the  brain  is  quite  warranted.     Convulsions  are  only 


TEE    THEORY    OF    ORGANIC   DISEASE.  827 

regarded  as^epileptic  when  no  apparent  cause  is  known.  But  this  is 
as  unsatisfactory  as  a  diagnosis  of  dropsy  or  dyspnoea,  and  is  equally 
unscientific.  And  the  apparent  absence  of  a  recognizable  cause  of  the 
disease  in  an  epileptic  is  no  proof  that  such  disease  is  absent.  The 
existence  of  the  symptom  should  rather  be  regarded  as  proof  of  some 
cause  behind  it.  In  many  cases  where  the  diagnosis  has  been  epilepsy 
at  the  outset,  the  outcome  has  shown  that  the  attacks  Were  merely 
symptomatic  of  organic  disease  of  the  brain.  I  believe  that  epilepsy  is 
always  an  expression  of  such  disease. 

It  is  a  chronic  disease  usually  incurable.  Even  the  most  optimistic 
writers  claim  but  six  per  cent,  of  recoveries,  and  this  fact  points  to  an 
underlying  stationary  cause. 

It  is  a  disease  which  is  present  in  forty  per  cent,  of  the  cases  of 
cerebral  atrophy  in  children  where  gross  maldevelopment  of  the  brain 
is  found.  And  in  many  cases  where  no  gross  maldevelopment  can  be 
asserted  the  disease  is  attended  by  such  manifest  traces  of  degeneracy 
and  mental  weakness  that  a  maldevelopment  of  the  brain  is  more  than 
probable. 

The  frequency  with  which  a  history  of  severe  convulsions  in  infancy 
is  found  among  epileptics  is  suggestive.  Such  convulsions  are  not  un- 
likely to  cause  small  hemorrhages  which  in  some  cases  leave  traces  in 
hemiplegia,  paraplegia,  idiocy,  or  sensory  defects,  and  are  thus  recog- 
nizable. In  other  cases  such  hemorrhages  lying  in  brain  areas  whose 
functions  are  unknown  may  be  equally  capable  of  causing  subsequent 
convulsions  without  giving  rise  to  any  other  symptoms. 

It  is  known  that  any  organic  brain  lesion  may  cause  epilepsy, 
but  there  are  certain  forms  of  organic  lesion  which  give  rise  to  few 
symptoms  except  to  epilepsy.  Such  are  multiple  sclerosis,  diffuse 
gliomatosis  of  the  cortex,  sclerosis  of  Ammon's  horn  and  of  the  frontal 
lobes.  These  conditions  have  all  been  found  in  epileptics.  But  the 
absence  of  uniformity  in  their  character  and  location  has  prevented 
their  being  accepted  as  the  lesion  of  the  disease ;  and  this  in  spite  of 
the  fact  that  tumor  or  abscess  or  softening  or  hemorrhage  (very  differ- 
ent forms  of  lesion)  are  known  to  cause  epilepsy,  and  no  matter  where 
any  of  these  lesions  are  located  in  the  brain  the  effect  is  the  same. 

Another  fact  which  points  to  an  organic  basis  of  the  disease  is  the 
uniformity  of  the  aura  in  epilepsy  in  any  given  patient.  In  studying 
irritative  lesions  of  the  cortex  we  have  shown  (page  91)  that  a  forced 
sensation,  or  a  local  spasm  is  a  reliable  guide  to  a  local  lesion.  But 
the  aura  in  epilepsy  is  nothing  but  a  similar  forced  sensation  or  local 
spasm.  In  every  case  when  an  aura  occurs  it  must  be  regarded  as  an 
evidence  of  cortical  irritation.  Experience  shows  that  in  an  epileptic 
tlie  aura  is  always  the  same  in  kind.  Is  not  this  a  positive  indication 
that  the  starting  point  of  irritation  in  that  particular  brain  is  always 
the  same?  And  may  not  the  character  of  the  aura  (visual,  tactile, 
auditory,  etc.),  be  taken  as  a  guide  to  the  seat  of  that  irritation?  If  a 
person  develops  epilepsy  with  a  visual  aura  and  after  a  year  or  two 
begins  U;  present  signs  of  a  brain  tumor  and  hemiopia,  one  would  not 


828  EFtLETSr. 

persist  in  the  diagnosis  of  epilepsy,  one  would  admit  at  once  tliat  the 
epilepsy  was  a  sign  of  the  organic  disease.  But  if  the  epileptic  with 
visual  aura  presents  no  other  signs  of  disease,  shall  we  conclude  that 
his  visual  cortex  is  in  a  normal  state?  I  have  seen  three  children 
brought  for  treatment  of  epilepsy,  in  whom  a  careful  examination  has 
shown  the  presence  of  congenital  hemianopsia  which  had  never  been 
discovered.  In  all  these  cases  a  visual  aura  was  a  symptom.  It  seems 
to  me,  therefore,  most  probable  that  when  in  an  epileptic  a  uniform 
aura  occurs  we  have  evidence  of  a  localized  focus  of  irritation  in 
the  brain.  Statistics  show  that  such  an  aura  is  present  in  40  per  cent, 
of  the  cases.  The  existence  of  traumatic  cases  is  another  proof  of  the 
organic  nature  of  the  disease.  We  have  seen  that  over  11  per  cent, 
develop  as  a  result  of  injury  to  the  head.  And  since  the  development 
of  brain  surgery,  these  cases  are  now  frequently  operated  upon  and  the 
condition  of  the  brain  is  looked  into  near  the  site  of  the  injury.  In 
many  cases  organic  lesions  of  the  brain  have  been  found. 

Thickening  and  adhesions  of  the  meninges,  adhesion  of  the  meninges 
to  the  cortex,  cysts  on  or  in  the  cortex,  patches  of  sclerosis  in  the  cor- 
tex or  extending  deep  into  the  white  matter,  spicula  of  bone  which 
have  penetrated  the  brain,  foreign  bodies  thrust  in  at  the  time  of  the 
fracture  of  the  skull,  local  areas  of  softened  tissue,  the  relic  of  pre- 
vious hemorrhagic  foci  or  of  a  bruise,  small  encapsulated  abscesses  and 
small  calcified  plaques  have  been  found.  I  have  seen  all  these  con- 
ditions in  epileptics  who  have  been  trephined  under  my  direction.  In 
some  cases  the  lesion  was  immediately  under  the  place  on  the  head  which 
was  injured.  In  other  cases  it  was  at  some  distance.  There  is  every 
reason  to  believe  from  analogy  that  a  brain  injury  may  lie  opposite  to 
the  head  injury  and  be  due  to  contre  coup.  And  in  many  cases  it  may 
be  so  deep  as  not  to  be  found.  In  over  half  of  the  cases  in  which  I 
have  seen  operations  done,  nothing  could  be  discovered  at  the  part  of 
the  brain  exposed.  But  as  so  many  cases  have  been  observed  in  which 
lesions  were  found,  I  do  not  doubt  that  in  every  traumatic  case  a 
lesion  exists.  It  is  well  known  that  several  surgeons  have  urged  that 
all  cases  of  epilepsy  be  trephined,  as  they  hope  to  find  and  remove 
the  cause  of  the  disease.  This  only  goes  to  show  that  the  belief  is 
extending  that  the  disease  has  an  organic  lesion,  and  in  these  trau- 
matic cases  there  seems  some  hope  of  finding  it. 

The  theory  that  the  disease  is  due  to  organic  lesion  in  the  brain  has 
also  the  support  of  recent  pathological  observations.  Since  the  found- 
ing of  epileptic  colonies,  the  observation  of  epileptics  has  become  more 
exact  and  their  brains  after  death  have  been  more  carefully  examined. 
It  is  true  that  observers  all  over  the  world  had  found  occasionally  dif- 
ferent forms  of  lesion  in  the  brains  of  epileptics.  Meynert  described 
a  sclerosis  of  Ammon's  horn  as  a  lesion  of  epilepsy  years  ago,  and  re- 
cently Bratz  has  again  called  attention  to  its  frequency  and  Oppen- 
heim  considers  it  one  of  the  many  signs  of  degeneracy  which  are  so 
common  among  epileptics.  Chaslin  found  isolated  plaques  of  sclerosis 
or  gliosis  in  several  brains  of  epileptics,  and  these  have  also  been 


TEE    THEORY    OF    TOXMMIA.  829 

observed  by  Bevavheurs,  Buchholz,  Sailer  (nodular  gliosis)  and  Alz- 
heimer. Onuf  has  found  various  forms  of  atrophy,  and  Clark  and 
Prout  have  found  degenerative  changes  in  cortical  cells  with  chro- 
matolysis,  vacuolization  and  atrophy.  De  Buck  ^  confirms  the  existence 
of  degenerative  changes  in  the  cortical  cells,  but  considers  them  the 
result  of  chronic  toxaemia.  He  likens  epilepsy  to  dementia  prsecox  in 
its  origin  and  lesion.  Moriyasu  ^  has  recently  found  marked  atrophy 
in  the  fibrils  within  the  nerve  cells  throughout  the  cortex  of  the  brain 
in  epileptics.  The  lesions  already  described  as  being  seen  in  trau- 
matic cases  have  been  observed  by  many  observers  in  cases  where  no 
trauma  was  known  as  a  cause.  It  is  true  that  in  some  cases  no  lesion 
has  been  found.  But  this  is  probably  due  to  the  difficulties  attendant 
upon  discovery  of  minute  brain  lesions  when  the  examination  is  not 
made  by  experts.  The  future  will  probably  correct  these  errors. 
While  therefore  it  is  not  contended  that  a  uniform  lesion  of  epilepsy 
will  be  found,  it  seems  probable  that  in  a  large  proportion  of  cases 
some  lesion  exists  in  the  brain  of  such  a  nature  as  to  form  a  focus  of 
irritation  or  a  source  of  weakening  of  normal  inhibition. 

The  Theory  of  Toxsemia.  —  There  are  some  who  hold  that  epilepsy 
is  due  to  a  condition  of  poisoning  in  the  body  from  the  development 
of  toxic  agents  in  the  blood  which  irritate  the  nervous  system.  The 
fact  that  convulsions  similar  in  nature  to  the  major  attacks  of  epilepsy 
occur  in  the  course  of  nephritis  and  of  diabetes  and  also  as  a  sequel  of 
alcoholic  and  other  poisoning  has  led  to  this  theory.  The  poison  may 
be  of  intestinal  origin,  the  toxsemia  of  imperfect  digestion  or  excretion 
as  well  as  of  external  origin,  alcohol,  lead,  etc.  While  such  agents 
undoubtedly  act  as  exciting  causes  of  single  attacks  it  is  not  likely  that 
a  long  continued  disease  of  such  a  kind  can  be  ascribed  to  toxines.  In 
an  epilepticj  gastric  or  intestinal  disturbance  is  likely  to  produce  a  fit, 
and  care  in  the  diet  as  well  as  the  elimination  of  toxsemia  will  diminish 
the  number  of  fits.  But  indigestion  does  not  cause  epilepsy,  and  diet 
alone  will  not  cure  it.  Donath^  holds  that  the  particular  poison 
which  causes  fits  is  cholin.  This  is  a  product  of  nervous  waste,  is 
found  in  excess  in  the  blood  and  cerebro-spinal  fluid  of  some  epileptics, 
but  whether  it  is  a  result  of  the  disease  or  its  cause  is  as  yet  uncertain. 
It  is,  of  course,  quite  possible  that  in  certain  individuals  the  chemistry 
of  nutrition  is  defective  and  that  they  have  a  constant  error  of  me- 
tabolism which  shows  itself  in  the  evolution  of  poison  which  from  time 
to  time  causes  epileptic  attacks.  Handelsman  concludes  from  his 
experiments  that  cholin  is  not  the  poison,  as  he  failed  to  find  it  in  the 
cerebro-spinal  fluid  of  epileptics. 

The  toxic  theory  of  the  disease  receives  a  considerable  amount  of 
confirmation  from  some  recent  experiments  by  Donath,  Hahn,  Massen, 
Nccki,  Pablo  w,  and  K  rain  sky. 

These  observers  have  found  that  if  a  small  amount   of  blood  from 

'  Anatomie  de  I'Epilepsie.     Le  Nevraxe,  June,  1907. 

''Arch.  f.  Psych.,  vol.  xliv.,  p.  84,  1908. 

^Donath,  Deut.  Zeitsch.  fiir  Nervenheilk.,  xxvli.,  p.  72. 


830  EPILEPSY. 

an  epileptic,  who  is  suffering  from  an  attack,  be  injected  into  guinea- 
pigs  and  rabbits,  it  will  produce  convulsions  immediately,  whereas  the 
blood  of  an  epileptic  drawn  during  the  interval  of  freedom  from 
attacks  will  not  produce  this  effect.  Krainsky  has  found  an  appreci- 
able amount  of  ammonium  carbamate  in  this  poisonous  blood.  This 
substance  normally  exists  in  the  blood  and  is  supposed  to  be  a  substance 
formed  from  proteid,  and  to  be  converted  subsequently  into  urea  and 
then  excreted.  Krainsky  believes  that  in  epileptics  this  process  of 
conversion  into  urea,  which  is  thought  to  go  on  in  the  liver,  is  arrested, 
and  that  the  ammonium  carbamate,  accumulated  in  the  circulation, 
acts  as  a  poison  to  the  nervous  system  producing  the  attacks.  Hahn 
and  Pablow  by  establishing  an  artificial  communication  between  the 
portal  vein  and  the  ascending  vena  cava,  thus  arresting  the  function 
of  the  liver  in  modifying  the  blood,  have  proven  that  the  ammonium 
salts  formed  in  the  process  of  proteid  metabolism  are  no  longer  changed 
into  urea  and  thus  accumulate  in  the  circulation.  These  salts  then 
produce,  in  several  animals,  convulsions,  somnolence  and  coma.  It 
would  seem  probable,  therefore,  that  the  carbamate  of  ammonia  may  be 
the  active  poison  in  the  blood  producing  epileptic  attacks.  This  theory 
receives  a  certain  amount  of  support  from  the  generally  accepted 
fact  that  a  diet  rich  in  nitrogenous  material,  or  proteid,  is  sure  to 
increase  the  number  of  epileptic  fits  in  a  person  suffering  from  the 
disease.  It  must  be  admitted,  however,  on  the  other  hand,  that  a 
diet  free  from  nitrogen  does  not  arrest  the  attacks.^  It  is  not  unlikely 
that  further  researches  along  this  line  may  reveal  other  poisons  in 
addition  to  the  ammonia  salts  in  the  blood  of  epileptics.  This  toxic 
theory,  while  capable  of  explaining  the  occurrence  of  individual  fits, 
does  not,  however,  seem  an  adequate  theory  to  account  for  the  disease. 
Some  added  factor,  namely,  an  imperfectly  developed  nervous  system, 
or  a  nervous  system  that  has  been  affected  by  some  organic  disease, 
thus  weakening  its  resistant  power,  is  probably  a  necessary  factor,  for 
there  is  every  reason  to  believe  that  these  varying  constituents  in  the 
blood  occur  in  everyone  from  time  to  time,  but  are  capable  of  produc- 
ing convulsions  in  but  a  small  percentage  of  the  human  race. 

Studies  of  the  blood  in  epilepsy  have  recently  been  made  by  Onuf  and 
Lograsso  and  these  show  that  there  is  no  parallelism  between  seizures 
and  leucocytosis,  in  so  far  as,  even  when  a  distinct  leucocytosis  is 
present,  such  may  reach  its  height  at  different  periods  in  different 
seizures.  Leucocytosis  is  independent  of  the  seizure ;  and  the  seizure 
need  not  necessarily  be  preceded  or  followed  by  a  leucocytosis.^ 

Diagnosis.  — The  diagnosis  of  epilepsy  can  never  be  made  from  one 
or  two  attacks,  as  such  attacks  may  be  merely  symptomatic  of  some 
other  disease.  Only  after  a  number  of  attacks  have  occurred  at 
decreasing  intervals  in  a  person  who  in  the  interim  shows  no  symptoms 
of  disease,  is  the  diagnosis  certain.  If  the  attacks  are  preceded  by  an 
aura,  if  they  follow  the  typical  course,  and  are  followed  by  sleep  the 

^  See  Rosanoff,  Journal  of  Nervous  and  Mental  Disease,  December,  1905, 
^American  Journal  of  the  Medical  Sciences,  February,  1906. 


PBOGNOSIS.  '  831 

diagnosis  may  be  made  after  two  or  three  attacks.  If  the  attacks  are 
of  the  nattfre  of  petit  mal  alone  or  of  psychical  epilepsy  the  diagnosis 
can  be  reached  early.  If  the  attacks  are  unusual,  it  is  well  to  suspend 
judgment  for  some  time.  Many  children  of  nervous  constitution  have 
irregular  epileptiform  attacks  during  early  life  which  are  ascribed  to 
indigestion  or  to  the  irritation  of  dentition,  and  yet  do  not  develop  the 
disease.  Hence  in  infants  and  young  children  the  diagnosis  must  be 
reserved  for  time  to  decide.  When,  however,  such  children  present 
other  signs  of  cerebral  atrophy,  the  diagnosis  may  be  made  early.  In 
adults  the  occurrence  of  a  fit  is  rather  more  suggestive  of  beginning 
organic  nervous  disease,  paresis,  multiple  sclerosis,  brain  syphilis,  brain 
tumor  or  arterial  sclerosis,  than  of  epilepsy,  and  here  again  the  absence 
of  intercurrent  symptoms  and  the  recurrence  of  attacks  afford  the  only 
means  of  diagnosis.  An  ophthalmoscopic  examination  often  enables 
one  to  reach  a  conclusion  early,  as  it  reveals  the  existence  of  organic 
disease.  The  diagnosis  between  epileptic  and  hysterical  attacks  is 
usually  easy.  It  is  possible  in  hysteria  to  obtain  a  history  of  a  neu- 
rotic constitution,  of  a  highly  excitable  temperament,  and  of  a  mental 
shock  or  of  a  long-continued  period  of  anxiety  or  distress  preceding  the 
attack.  This  attack  itself  is  slower  in  onset  and  lasts  a  much  longer 
time,  usually  for  several  hours  instead  of  minutes.  The  patient  never 
injures  herself  by  biting  the  tongue  and  never  wets  herself  in  an  attack. 
Some  sign  of  consciousness  of  surroundings  is  usually  obtainable  and 
the  patient  struggles  with  attendants  with  apparent  design  to  get  away 
from  them.  When  rigidity  occurs  it  is  usually  in  peculiar  postures  as 
with  the  back  arched  and  the  limbs  extended,  and  the  rigidity  alter- 
nates with  struggling.  And  in  hysterical  attacks  the  patient  usually 
talks  as  if  delirious.  There  is  no  rise  of  temperature  during  or  after 
an  attack.  There  is  no  increase  in  the  deep  reflexes  during  a  hysterical 
attack  and  the  Babinski  reflex  cannot  be  obtained.  In  epileptic 
attacks  the  reflexes  are  increased.  The  face  is  not  congested,  cyanotic 
and  mottled  in  hysterical  attacks,  as  it  is  in  the  first  stage  of  grand 
mal.  The  attack  terminates  by  a  gradual  or  sudden  return  to  con- 
sciousness and  not  by  sleep. 

Convulsions  due  to  uraemia,  or  to  diabetes,  or  to  alcoholism  cannot 
be  distinguished  from  epileptic  attacks  except  by  the  discovery  of  the 
symptoms  of  these  affections. 

Prognosis.  —  The  facts  in  the  consideration  of  the  etiology,  of  the 
theory  of  organic  origin  of  the  disease,  and  of  the  usual  course  of  epi- 
lepsy tend  to  the  conclusion  that  the  prognosis  in  epilepsy  is  almost 
always  unfavorable.  Recoveries  are  the  rare  exception  (5  per  cent.  ?) 
and  this  fact  should  not  be  concealed  from  the  parents  or  friends  of 
patients.  The  earlier  the  onset,  the  more  severe  and  frequent  the 
attacks,  the  deeper  the  coma  and  the  more  intense  the  mental  con- 
fusion after  an  attack,  the  worse  is  the  prognosis.  Nocturnal  epilepsy 
has  a  better  prognosis  than  diurnal,  as  the  chances  of  injury  are  less 
while  a  patient  is  asleep.  The  prognosis  in  petit  mal  is  not  better 
because  the  disease  is  petit  mal,  in  fact  mental  deterioration  seems  to 


832  EPILEPSY. 

be  of  earlier  onset  in  these  eases.  The  prognosis  in  cerebellar  epilepsy 
—  vertigo  with  or  without  running  attacks  —  is  worse  than  in  grand 
mal.  The  better  the  general  constitution  and  digestive  powers  of  the 
patient,  and  the  better  his  reaction  to  drugs,  the  better  the  prognosis. 
In  the  cases  where  bromides  are  not  well  taken  the  prognosis  is  bad, 
as  relief  is  difficult.  About  ten  per  cent,  of  epileptics  eventually 
become  insane. 

Treatment.  — An  epileptic  should  lead  a  life  free  from  anxiety, 
responsibility  or  eifort.  It  should  be  a  life  out  of  doors  with  healthful 
surroundings  and  agreeable  occupation,  in  the  country  rather  than  in 
town.  His  diet  should  be  simple  and  in  moderate  amount,  but  nutritious, 
and  its  chief  limitations  should  be  in  regard  to  red  meats,  and  rich  foods. 
He  should  avoid  all  alcoholic  beverages,  but  coffee  and  tea  in  modera- 
tion may  be  allowed.  Tobacco  may  be  permitted.  If  his  digestive 
organs  are  defective,  greater  care  in  diet  and  a  more  rigid  regimen  are 
to  be  prescribed,  but  the  ordinary  epileptic  does  not  need  such  restric- 
tions, though  occasionally  attacks  may  be  traced  to  over-eating.  In 
some  cases  for  example  a  simple  milk  diet  for  a  month,  or  a  diet  of 
vegetables  and  cereals  with  eggs  only,  for  a  short  time,  or  the  abso- 
lute abstention  from  meat  in  any  form  may  prove  of  benefit.  Such 
restrictions  should,  however,  be  temporary  measures,  as  too  long  con- 
tinued abstinence  from  the  foods  which  experience  has  shown  the 
human  system  to  require  is  harmful.  His  food  must  be  chewed  finely, 
as  the  tendency  is  to  bolt  the  food  and  to  over-eat.  His  general  con- 
dition should  be  kept  up  by  daily  baths,  either  a  3-miuute  hot  (103°) 
bath  followed  by  a  cold  (75°)  sponge  off,  or  a  cold  shower  bath.  A 
full  amount  of  daily  exercise  is  to  be  taken,  so  distributed  that  over- 
fatigue at  any  one  time  may  be  avoided.  Study  and  reading  are  not 
to  be  neglected  but  should  be  done  with  intervals  of  walking  or  exer- 
cise or  play,  so  as  not  to  demand  too  long  continued  mental  eifort.  As 
epileptic  children  are  excluded  from  schools,  special  teachers  should  be 
employed  by  those  who  can  afford  it,  for  the  resources  of  a  liberal  edu- 
cation are  needed  in  later  life  by  this  class  of  invalids.  Mental  occu- 
pation should  be  encouraged  in  every  possible  way,  as  idleness  is  con- 
ducive to  evil  habits,  and  to  introspection  and  unhappiness.  Marriage 
is  to  be  discouraged,  as  the  offspring  is  likely  to  be  degenerate  in  type, 
and  there  is  no  greater  nervous  strain  than  that  of  living  with  a  per- 
son liable  to  an  attack  of  an  epileptic  nature. 

Epileptics  should  be  informed  of  the  nature  of  their  disease  when 
they  reach  years  of  discretion  and  be  warned  of  the  danger  they  expose 
themselves  to  in  going  about  alone,  in  standing  near  railway  tracks  or 
on  high  places,  in  going  alone  in  boats,  in  riding  the  bicycle  or  on 
horseback  or  in  swimming.  Such  warning  is  soon  enforced  by  the 
occurrence  of  injuries  during  attacks  and  may  prevent  serious  accidents. 
Epileptics  should  always  be  under  some  supervision  and  attendance,  to 
prevent  the  occurrence  of  serious  injuries.  The  watching  may  be 
resented  but  it  is  necessary. 

In  the  medical  treatment  the  first  thing  is  to  seek  for  and  to  remove 
if  possible  any  form  of  peripheral  irritation  which  may  act  as  an  ex- 


TREATMENT.  833 

citant  of  nervousness.     Reference  to  the  section  on  etiology  may  here 
be  made,  where  these  irritants  are  discussed. 

Another  matter  to  be  looked  into  is  the  condition  of  the  digestion ; 
and  in  case  the  existence  of  indican,  skatol  or  other  products  of  imper- 
fect metabolism  in  the  urine  shows  a  state  of  intestinal  toxaemia,  the 
continued  use  of  borax,  naphthaline,  salol,  salicin,  sulpho-carbolate  of 
soda,  benzoate  of  soda  and  remedies  of  this  type  is  indicated.  There 
are  so  many  patients  in  whom  each  attack  can  be  traced  to  a  state  of 
indigestion  that  I  regard  this  line  of  treatment  of  almost  as  much  im- 
portance as  the  use  of  bromides. 

A  free  daily  action  of  the  bowels  is  to  be  enforced,  by  the  use  of  fruits, 
plenty  of  water,  massage  of  the  abdomen  and  the  cultivation  of  a  habit 
of  going  to  stool  at  a  definite  time.  Laxatives  and  purgatives  are  to 
be  avoided,  as  once  begun  they  will  have  to  be  kept  up  indefinitely. 
An  occasional  dose  of  calomel  or  podophylliu  will  do  no  harm  and  may 
be  indicated  from  time  to  time. 

The  remedies  for  the  disease  in  the  order  of  their  efficacy  are  brom- 
ides, simulo,  solanum  and  chloral.  The  bromide  of  potassium,  sodium, 
ammonium,  lithium  and  strontium  given  in  watery  solution,  bromipin 
given  in  sesame  oil  and  bromotone  given  in  capsules  and  hydrobromic 
acid  are  the  various  methods  of  giving  bromides.  It  is  wise  to  try 
one  after  another  of  these  and  to  select  for  permanent  use  the  one 
which  seems  to  agree  best  with  the  patient.  Some  patients  bear  one 
better  than  others.  Some  bear  a  combination  of  several  better  than 
any  one  alone.  Some  do  better  when  the  bromide  is  given  in  one 
large  dose  at  night,  or  in  two  doses  one  at  night  and  one  on  waking. 
Others  seem  to  absorb  the  bromide  more  readily  when  it  is  given  after 
each  meal.  Where  regularity  in  the  use  of  a  remedy  is  necessary,  a 
dose  on  waking  and  on  retiring  is  the  best  method.  Nothing  should 
be  added  to  the  bromide,  as  a  simple  salty  taste  is  least  objectionable  in 
the  long  run.  Bromipin  is  given  in  sesame  oil,  the  dose  of  one  table- 
spoonful  being  equivalent  to  20  grains  of  bromide  of  potassium. 
Brometone  is  given  in  capsules  of  5  grains,  one  capsule  being  equiva- 
lent to  20  grains  of  bromide  of  potassium.  One  drachm  of  dilute  hydro- 
bromic acid  is  equal  to  30  grains  of  bromide.  The  dose  of  bromide 
must  be  determined  for  each  patient  by  observation.  It  is  well  to 
begin  in  a  child  of  ten  with  20  grains  at  night,  in  a  child  of  fifteen 
with  30  grains,  and  in  an  adult  with  40  grains,  and  this  dose  may  be 
increased  week  by  week  if  the  attacks  do  not  yield  to  it,  up  to  150 
grains  a  day  for  an  adult.  Larger  doses  cannot  be  continued  indefi- 
nitely. The  smallest  dose  which  will  hold  the  attacks  in  check  is  the 
dose  to  be  permanently  taken.  It  is  useless  to  take  bromide  for  a 
short  time  or  to  intermit  its  use.  It  should  be  kept  up  for  six  years 
at  least  if  begun  at  all,  and  if  this  is  understood  by  the  patient  at  the 
outset  ho  may  be  more  willing  to  persist.  When  the  system  is  satu- 
rated with  l>romide  the  bromide  may  be  safiily  stopped  for  ten  days  in 
case  of  absolute  need  —  as  in  an  intercurrent  illness  —  but  after  two 
weeks  all  the  bromide  has  been  eliminated  from  the  system  and  no 
effects  remain. 
(i3 


834 


EPILEPSY. 


It  is  well  to  add  to  any  bromide  salt  which  is  used  some  glycero- 
phosphate of  soda,  20  grains  three  times  a  day  for' an  adult  being  the 
usual  dose.  This  seems  to  counteract  some  of  the  ill  effects  of  bromide 
and  at  the  same  time  is  an  excellent  food  for  the  nervous  system.  The 
two  can  be  combined  in  the  same  solution.  While  bromide  is  being 
given  patients  should  use  little  or  no  salt  with  their  food. 

The  ill  effects  of  bromide  are  first  to  disturb  the  stomach  by  setting 
up  a  catarrhal  inflammation.  This  may  be  avoided  in  some  degree  by 
giving  it  very  freely  diluted  in  water.  Sometimes  the  use  of  poor 
preparations  of  bromide  which  contain  arsenic  is  responsible  for  the 
irritation  of  the  stomach.  The  second  ill  effect  is  the  appearance  of 
acne.  Proper  care  of  the  skin,  by  hot  baths  and  the  free  use  of  soap 
followed  by  massage,  prevents  this  effect  in  some  patients.  In  others 
the  addition  of  small  doses  of  arsenic  given  not  longer  than  one  week 
at  a  time  will  prevent  the  acne.     The  pustules  should  be  pricked  and 

the  contents  expressed,  and  an 
Fig.  309.  antiseptic     lotion     or    ointment 

applied.  If  the  pustules  become 
confluent  and  large  thick  crusts 
form  the  bromide  must  be  stopped 
for  two  or  three  weeks  even  at 
the  risk  of  attacks  ;  its  elimina- 
tion hastened  by  increased  water 
drinking  and  saline  laxatives, 
and  the  surface  dressed  aseptic- 
ally  until  it  heals. 

The  third  ill  effect  of  bromide 
is  its  effect  upon  the  mind.  In 
all  cases  it  dulls  mental  proc- 
esses, makes  the  perception  less 
keen,  the  memory  poor,  and 
reasoning  slower.  This  is  in- 
evitable but  these  effects  are 
temporary  and  cease  when  the 
bromide  is  stopped.  In  a  few 
cases,  which  I  have  seen,  bro- 
mide causes  active  mania  or  all 
the  symptoms  of  the  early  stage 
of  paresis.  In  these  cases  its  use 
must  be  abandoned.  Epileptics 
who  are  taking  bromide  should 
be  watched  for  occasional  cumu- 
lative effects,  and  when  these  appear  the  drug  should  be  stopped  for 
a  time. 

The  effect  of  bromide  on  the  disease  is  to  lengthen  the  interval 
between  attacks,  and  they  may  even  stop  entirely  after  the  intervals 
have  been  lengthened  to  several  months.  The  dose  should  be  kept  up 
for  two  years  after  the  last  attack  and  then  gradually  reduced  during 
one  year  and  finally  stopped.      It  is  sometimes  found  that  a  rapid 


Bromide  eruption  iu  an  epileptic. 


TBEATMENT.  835 

graduated  increase  in  the  amount  of  bromide  given  from  20  grains  up 
to  150  grains  at  night,  and  then  a  gradual  decrease  to  the  original  dose 
has  a  good  effect.  Such  a  course  can  be  spread  over  six  months  and 
may  be  repeated  once  a  year. 

Chloral  hydrate  in  dose  of  5  up  to  15  grains  three  times  a  day  may 
be  added  to  bromide,  and  often  aids  its  effects.  This  drug  may  be 
given  by  rectum  in  the  status  epilepticus.  It  cannot  be  continued  very 
long  without  causing  depressing  effects  on  the  heart. 

Tincture  of  belladonna  has  been  given  with  bromide  in  gradually 
increasing  doses.     I  have  never  had  any  favorable  results. 

Tincture  of  digitalis  has  been  given  with  bromide  in  cases  where  the 
heart  was  feeble.  I  have  not  been  convinced  of  any  effect  upon  the 
epilepsy. 

When  bromide  fails  of  effect  or  has  to  be  stopped  because  of  its  ill 
effects  tincture  of  simulo  may  be  used  in  1  up  to  3  drachm  dose  three 
times  a  day.  I  have  seen  good  effects  from  it  and  regard  it  is  a  valu- 
able remedy.  It  may  be  continued  a  long  time,  and  often  diminishes 
the  number  of  fits.  It  may  be  given  with  bromide,  thus  enabling  one 
to  reduce  the  dose  of  the  latter. 

Fluid  extract  of  solanum  caroliuense  (horse  nettle)  in  dose  from  1 
to  4  drachms  three  times  a  day  is  also  a  valuable  remedy  either  with, 
or  without  bromides. 

Calcium  lactate  in  doses  of  15  grains  three  times  a  day  has  been  of 
service  in  some  cases.  I  have  had  success  with  this  remedy,  and  it 
appears  to  have  no  ill  effect. 

Antipyrin  is  the  only  one  of  the  coal  tar  derivatives  which  thus  far 
has  been  found  to  have  any  effect  upon  epilepsy.  Given  in  15  grain 
powder  at  night  and  15  grains  on  rising,  it  sometimes  mitigates  the 
attacks. 

Chlorotone,  a  synthetic  product  of  Parke,  Davis  &  Co.,  will  decrease 
epileptic  attacks  but  renders  the  patient  so  stupid  and  dizzy  that  its 
use  must  be  limited  to  a  short  time.  It  acts  well  to  arrest  a  series  of 
severe  attacks. 

In  a  status  epilepticus  large  doses  of  bromides  and  chloral  by  rectum 
may  be  of  service,  and  sometimes  the  fits  may  cease  under  chloroform 
inhalations  given  carefully  for  several  hours. 

In  a  few  cases  where  an  aura  of  some  duration  precedes  the  attack, 
the  inhalation  of  amyl  nitrite  may  arrest  the  attack.  Pearls  of  this 
can  be  carried  and  broken  in  the  handkerchief  or  a  bottle  filled  with 
cotton  wool  soaked  in  amyl  nitrite  can  be  carried  and  inhaled.  In 
some  cases  the  aura  consists  of  a  numb  feeling  in  one  limb.  A  band 
tied  tightly  about  his  limb  when  the  numbness  is  first  felt  has  been 
known  to  arrest  the  attack.  One  of  ray  patients  wears  a  strap  about 
his  wrist  and  pulls  it  tight  when  he  feels  the  tingling  in  the  fingers  and 
thus  aborts  the  fit.  In  senile  cases  or  in  cases  due  to  arterial  sclerosis, 
the  use  of  nitroglycerine  y,|jj  grain  two  to  five  times  daily,  or  of  ni- 
trite of  sodium  •'{  grains  four  times  daily,  combined  with  heart  stimu- 
lants, is. of  much  more  service  than  the  use  of  bromides.  It  is  in  these 
cases  that  chloral  in  3  to  5  grain  dose  four  times  a  day  and  also  5  grain 


836  EPILEPSY. 

doses  of  potassium  iodide  has  been  of  some  service.  In  old  persons  a 
drink  of  whiskey  will  sometimes  abort  an  attack. 

I  have  never  found  much  effect  from  any  remedy  in  petit  mal, 
though  the  attacks  may  diminish  under  any  of  the  lines  of  treatment 
described.  Occasionally  nitroglycerine  in  ^-i-^  grain  dose  two  to  four 
times  a  day  has  a  favorable  effect.  Oxide  of  zinc  5  grains  after  each 
meal  in  capsule  has  been  of  some  service  in  epilepsy. 

Borax  15  to  25  grains  after  each  meal  given  in  powder  has  done  good. 

Forel,  of  Zurich,  recommends  a  course  of  treatment  by  opium,  the 
patient  being  put  to  bed  and  fully  narcotized  for  six  weeks,  the  opium 
being  then  slowly  diminished,  and  a  larger  dose  of  bromide  being 
given  daily  as  the  opium  is  decreased.  I  have  not  had  good  results 
from  this  course  of  treatment.  I  have  known  the  opium  habit  to  be 
induced.  It  is  a  method  which  is  fallipg  into  disrepute.  Opium  and 
morphine  should  be  avoided  in  epileptics. 

In  an  attack  nothing  can  be  done  except  to  prevent  injuries,  to  pre- 
vent if  possible  biting  of  the  tongue,  by  placing  some  hard  substance 
between  the  teeth  and  to  prevent  congestion  of  the  head  by  loosening 
the  neck  band.  The  patient  should  always  be  permitted  to  sleep  after 
the  attack  as  long  as  he  desires.  A  warm  salt  bath  will  relieve  the 
fatigue  of  the  muscles  which  is  inevitable. 

Surgical  Treatment.  —  There  is  a  small  percentage  of  cases  of  epilepsy 
open  to  surgical  treatment.  When  a  focus  of  disease  in  the  brain  can 
be  exactly  located  surgery  may  remove  it.  In  cases  following  injury 
or  depressed  fractures  of  the  skull  it  is  always  possible  that  a  spiculum 
of  bone  may  be  irritating  the  brain  and  in  these  cases  its  removal  is 
indicated.  I  have  published  elsewhere^  numerous  cases  of  this  kind. 
In  cases  where  the  aura  or  attack  is  distinctly  cortical  in  type,  is  uni- 
form, and  is  not  attended  at  the  outset  by  a  loss  of  consciousness  it  is 
often  possible  to  locate  some  focus  of  irritation  if  the  case  is  studied 
with  the  facts  concerning  localization  mentioned  on  pages  91  to  96 
in  mind.  In  such  cases  exposure  of  the  cortex  may  reveal  a  plaque 
of  adherent  meninges  or  a  small  area  of  sclerosis  or  of  gliosis  and 
this  niay  be  removed.  So  many  tumors  begin  with  epileptic  attacks 
that  this  procedure  is  advocated  whenever  the  attacks  are  distinctly 
localizable. 

In  all  non-traumatic  cases  with  no  localizing  symptoms  surgical 
treatment  is  entirely  unwarranted  and  useless.  Even  in  the  cases  in 
which  it  is  indicated  and  properly  carried  out  it  is  not  uniformly  cura- 
tive. Any  scar  in  the  brain,  whether  produced  by  disease  or  by  a 
surgeon's  knife,  may  act  as  an  irritant  and  cause  attacks.  Hence 
after  any  operation  on  the  brain,  even  for  the  removal  of  tumors  or 
abscess,  epilepsy  may  develop  as  a  sequel.  It  is  not  surprising  there- 
fore that  even  in  cases  of  epilepsy,  which  have  been  trephined,  and  in 
which  some  focus  of  disease  has  been  found  and  removed,  the  patient 
may  have  a  recurrence  of  the  attacks.  The  surgical  treatment  of  even 
selected  cases  of  epilepsy  has  not  been  as  successful  as  was  at  first 
expected. 

^  Brain  Surgery,  William  Wood  &  Co. 


CHAPTEE,  L. 

NEURASTHENIA  AND  THE  OCCUPATION  NEUROSES. 

Neurasthenia  is  a  condition  of  exhaustion  in  the  general  nervous 
system.  It  may  be  general,  all  parts  of  the  system  being  affected. 
It  may  be  local,  either  the  brain,  or  the  spinal  cord,  or  the  vasomotor 
system  being  chiefly  involved.  Hence  its  symptoms  vary  widely  in 
diflPerent  cases,  and  are  almost  as  numerous  as  the  various  activities 
possible  in  mind  and  body. 

Every  act  of  mind  or  body  is  attended  by  certain  chemical  and 
physical  changes  in  the  cells  of  that  part  of  the  nervous  system  con- 
trolling the  act.  These  go  on  for  a  time  within  physiological  limits 
without  harm.  But  if  continued  beyond  those  limits  they  result  in 
exhaustion  of  the  cells,  giving  rise  to  uncomfortable  sensations  of 
fatigue  and  inability  to  continue  work.  The  initial  state  of  the  cells  ; 
their  degree  of  development ;  their  inherent  strength  ;  and  their  state 
of  nutrition  determine  the  limit  of  their  endurance ;  for  what  is  healthy 
effort  to  a  strong  man  may  be  impossible  overstrain  for  a  weak  one. 
The  condition  of  exhaustion  may  be  rapidly  or  slowly  compensated  for 
in  different  conditions  or  in  different  persons ;  the  capacity  for  recu- 
peration differing  widely.  But  it  is  probable  that  permanent  changes 
are  rarely  produced  in  cells  by  work  and  that  a  return  to  a  normal 
state  is  the  rule  when  sufficient  rest  is  given.  (See  page  27.)  Edinger 
however  believes  that  if  the  cells  of  any  part  of  the  nervous  system 
are  overstrained  by  excessive  work  they  may  undergo  a  process  of 
degeneration,  which  in  turn  may  be  followed  by  a  secondary  sclerotic 
process  in  the  connective  tissue  which  holds  the  cells  together.  When 
such  sclerotic  changes  opcur  they  are  permanent  and  form  the  basis  of 
organic  disease.  Hence  he  believes  that  overwork  may  cause  perma- 
nent inefficiency.  His  views  are  so  important  that  the  following 
summary  is  quoted : 

"  The  overfunctioning  of  a  sound  nerve,  or  normal  functioning  of  a 
degenerated  or  intoxicated  nerve  causes  fatigue  which  may  amount  to 
exhaustion,  and  the  power  of  recuperation  may  be  transiently  or  per- 
manently lost.  Poisons,  like  alcohol  and  lead,  reduce  the  recuper- 
ative power,  and  nerves  subjected  to  strain  from  the  occupation  may 
experience  exhaustion  and  ultimate  paralysis  and  atrophy.  Too  little 
attention  has  been  paid  hitherto  to  the  questions,  What  becomes  of  a 
normal  nerve  subjected  to  unduly  great  strain,  or  of  a  weak  nerve  of 
which  normal  functioning  is  demanded?  and  How  does  the  central 
organ  fare  with  these  absolute  or  relative  overdemands?  A  constitu- 
tional, defective  composition  of  a  part  leads  to  atrophy  when  func- 

837 


838         NEUBASTHENIA    AND    THE    OCCUPATION    NEUEOSES. 

tional  demands  are  made  on  it.  A  similar  process  occurs  in  another 
group  of  nervous  affections  which  are  not  congenital  but  result  from 
the  action  of  poisons,  as  in  the  nervous  affections  after  ergot  poisoning, 
after  pellagra  and,  above  all,  after  infection  with  syphilis,  as  in  tabes 
and  paralysis.  All  these  have  an  injurious  influence  even  on  neurones 
of  which  only  normal  function  is  demanded." 

•  Etiology. — Predisposing  causes  are  prominent  in  the  production 
of  neurasthenia.  In  the  majority  of  cases  it  is  possible  to  discover 
some  factor  in  the  heredity  of  the  patient,  which  has  induced  an  imper- 
fect development  and  consequent  inherent  weakness  of  his  nervous 
system.  One  or  both  parents  may  have  been  the  subject  of  some 
disease,  either  nervous  or  of  any  other  character  ;  they  may  have  been 
subject  to  intoxication  by  alcohol,  drugs,  or  autoinfection  ;  they  may 
have  been  old  or  feeble  ;  they  may  have  been  suffering  from  overwork 
or  strain  of  a  mental  or  emotional  kind,  or  they  may  themselves  have 
been  neurasthenic.  Anxiety  or  illness  in  a  mother  during  pregnancy 
impairs  the  strength  of  the  child's  brain.  Under  these  conditions  the 
child  is  born  with  a  nervous  system  incapable  of  the  highest  develop- 
ment, and  unable  to  stand  exhausting  strains,  and  defective  in  recup- 
erative power ;  hence  he  becomes  an  easy  prey  to  the  exciting  causes 
of  the  disease  in  later  life.  This  factor  is  often  overlooked  both 
by  patients  and  by  physicians,  who  are  disappointed  at  the  lack  of 
result  from  treatment  or  from  periods  of  rest,  which  otherwise 
would  be  effective.  A  firm  building  cannot  be  erected  on  a  foun- 
dation of  sand. 

The  exciting  causes  are  numerous.  A  life  of  too  continued  effort 
without  proper  periods  of  rest  and  sleep,  without  proper  food  and 
proper  exercise,  and  harassed  by  the  worry  which  the  struggle  for 
success  entails  is  the  history  usually  obtained  from  a  neurasthenic. 
Often  alcoholic  or  sexual  indulgence  is  an  added  cause.  Sometimes 
some  acquired  disease,  especially  syphilis,  some  infectious  disease,  e.  g., 
grippe,  or  some  chronic  exhausting  affection,  like  intestinal  or  gastric 
indigestion,  nephritis,  gout  or  rheumatism  is  the  evident  exciting  cause. 

Sometimes  severe  shocks  or  intense  anxiety  or  grief  are  productive 
of  the  disease  ;  and  worry  may  be  said  to  cause  more  cases  than  over- 
work alone.  Thus  family  care  or  affliction,  the  distress  of  mind  con- 
sequent upon  money  losses,  or  illness  or  ill  conduct  of  some  relative, 
an  unfortunate  love  affair,  a  disappointed  ambition,  or  intense  remorse 
are  causes  of  neurasthenia. 

A  sudden  change  in  one's  habit  of  life  may  so  disarrange  the  chem- 
istry of  nutrition  as  to  produce  neurasthenia.  Thus  athletes  who  stop 
exercise,  hard  workers  who  rest  completely,  women  who  make  a  com- 
plete change  of  diet  or  who  stop  taking  fluids  in  order  to  get  thin, 
people  who  suddenly  adopt  some  fad  in  their  food,  men  who  do  un- 
usual work  at  night  and  sleep  little  by  day,  persons  who  subject  them- 
selves to  great  changes  of  altitude  or  climate,  are  frequently  liable  to 
develop  neurasthenia. 

Great  excess  in  work,  especially  if  attended  by  anxiety,  is  a  very 


ETIOLOGY.  839 

frequent  cause.  Thus  college  students  after  final  examinations,  pro- 
fessional men  after  an  intense  effort  in  the  pulpit  or  at  the  bar,  or 
medical  men  after  trying  cases,  or  engineers  after  unusual  exertions 
often  develop  an  acute  neurasthenia. 

In  women  menstrual  disorders,  or  uterine  and  ovarian  disease  are 
very  likely  to  cause  continued  irritation  and  exhaustion  of  the  nervous 
system.  A  laceration  of  the  perineum  with  its  secondary  displace- 
ments of  uterus  and  bladder  may  be  a  source  of  constant  sensory  irri- 
tation and  discomfort",  which  after  months  may  so  wear  upon  the  ner- 
vous system  as  to  produce  a  severe  general  neurasthenia.  Excessive 
maternal  cares  are  also  an  occasional  cause.  In  old  age  the  condition 
is  rarely  seen,  as  the  old  are  less  subject  to  strain  and  to  worry. 

Neurasthenia  has  been  ascribed  to  "  reflex  irritation."  By  this  is 
meant  that  in  some  organ  of  the  body,  e.  g.,  eye,  or  ear  or  nose,  or 
genitals,  etc.,  a  condition  is  present  which  causes  constant  nervous  irri- 
tation ;  and  such  constant  irritation  by  the  law  of  summation  of  im- 
pulses results  in  an  occasional  nerve  storm  which  disturbs  the  nutri- 
tion of  the  nervous  centers  and  leads  to  irregular  and  defective  action. 
Some  of  the  worst  cases  I  have  seen  have  been  due  to  ulceration  of  the 
rectum  long  overlooked,  or  to  chronic  urethritis  with. granulations. 
This  subject  is  more  fully  considered  in  the  chapter  upon  Reflex  Neu- 
roses, page  909. 

Neurasthenia  has  also  been  ascribed  to  chemical  poisons  developed 
in  the  body  by  imperfect  metabolism,  uric  acid  poisoning,  lithemia, 
oxaluria,  or  uremia  being  found  in  many  cases.  The  uric  acid  dia- 
thesis is  undoubtedly  a  factor  in  some  cases,  and  there  are  many  per- 
sons who  become  neurasthenic  unless  they  take  occasional  doses  of 
calomel,  use  alkaline  waters  constantly  and  refrain  from  a  diet  con- 
taining red  meat  and  alcohol.  It  is  a  question  whether  chronic  auto- 
intoxication by  defective  digestion  is  a  cause  or  a  result  in  neuras- 
thenics, but  the  two  conditions  are  very  frequently  combined.  Atonic 
states  of  the  stomach  with  enteroptosis,  distension  of  the  intestines  and 
chronic  colitis,  rectal  diseases,  especially  bleeding  piles,  strictures  and 
fissures,  are  certainly  liable  to  give  rise  to  neurasthenic  states.  The 
excessive  use  of  alcohol,  or  tobacco,  or  coffee,  or  tea,  may  lead  to 
the  development  of  neurasthenia.  The  neurasthenia  seen  among 
syphilitics  is  occasionally  due  to  that  disease ;  it  is  more  commonly 
due  to  the  exhausting  effect  of  long  courses  of  treatment;  and  it 
is  sometimes  entirely  independent  of  the  disease  and  due  to  the 
worry  or  fear  that  the  patient  feels  in  consequence  of  his  infection,  his 
anxiety  to  marry,  or  his  fear  of  transmitting  his  disease  to  his  wife 
or  children. 

The  importance  of  errors  on  the  action  of  the  ductless  glands  as  a 
cause  of  neurasthenia  has  been  urged  by  Hertoghe  ^  and  by  Levi  and 
Rothschild.^     Temporary  cessation  of  secretion,  or  temporary  increase 

'Bull,  de  la  Soc.  Med.  de  Bruxelles,  1909. 

^Etudes  sur  la,  phy.sio-patiiologie  du  Coi-jjs  Thyroide  et  do  F liypophyse  (p.  02),  Doin, 
Pariw,  1908. 


840        NEVEASTEENIA    AND    TEE    OCCUPATION   NEUROSES. 

of  secretion  of  the  thyroid  gland  may  produce  neurasthenic  symptoms ; 
in  the  first  case  attended  by  minor  signs  resembling  the  symptoms  of 
myxoedema,  a  sensation  of  chilliness,  dryness  of  the  skin  and  hair,  swell- 
ing of  the  hands  and  neck,  a  slow  pulse  and  diminished  menstruation ; 
in  the  second  case  signs  resembling  those  of  Graves'  disease,  feelings 
of  heat,  sweating,  emaciation,  a  rapid  pulse  and  severe  headache  with 
too  profuse  menstruation.  These  can  best  be  treated  in  the  first  case 
by  administration  of  small  doses  of  thyroid  extract  and  in  the  second 
by  the  use  of  pituitary  extract,  or  belladonna. 

Injuries  of  the  body,  especially  if  attended  by  mental  shock  and 
fright,  are  capable  of  setting  up  a  severe  type  of  neurasthenia.  This 
is  particularly  frequent  after  railway  and  automobile  accidents  ;  and 
has  been  termed  traumatic  neurasthenia  or  the  "  traumatic  neurosis." 
It  diifers  from  ordinary  neurasthenia  in  the  presence  of  more  acute, 
intense  and  variable  symptoms,  in  the  prominence  of  mental  symptoms, 
many  of  which  are  of  an  hysterical  kind,  and  in  its  longer  course.  It 
is  probable  that  the  concussion  of  the  body  in  such  accidents  produces 
a  physical  change  in  the  cells,  which  for  a  long  time  hampers  their 
normal  metabolism  and  causes  a  permanent  exhausted  state.  The 
fright  probably  causes  a  chemical  change  in  them,  with  similar  results. 

The  disease  is  more  common  in  men  than  in  women.  It  is  an  auc- 
tion of  adult  life,  but  many  cases  develop  early,  the  large  majority  be- 
fore the  age  of  forty,  during  the  period  of  greatest  eifort.  Occasion- 
ally children  are  affected,  especially  those  who  are  pushed  in  school 
and  are  made  to  share  in  adult  amusements.  It  is  more  common  in 
cities  than  in  the  country.  It  is  more  common  among  the  better  classes 
and  among  the  highly  educated,  than  among  the  laboring  class.  Cli- 
mate does  not  enter  as  a  factor,  as  it  develops  in  both  north  and  south ; 
on  the  plains  and  in  the  mountains  ;  but  when  a  person  is  neurasthenic, 
change  of  altitude  has  a  marked  effect.  It  is  more  common  in  winter 
than  in  summer.  It  is  more  frequent  among  those  people  who  live 
under  constant  strain  and  efibrt,  with  few  rational  amusements ;  and 
who  take  little  out  of  door  exercise ;  hence  the  disease  is  more  com- 
mon among  Americans  than  among  Germans  or  English.  It  is  a 
disease  of  civilization  and  incidental  to  the  ambition  of  active  men. 
In  women  it  is  often  the  outcome  of  a  congenitally  weak  nervous 
system,  over-trained  in  school,  or  subjected  to  disappointment  in  love. 
Sometimes  ambitions  beyond  capacity,  or  the  distress  after  marriage 
of  an  unhappy  or  a  childless  life  produce  it. 

Symptoms.  —  These  may  be  classified  into  mental,  cerebral,  spinal, 
sexual,  vaso-raotor,  and  sympathetic. 

The  mental  and  cerebral  symptoms  are  the  most  common.  There 
is  always  a  certain  consciousness  of  our  mental  processes,  attended 
by  a  sense  of  enjoyment  or  of  distaste,  but  this  in  a  normal  person 
is  never  very  vivid.  The  mind  rarely  watches  itself  as  it  acts,  and 
is  not  conscious  of  its  application,  of  storing  up  impressions,  or 
of  its  acts  of  reasoning.  Thus  we  have  our  attention  fixed  on  the 
result,  and  not  on  the  process  of  thought.     If  mental  action  becomes 


SYMPTOMS.  841 

hampbred  gne  of  the  first  results  is  to  attract  our  attention  to  the 
process,  and  away  from  the  end.  The  mind  labors  in  its  work.  It 
becomes  difficult  to  fix  attention.  It  is  hard  to  remember.  It  is  im- 
possible to  reason  consecutively  and  accurately.  Impressions  leave 
little  trace.  Logical  sequences  do  not  follow  from  premises.  And  in 
consequence  of  this  difficulty  in  mental  activity  a  sense  of  discomfort 
arises,  a  distaste  for  mental  occupation  and  a  feeling  of  repugnance  to 
effort  or  to  labor.  This  is  what  the  neurasthenic  feels  and  complains 
of.  He  cannot  put  his  mind  on  his  work.  He  reads,  but  forgets  what 
he  has  read.  He  cannot  come  to  any  conclusion  about  anything,  living 
in  a  state  of  perplexity.  He  accomplishes  nothing,  and  this  fact  alone 
causes  annoyance  and  distress.  He  soon  becomes  irritable  not  only  at 
himself  and  his  state,  but  at  his  surroundings,  family  and  friends.  He 
wishes  to  be  alone,  and  then  broods  on  his  condition,  usually  fearing 
insanity. 

As  soon  as  he  begins  to  notice  his  own  sensations  they  all  become 
intensified  and  many  normal  feelings  usually  unnoticed  are  thought  to 
be  symptoms.  Thus  pulsations  in  the  head,  feelings  of  distress  in  the 
head,  pressure  on  the  top  or  back  of  the  head,  particularly  at  the  occi- 
put, constriction  about  the  forehead,  tenderness  of  the  scalp  or  actual 
pain  in  the  head  are  complained  of.  These  are  classed  together  as 
cephalic  sensations,  and  are  very  common  symptoms.  The  headache 
of  neurasthenia  varies  in  intensity  during  the  day,  being  increased  by 
mental  exertion  and  relieved  by  food,  it  is  worse  in  the  morning,  often 
subsides  at  night,  and  never  keeps  the  patient  awake.  Rarely  vertigo 
is  felt  as  well  as  headache  or  if  there  is  no  actual  dizziness  there  is  a 
sense  of  movement  of  the  body  when  at  rest,  and  a  feeling  of  unsteadi- 
ness in  walking  giving  rise  to  much  discomfort.  Numbness  in  the 
face,  or  tongue,  or  extremities,  cold  or  hot  sensations  there  or  in  the 
back  ;  feelings  of  weakness  in  the  legs  and  hands  ;  inability  to  use  the 
eyes,  unwillingness  to  listen  to  music  or  sounds  of  any  kind,  defective 
appetite  and  disordered  taste,  all  indicate  an  imperfect  state  of  function 
in  the  cortical  centres  of  sensation.  Subjective  sensations,  such  as 
spots  before  the  eyes,  ringing  in  the  ears,  or  a  bad  taste  in  the  mouth 
are  often  complained  of.  In  the  majority  of  patients  an  inability  to 
read  is  noticed.  This  may  be  due  to  a  sensitiveness  of  the  optic  nerves, 
so  that  looking  at  anything  is  painful,  or  it  may  be  due  to  an  inability 
to  focus  the  eyes,  the  accommodative  apparatus  being  easily  fatigued. 
More  often  it  is  due  to  the  inability  to  concentrate  the  attention  or  to 
get  up  any  interest  in  subjects. 

The  cortical  centres  of  motion  also  suffer.  Patients  complain  of 
being  tired  all  the  time.  A  continued  muscular  effort  is  imjjossible. 
The  grip  is  poor  and  becomes  worse  as  efforts  are  repeated.  Tremor 
of  the  hands  and  face  and  eyelids  may  be  seen.  Walking  any  distance 
causes  undue  fatigue.     Talking  and  writing  are  irksome. 

The  sub-cortical  processes  of  association  are  equally  involved.  A 
face  no  longer  suggests  a  name.  Memories  which  were  formerly 
grouped   together   are    no    longer   associated.       Hence  any  thought 


842         NEUBASTEENIA    AND    THE    OCCUPATION   NEUROSES. 

becomes  slow  and  more  of  an  effort  than  usual,  and  mental  application 
involving  many  associative  processes  is  impossible.  The  defects  of 
memory,  which  are  noticed  early,  give  rise  to  much  alarm. 

The  emotional  element  in  mental  activity  is  also  affected.  Nothing 
gives  rise  to  a  sense  of  pleasure.  All  processes  become  painful,  and  a 
sense  of  discomfort  and  distress  attends  all  thousrht.  A  marked 
depression  of  spirits  is  complained  of,  and  the  patient  fears  melan- 
cholia. His  depression  differs  from  that  of  melancholia,  however,  as 
he  never  blames  liiraself  for  imaginary  ill-doing.  The  true  melan- 
cholic has  a  conviction  that  he  himself  is  to  blame  for  his  misery,  that 
it  is  a  just  punishment  for  some  supposed  sin  of  omission  or  commis- 
sion, and  that  there  is  no  hope.  The  neurasthenic  ascribes  his  distress 
to  every  possible  cause  except  to  his  own  errors  of  life  or  work. 

Morbid  fears,  termed  j^hobias,  are  very  common  in  neurasthenia. 
This  may  be  an  indefinite  sense  of  apprehension,  or  it  may  be  a  fear  of 
certain  things.  Thus  one  patient  may  fear  being  in  a  room  or  hall  or 
theatre  or  church,  or  any  closed  place,  and  will  rush  out  into  the  open 
air  for  relief;  this  has  been  termed  claustro-phobia.  Another  may 
fear  open  spaces,  and  will  go  long  distances  through  narrow  streets 
rather  than  cross  a  square ;  this  has  been  termed  agoraphobia.  Many 
fear  to  make  any  effort,  and  will  not  attempt  any  in  consequence. 
Many  fear  some  form  of  disease,  and  usually  make  themselves  familiar 
with  its  symptoms,  and  then  notice  their  own  sensations  and  misinterpret 
them ;  thus  persuading  themselves  that  they  have  the  disease  in  ques- 
tion. Physicians  often  fear  locomotor  ataxia  or  paresis  when  neuras- 
thenic. The  mistakes  of  the  laity  are  easier  to  correct.  Some  have 
apprehensions  about  their  family  or  friends,  rather  than  about  them- 
selves, are  especially  afraid  of  lightning,  of  journeys,  of  fire,  etc.  In 
a  study  of  such  morbid  fears  Stanley  Hall  has  described  over  two 
hundred  varieties  which  have  been  noticed.  These  morbid  fears  are 
very  common  symptoms  of  the  disease,  and  when  they  overcome  a 
patient  suddenly  give  rise  to  attacks  of  anxiety  which  are  very  dis- 
tressing and  may  produce  physical  effects,  such  as  palpitation,  pallor, 
faintness,  and  exhaustion.  Such  fears  are  particularly  common  in 
neurasthenia  due  to  alcoholism. 

The  emotional  control  is  very  defective  in  neurasthenia.  Patients 
are  easily  moved  to  tears,  cry  as  they  relate  their  symptoms  and  may 
act  in  a  way  suggestive  of  hysteria.  In  many  cases  there  is  a  mingling 
of  the  symptoms  of  both  diseases. 

Another  mental  symptom  is  some  morbid  impulse.  The  patient 
suddenly  becomes  possessed  with  an  idea  which  he  cannot  resist.  He 
may  have  an  impulse  to  count  objects,  or  to  repeat  certain  words.  He 
cannot  sit  down,  and  has  to  walk  about ;  he  cannot  listen,  but  must 
talk  constantly  ;  he  is  beset  by  doubts,  and  questions  everything ;  is 
perplexed  as  to  whether  he  has  done  right  or  wrong,  whether  he  has 
told  the  truth,  whether  he  has  remembered  important  things,  whether 
he  is  understood  by  his  family  or  his  doctor.  Thus  his  mind,  being 
occupied  by  these  fears  and  impulses,  and  by  these  exaggerated  sensa- 


SYMPTOMS.  843 

tions,  is  inpapable  of  working  along  its  accustomed  lines  and  is  beyond 
his  control. 

Some  patients  influenced  by  both  morbid  fears  and  morbid  impulses 
will  suddenly  leave  home  and  wander  away  or  go  on  journeys  and  only 
after  some  days  realize  where  they  are  and  the  need  of  informing  their 
family  of  their  whereabouts.  They  usually  return  in  a  bewildered 
state. 

The  view  of  Striimpell,  that  neurasthenia  is  largely  a  congeries  of 
mental  symptoms,  with  a  disturbed  process  of  thought  (Gestortes  Vor- 
stellungs  leben),  and  that  such  physical  symptoms  as  are  present  are 
largely  due  to  auto-suggestion  and  to  the  effect  of  the  mind  upon  the 
body,  is  in  many  cases  quite  correct.  The  psychasthenia  is  often  more 
prominent  than  the  neurasthenia.  This  is  particularly  true  of  the  type 
which  develops  after  accidents,  the  so-called  traumatic  neurosis. 

These  mental  and  cerebral  symptoms  develop  slowly  or  rapidly, 
come  to  their  height,  remain  for  some  time,  subside  gradually  until 
finally  they  all  disappear  and  the  patient  recovers.  Or  they  attain  a 
certain  height  and  remain  for  several  years,  being  in  abeyance  for 
weeks  at  a  time  when  life  is  uneventful  and  easy,  but  recurring  when- 
ever an  effort  to  work  is  made.  Occasionally  they  last  for  the  remainder 
of  one's  life.  They  rarely  go  on  to  insanity  although  occasionally  a 
state  of  neurasthenia  precedes  general  paresis ;  and  they  never  cause  a 
fatal  termination. 

The  spinal  symptoms  develop  in  some  cases  to  the  exclusion  of  all 
others,  but  in  many  cases  of  cerebral  type  there  are  a  few  spinal 
symptoms  as  well. 

Pain  in  the  nape  of  the  neck  and  down  the  spine  is  the  most  com- 
mon symptom.  This  is  constant  and  very  annoying.  It  may  become 
so  intense  as  to  keep  the  patient  in  bed  ;  he  is  afraid  to  move,  and 
shows  signs  of  pain  on  either  active  or  passive  motion.  He  is  tender 
along  the  entire  spine  to  pressure,  and  a  light  touch  may  give  even 
more  distress  than  deep  pressure.  The  pain  radiates  from  the  back 
into  the  limbs,  and  about  the  body.  Sometimes  intense  pain  and  ten- 
derness are  felt  in  the  tip  of  the  sacrum,  the  so-called  coccygodynia 
being  very  common  in  neurasthenia.  Such  cases  should  not  be  sub- 
jected to  operation  as  the  pain  is  rather  a  symptom  of  a  nervous  state 
than  an  indication  of  organic  disease.  Usually  there  is  hypersensitive- 
ness  to  heat  and  to  cold,  especially  to  cold.  The  patients  complain 
of  undue  sensitiveness  to  any  impression,  the  light  touch  of  the  bed- 
clothes being  sometimes  annoying,  and  any  jar  of  the  bed  distressing. 
This  sensitiveness  is  greatest  along  the  costal  cartilages. 

There  also  seems  to  be  some  feebleness  in  motion.  The  body  is 
moved  slowly  and  with  difficulty.  The  jar  of  standing  or  stepping  is 
avoided.  The  legs  give  way  under  the  body,  though  in  bed  resistance 
to  their  movement  may  be  good.  Patients  often  say  and  believe  that 
they  cannot  stand  or  walk,  yet  under  some  sudden  emotional  excite- 
ment it  is  usually  found  that  they  can  do  so.  These  are  tlie  cases  in 
which   "miracles  of  healing"  are  observed.     The    muscles    become 


844        NEURASTHENIA    AND    THE   OCCUPATION   NEVBOSES. 

flabby  and  weak  from  inactivity,  but  they  never  lose  their  reaction  to 
faradism,  a  most  important  point  in  a  differentiation  from  myelitis. 

The  reflexes  are  usually  exaggerated,  knee-jerks  are  high,  and  a 
spurious  ankle  clonus  may  be  elicited,  a  sort  of  semi-voluntary  pressure 
downward  once  or  twice  when  the  foot  is  suddenly  pushed  backward ; 
but  a  true  Babinski  reflex  is  never  found,  a  most  important  fact  in  a 
differentiation  from  lateral  sclerosis.  But  often  if  the  knee  jerks  are 
tested  again  and  again  in  quick  succession  the  response  becomes  weaker 
and  weaker,  showing  the  easy  fatigue  of  the  muscles.  An  unusual 
mobility  of  the  iris  is  often  seen,  the  pupil  first  contracting  and  then 
dilating  or  oscillating  under  a  strong  light.  The  sexual  reflexes  are 
also  increased,  sexual  desire  may  be  excessive,  erections  and  emissions 
too  frequent,  but  the  function  imperfect  and  weak.  The  bladder  may 
be  irritable,  so  that  urination  is  too  frequent.  It  is  rarely  painful, 
and  retention  is  rare.  There  is  usually  chronic  constipation  from  an 
atonic  state  of  the  colon.  This  group  of  symptoms  was  formerly  called 
spinal  irritation.  They  are  not  exclusively  spinal,  as  the  mental  attitude 
is  never  normal,  and  the  attention  is  always  concentrated  on  all  the 
bodily  sensations  and  functions. 

This  type  of  neurasthenia  is  very  common  after  accidents  which 
involve  a  blow  on  the  back,  or  a  jar  to  the  entire  body  or  a  severe 
fright.  Traumatic  neurasthenia  is  more  common  in  cases  where  no 
physical  injury  can  be  detected.  The  symptoms  have  been  ascribed 
to  capillary  hemorrhages  in  the  cord,  to  concussion  of  the  cord,  to  con- 
cussion or  bruising  of  the  spinal  ganglia,  to  strains  of  the  spinal  liga- 
ments, to  bruising  of  the  intervertebral  cartilages  ;  though  it  is  ques- 
tionable whether  any  of  these  conditions  have  been  shown  to  exist. 
They  are  really  manifestations  of  an  exhausted  state  and  of  an  imper- 
fect function  in  the  spinal  mechanisms  and  in  the  cerebral  centres 
which  are  connected  with  and  control  them.  This  has  been  proven 
by  the  fact  that  hypnotic  suggestion  often  produces  an  immediate 
cure ;  and  that  firm  persuasion  of  relief  by  some  inert  agent  often 
causes  these  symptoms  to  disappear.  Such  symptoms  are  often  ob- 
served in  anaemic  young  women,  who  have  had  menstrual  disorders 
and  who  gradually  sink  into  a  life  of  invalidism,  being  bed-ridden  for 
years,  the  despair  of  their  relatives  and  the  bane  of  sanitariums. 

After  a  number  of  months,  or  even  two  years  of  intense  suffering 
from  these  symptoms,  the  patients  usually  recover.  A  few  remain  in 
a  condition  of  chronic  invalidism,  confined  to  bed,  and  a  burden  to 
themselves  and  their  friends. 

The  vasomotor  and  syi apathetic  symptoms  of  neurasthenia  are  almost 
always  present,  and  in  many  cases  are  most  intense. 

A  certain  tone  of  the  blood  vessels  is  present  in  health,  and  is  essen- 
tial to  normal  activity.  The  flow  of  blood  to  an  organ  varies  with  the 
function  of  that  organ,  being  intense  when  the  function  is  being  called 
Into  play,  and  slight  when  the  organ  is  at  rest.  Ordinarily  one  does 
not  notice  this  alternation  of  flushing  and  pallor,  or  the  corresponding 
lymphatic  distension.     But  in  some  persons  the  vasomotor  stability  is 


SYMPTOMS.  845 

easily  affected  by  various  influences.  Thus  a  change  in  barometric 
pressure,  in  the  electrical  tension,  or  in  the  temperature  of  the  air  is 
enough  to  make  the  rings  on  one's  finger  tight  or  loose ;  to  make  the 
tone  of  the  muscles  firm  or  relaxed  ;  to  make  the  color  of  the  face  pale 
or  flushed,  to  make  the  pulsations  of  the  heart  fast  or  slow.  Corre- 
sponding changes  in  mental  activity  and  in  temper  occur,  as  has  been 
shown  by  Dexter,  who  found  that  in  school  children  disorder  marks 
from  bad  behavior  coincide  with  windy  days,  low  barometer  and  hot, 
moist  air.^  These  variations  appear  to  be  exaggerated  in  degree  in 
neurasthenic  persons,  and  when  neurasthenia  develops  they  become  dis- 
tressing symptoms,  particularly  as  the  attention  is  frequently  directed 
to  them.  Sensations  of  a  rush  of  blood  to  the  head,  of  flushing  of  the 
face  or  of  various  parts  of  tlie  body,  sensations  of  cold,  accompanied 
by  pallor  of  the  skin  are  often  felt.  If  a  sharp  point  is  drawn  across 
the  skin  anywhere,  but  particularly  on  the  trunk,  a  white  line  appears, 
and  then  a  broad  red  band  on  each  side  of  it,  which  may  remain  for 
ten  minutes  or  longer.  A  consciousness  of  the  pulsations  of  the  heart 
or  of  the  larger  arteries  is  often  present  and  is  annoying,  especially  at 
night  when  it  may  prevent  sleep.  The  heart  may  be  constantly  heard 
to  beat  from  the  impulses  of  the  distended  vessels  in  the  ear  setting 
up  vibrations  in  the  ear  drum.  A  pain  like  that  of  angina  pectoris 
in  the  chest,  and  especially  down  the  back  of  the  arms  and  elbows,  is 
sometimes  felt.  In  some  cases  pressure  on  a  painful  point  such  as  the 
ribs  may  increase  the  heart  beat  10  or  20  a  minute.  This  is  Rumpf's 
symptom.  An  actual  weakness  of  the  heart  is  often  found,  and  a 
lowering  of  arterial  tension,  as  shown  by  the  sphygmomanometer.  A 
feeling  of  unusual  swelling  of  a  limb  may  be  complained  of,  and  a 
slight  oedema  may  often  be  observed,  as  shown  by  the  rings,  collar  and 
wrist  bands  being  tight.  Many  of  the  cerebral  sensations  already 
mentioned  are  probably  due  to  corresponding  vasomotor  states  in  the 
brain,  as  shown  by  their  sudden  onset  and  disappearance. 

In  addition  to  the  rapid  alternations  of  vascular  tone,  there  are  often 
long  continued  states  of  venous  or  arterial  congestion  of  the  same  ori- 
gin. These  are  the  conditions  which  led  early  observers  to  describe 
neurasthenia  as  cerebral  and  spinal  congestion.^  There  is  a  flushed 
appearance  of  the  face,  a  blueness  of  the  lips  and  tongue,  a  distension 
of  the  retinal  arteries,  a  blueness  and  coldness  with  sweating  of  the 
extremities,  indicative  of  venous  congestion.  In  women  menorrhagia 
is  very  common,  and  all  the  symptoms  of  a  vasomotor  type  accom- 
panying normal  menstruation  are  markedly  exaggerated.  Many  of 
these  cases  find  their  explanation  in  defects  of  secretion  in  the  ductless 
glands,  especially  in  a  diminution  in  the  supply  of  thyroid  secretion. 

The  variations  in  secretion,  either  of  perspiration  or  of  urine,  are 
probably  also  of  vasomotor  origin.  A  patient  may  be  constantly 
bathed  in  a  cold  perspiration,  or  sudden  secretion  of  abnormal  quantity 
ma,y  occur.     I  have  seen  one  woman  from  whose  hands  the  sweat 

'Weather  Influences,  E.  G.    Dexter,  The  Macmillan  Co.,  1904, 
2  Dr.  Hammond,  Nervous  Diseases, 


846         NEUEASTHENIA    AND    THE    OCCUPATION    NEUBOSES. 

dropped  for  periods  of  half  an  hour  at  a  time.  The  flow  of  saliva 
may  be  increased,  leading  to  frequent  acts  of  swallowing,  and  this  in 
time  may  lead  to  belching  and  to  borborygmi,  which  are  incessant. 
The  flow  of  urine  is  often  increased,  patients  finding  it  necessary  to 
empty  the  bladder  every  hour.  Jn  other  cases  the  urine  is  much 
diminished  in  amount. 

Much  attention  has  been  paid  to  the  examination  of  the  urine,  by 
those  wlio  believe  that  neurasthenia  is  due  to  chemical  changes  in  the 
body.  In  some  cases  oxaluria  is  present,  crystals  of  oxalate  of  lime 
being  found.  In  many  cases  an  excess  of  uric  acid  is  a  constant  con- 
dition, and  the  normal  ratio  of  uric  acid  to  urea  (1  to  50)  is  changed, 
the  ratio  being  1  to  40,  or  even  1  to  30.  In  many  cases  the  excretion 
of  phosphates  is  increased,  rendering  the  urine  cloudy  and  even  milky 
in  appearance.  In  almost  all  cases  indican  is  excreted  in  excess.  It 
is  rare  for  albumin  to  be  found,  though  occasionally  it  appears  for  a 
fevv  days  without  casts  or  epithelia.  Temporary  glycosuria  is  a  symp- 
tom which  is  sometimes  found. 

Any  one  of  these  conditions  is  an  evidence  of  some  defective  process 
of  metabolism,  and  is  usually  associated  with  some  form  of  gastric  or 
intestinal  indigestion.  It  is,  however,  a  mistake  to  ascribe  the  disease 
entirely  to  this  cause,  and  those  who  consider  neurasthenia  as  due  to 
lithsemia  are  as  much  mistaken  as  those  who  formerly  ascribed  it  to 
congestion  of  the  brain. 

The  general  health  suifers  greatly  in  neurasthenia,  and  there  are 
few  diseases  which  cause  such  continued  discomfort.  Both  mind  and 
body  are  enfeebled.  A  loss  of  weight  is  common,  and  patients  feel 
the  weakness  which  attends  it.  Sleep  is  usually  poor,  it  is  interrupted, 
disturbed  by  distressing  dreams,  does  not  give  a  sense  of  rest,  and 
sometimes  insomnia  becomes  distressing.  The  patient  may  be  restless 
at  night,  fearful  of  lying  awake,  and  may  have  to  resort  to  hypnotics 
to  get  sleep.  More  often  he  is  drowsy  after  a  meal,  gets  to  sleep  at 
once  on  going  to  bed,  but  awakes  at  four  or  five  in  the  morning 
depressed  and  nervous,  and  does  not  go  to  sleep  again.  The  insomnia 
of  neurasthenia  is  an  early  symptom  and  one  of  the  most  distressing 
and  difficult  to  treat.  It  is  better  to  resort  to  suggestive  treatment 
for  this  symptom  than  to  give  hypnotics,  for  these  patients  become 
dependent  upon  any  means  which  has  once  given  relief.  For  this 
reason  massage,  faradism,  salt  rubs,  warm  baths,  physical  exercises, 
respiratory  exercises,  inhalations  of  oxygen  or  of  aromatic  alcoholic 
solutions,  various  foods,  and  hop  pillows  are  to  be  tried. 

As  a  rule  a  neurasthenic  feels  worse  in  the  morning,  complains  that 
his  night's  rest  has  done  him  no  good,  and  has  no  energy  for  the  duties 
of  the  day.  In  some  cases,  however,  the  symptoms  subside  as  the  day 
goes  on,  and  by  evening  the  patient  feels  quite  well  and  happy,  and 
goes  to  sleep  easily  only  to  wake  again  at  an  early  hour,  depressed 
and  listless.  This  type  of  insomnia  is  usually  attended  by  marked 
intestinal  indigestion,  by  indican  in  the  urine,  and  by  chronic  constipa- 
tion ;  and  it  is  best  treated  by  intestinal  antiseptics,  rather  than  by 


SPECIAL    TYPES    OF    NEURASTHENIA.  847 

hypnotics.  The  digestive  disturbances  usually  last  for  a  long  time, 
often  remaining  when  the  other  symptoms  subside,  but  gradually  they 
become  less  marked,  and  rarely  in  my  experience  lead  to  any  gastric 
or  intestinal  inflammation.  Hemorrhoids  which  may  develop,  however, 
are  permanent,  and  often  require  subsequent  operation.  Blood  tests 
occasionally  show  anaemia,  but  not  uniformly,  as  the  blood  is  often 
normal  when  patients  appear  pale. 

In  many  neurasthenics  a  marked  tremor  of  the  hands  is  present, 
especially  when  the  fingers  are  extended.  A  tremor  of  the  eyelids  is 
seen  when  the  patient  is  asked  to  shut  the  eyes. 

Special  Types  of  Neurasthenia.  Gastro-intestinal  Neurasthenia. 
—  Disturbances  of  digestion  are  very  common  in  neurasthenics.  A 
loss  of  appetite,  or  a  craving  for  special  articles  of  diet  is  frequent. 
Then  it  is  found  that  many  ordinary  forms  of  food  produce  discomfort, 
flatulence,  acidity  of  the  stomach,  heartburn,  and  even  pain.  Some 
patients  feel  much  better  after  a  meal,  but  in  the  course  of  an  hour 
a  sense  of  distension  and  discomfort  in  the  stomach  appears,  and 
they  raise  sour  fluid  and  gas.  This  is  followed  by  oppression  in 
breathing,  flushes,  palpitation,  and  finally  sleepiness.  Such  symp- 
toms indicate  an  atony  of  the  stomach  wall.  Some  patients  crave 
food  frequently.  Examination  of  the  stomach  contents  often  shows 
a  lack  of  HCl.  In  some  cases  progressive  emaciation  affords  proof 
that  food  is  not  assimilated.  One  patient,  a  physician,  has  an 
absolute  suspension  of  all  stomach  digestion  with  vomiting  of  undi- 
gested food  for  two  or  three  days  at  a  time,  when  under  the  anxiety 
of  a  serious  case.  Intestinal  distress  is  also  common,  and  constipation 
is  the  rule.  The  abdomen  is  sometimes  unduly  distended  with  gas, 
and  sometimes  an  atonic  state  both  of  the  intestines  and  of  the 
abdominal  walls  leads  to  a  state  of  enteroptosis,  which  requires  treat- 
ment. Various  rigid  forms  of  diet  are  often  followed  by  neurasthenics, 
under  the  mistaken  idea  that  they  are  suff*ering  from  dyspepsia,  but 
rarely  with  good  efiect. 

The  most  serious  symptom  of  a  digestive  kind  is  a  prolonged  diar- 
rhoea, occurring  chiefly  early  in  the  morning  and  causing  great  weak- 
ness during  the  day.  This  is  accompanied  by  pain,  by  expulsion  of 
gas,  and  is  watery  and  profuse.  In  some  cases  diarrhoea  occurs  only 
under  nervous  strain  or  excitement,  subsiding  immediately  when  this 
is  over.  Thus  one  college  professor,  when  neurasthenic,  has  profuse 
diarrhoea  for  several  hours  before  his  lecture,  but  only  on  his  lecture 
days. 

Sometimes  the  diarrhoea  is  attended  by  the  excretion  of  long,  ropy 
or  stringy  masses  of  mucus,  supposed  to  be  casts  of  the  intestine. 
They  are  white  and  firm,  and  consist  of  albuminous  substances,  with 
epithelia  of  the  intestines  upon  them.  They  are  only  occasionally 
found  in  the  stools,  after  specially  painful  movements,  and  are  attended 
by  an  intense  feeling  of  exhaustion.  They  are  often  preceded  by 
severe  colic.  This  condition  has  been  described  as  mucous  colitis. 
It  is  so  uniformly  associated  with  neurasthenia,  and  its  degree  corre- 


848         NEUBASTEENIA    AND    THE    OCCUPATION   NEUBOSES. 

sponds  so  closely  with  that  of  that  disease,  that  it  is  now  regarded  as  a 
symptom. 

Neurasthenia  from  Errors  of  Secretion  of  the  Ductless  Glands.  — 
There  are  certain  types  of  neurasthenia  that  appear  to  be  traceable 
directly  to  an  auto-intoxication  of  the  system  due  to  imperfect  action 
of  the  ductless  glands. 

(a)  The  thyroid  gland  when  secreting  in  excess  gives  rise  to  symp- 
toms resembling  those  of  Basedow's  disease,  but  in  mild  cases  where 
the  secretion  is  not  sufficient  to  cause  a  very  rapid  pulse,  a  bulging  of 
the  eyes  or  a  distinct  swelling  of  the  neck,  all  the  symptoms  of  a  neu- 
rasthenic type  which  are  common  in  Basedow's  disease  may  be  present, 
and  the  underlying  cause  may  escape  the  notice  of  the  physician.  The 
symptoms  that  are  most  marked  in  this  type  of  neurasthenia  are  wake- 
fulness that  is  persistent  and  does  not  yield  to  ordinary  hypnotics,  a 
mental  irritability,  fretfulness,  restlessness  and  excitability  which  is  so 
intense  as  at  times  to  awaken  the  suspicion  that  the  individual  is  ill 
balanced  mentally,  and  very  marked  vasomotor  symptoms  consisting  of 
flushings  of  the  surface,  throbbing  sensations  of  the  head  and  all  over 
the  body,  undue  perspiration  occurring  suddenly,  so  that  the  patient 
will  break  out  into  a  profuse  sweat,  feelings  of  fever  without  any  rise 
of  temperature,  and  intense  congestive  headaches.  Sometimes  in  addi- 
tion to  these  symptoms  there  are  feelings  of  tremor  through  the  body  and 
a  great  desire  for  fresh  air  which  makes  any  confinement  in  a  closed 
room,  in  theatres  or  churches  impossible  for  the  individual.  This  combi- 
nation of  symptoms  is  often  termed  hysterical  rather  than  neurasthenic 
and  is  particularly  liable  to  occur  in  girls  at  the  menstrual  period  and 
in  women  at  the  menopause.  If  the  actual  origin  of  these  symptoms 
in  the  increased  secretion  of  thyroid  fluid  is  appreciated,  treatment 
directed  to  relieve  this  condition  will  soon  cause  a  relief  of  the  symp- 
toms of  neurasthenia  which  otherwise  will  resist  ordinary  forms  of 
treatment.  The  use  of  belladonna  or  hyoscine  which  tend  to  decrease 
the  secretion,  the  administration  of  rodagen  or  thyrodectin  continuously 
for  a  considerable  period,  or  the  hypodermic  use  of  the  serum  prepared 
by  Beebee  and  Rogers  for  the  treatment  of  exophthalmic  goitre  are  of 
great  advantage  in  the  treatment  of  this  condition. 

(6)  Another  type  of  neurasthenia  due  rather  to  a  lack  of  secretion 
of  the  thyroid  gland  than  to  any  excess  in  its  activity  is  more  common 
than  the  previous  type.  The  extreme  of  this  condition  is  seen  in 
myxoedema,  but  there  are  a  good  many  cases  which  do  not  go  on  to 
development  of  true  myxoedema  and  yet  present  symptoms  of  a  nervous 
kind  which  are  present  in  that  disease.  These  symptoms  are  an  extreme 
degree  of  lassitude,  and  unwillingness  to  attempt  any  physical  exertion 
or  to  take  any  exercise,  with  marked  fatigue  on  minor  eiforts  ;  a  mental 
sluggishness  and  dullness  foreign  to  the  ordinary  temperament  of  the 
patient,  manifesting  itself  in  a  lack  of  interest,  a  willingness  to  sit  for 
hours  unoccupied  and  a  marked  loss  of  memory  ;  the  patients  appear 
to  be  indifferent  to  their  own  condition  and  show  a  lack  of  interest  in 
things  that  ordinarily  would  excite  them.     They  are  at  times  somno- 


SPECIAL    TYPES    OF   NEUBASTHENIA.  849 

lent,  sleep  heavily  but  do  not  awake  refreshed.  They  often  suffer  from 
muscular  pains,  especially  about  the  nape  of  the  neck  and  in  the  head. 
They  do  not  perspire  freely  and  the  skin  is  apt  to  be  dry  and  cracked 
and  may  be  slightly  pigmented  ;  brown  spots  appear  on  the  hands  or 
extensor  surfaces  of  the  extremities.  The  hair  is  dry,  breaks  off  or 
comes  out  and  is  wholly  lacking  in  the  natural  oil  and  may  turn  gray. 
Even  the  eyebrows  may  tend  to  fall.  In  some  cases  the  skin  may 
become  slightly  thickened  as  in  true  myxoedema.  In  this  condition 
the  patient  is  very  liable  to  gain  in  weight  constantly  and  a  peculiar 
swelling  of  the  extremities  or  of  the  body  is  not  infrequently  observed, 
which  renders  shoes  and  gloves  too  tight,  rings  too  tight,  though  this  is 
not  attended  by  any  oedema  that  can  be  discovered  by  pressure.  These 
patients  suffer  extremely  from  cold.  Their  hands  and  feet  are  cold 
constantly.  They  have  creeping  sensations  down  the  back  and  are 
liable  to  have  what  are  termed  nervous  chills  without  any  rise  of  temp- 
erature. The  appetite  is  as  a  rule  poor  and  there  is  obstinate  constipa- 
tion. This  is  frequently  attended  by  signs  of  intestinal  fermentation 
and  by  the  presence  of  indican  in  the  urine.  When  a  person  with 
neurasthenia  is  found  to  suffer  from  this  combination  of  symptoms,  it 
is  probable  that  her  neurasthenia  is  really  due  to  an  imperfect  secretion 
of  thyroid  glandular  extract,  and  the  only  relief  that  can  be  secured  is 
by  the  administration  of  thyroid  extract  which  will  be  found  to  relieve, 
not  only  these  symptoms,  but  also  those  of  the  accompanying  neuras- 
thenia. The  administration  should  be  by  small  doses,  one  grain  or 
possibly  two  grains  once  or  twice  a  day  for  ten  days,  followed  by  an 
intermission  of  five  days  and  a  gradual  increase  of  the  thyroid  up  to 
five  grains  a  day  given  preferably  in  divided  doses  during  the  twenty- 
four  hours. 

(c)  Another  form  of  auto-intoxication  giving  rise  to  a  marked  type 
of  neurasthenia  occurs  at  the  menopause,  and  is  undoubtedly  due  to 
the  fact  of  the  cessation  of  the  internal  secretion  of  the  ovaries.  This 
is  proven  by  the  fact  that  a  similar  set  of  symptoms  may  be  observed 
after  removal  of  the  ovaries  in  a  person  who  has  not  reached  the 
menopause.  Some  authors  ascribe  this  set  of  neurasthenic  symptoms 
to  chemical  changes  going  on  in  the  liver  and  spleen  and  accompanied 
by  intestinal  toxaemia.  Others  ascribe  it  to  the  increased  activity  or 
abnormal  activity  of  the  thyroid  gland  which  accompanies  the  cessa- 
tion of  the  activity  of  the  ovary.  The  physiological  actions  of  the 
ovary  and  thyroid  glands  are  closely  related,  and  many  of  the  symp- 
toms occurring  at  the  menopause  of  a  nervous  kind  are  similar  to  those 
already  described  are  due  to  an  excess  of  thyroid  secretion.  It  is 
more  probable,  therefore,  that  the  neurasthenia  of  the  menopause  is 
due  to  a  lack  in  the  blood  of  the  internal  secretion  of  the  ovary.  A 
certain  amount  of  success  has  attended  a  treatment  of  this  type  of 
neurasthenia  by  the  administration  of  luteine  prepared  from  the  corpus 
luteum  of  the  pig.  In  some  cases  where  the  symptoms  resemble  Base- 
dow's disease  it  is  wise  to  add  rodagen  or  thyrodectin  to  the  use  of 
luteine.     In  other  cases  where  the  woman  tends  to  become  very  fat 

54 


850         NEUBASTHENIA    AND    TEE    OCCUPATION   NEUBOSES. 

and  sluggish  at  the  time  of  the   menopause,  benefit  is  to  be  derived 
from  the  use  of  thyroid  extract. 

Sexual  neurasthenia  is  a  type  sufficiently  distinct  and  common  to 
warrant  notice.  It  occurs  chiefly  in  boys  and  young  men,  though  girls 
sometimes  suffer.  It  develops  in  persons  who  have  indulged  in  self 
abuse,  or  in  great  sexual  excesses.  The  mind  being  continually  cen- 
tered upon  sexual  matters,  there  occurs  a  constant  state  of  unnatural 
sexual  activity  in  the  spinal  centres,  which  soon  results  in  a  condition 
of  abnormal  irritation  and  excessive  secretion.  Erections  occur  at 
slight  provocation,  emissions  are  frequent,  at  first  at  night  with  dreams, 
and  later  during  the  day,  even  caused  by  imagination  ;  and  soon  the 
excess  is  attended  by  a  feeling  of  weakness,  of  parsesthesia  in  the  organs, 
and  of  pain  in  the  back.  The  erection  becomes  imperfect  and  emission 
premature,  and  natural  sexual  intercourse,  if  attempted,  is  found  to  be 
impossible.  This  at  once  affects  the  mind,  causing  great  depression  of 
spirits  and  a  state  of  constant  introspection  and  distress.  This  dis- 
tress is  manifest  in  the  facial  expression,  which  is  one  of  shame  and 
dejection.  The  patient  will  not  look  anyone  in  the  eye,  and  is  reluctant 
to  admit  his  symptoms  or  to  confess  his  fault.  The  fear  of  being  im- 
potent may  entirely  suspend  erections  and  a  state  of  psychical  impo- 
tence results,  which  in  turn  may  be  followed  by  many  neurasthenic 
symptoms.  The  attention  being  centered  upon  the  body,  external 
matters  are  neglected,  and  hence  not  remembered,  and  these  patients 
complain  of  inability  to  work  and  failure  of  memory.  Many  of  the 
vasomotor  symptoms  of  neurasthenia  are  usually  associated  with  these 
mental  and  spinal  symptoms. 

There  is  usually  some  actual  physical  disarrangement  of  the  sexual 
apparatus  present  in  these  cases,  a  chronic  urethritis,  or  a  prostatic 
enlargement  or  seminal  vesiculitis  with  hyperemia  and  hypersesthesia 
of  the  deep  urethra.  All  of  these  conditions  must  be  looked  for  and 
treated  in  addition  to  the  attention  given  to  the  neurasthenic  manifes- 
tations. The  condition  is  always  recovered  from  if  sufficient  rest  of 
the  sexual  organs  is  given.  Some  local  treatments  such  as  cold  baths, 
the  passage  of  cold  sounds  or  urethral  injections  of  a  boric  acid  solution, 
or  nitrate  of  silver  solutions,  may  be  used  ;  but  the  mind  must  be  kept 
occupied  by  healthy  pursuits  and  a  strong  mental  persuasion  of  rapid 
recovery  by  treatment  must  be  constantly  exerted  in  order  to  secure  a 
cure.  This  is  the  secret  of  the  success  of  so  many  patent  remedies, 
electric  belts,  etc.,  which  are  so  widely  advertised. 

Alcoholic  Neurasthenia.  —  Alcoholic  neurasthenia  develops  in  persons 
who  drink  steadily  for  a  long  time,  taking  small  amounts  of  alcohol 
frequently,  but  who  do  not  appear  to  be  intoxicated.  Thus  the  cause 
of  the  condition  may  not  be  evident  and  may  be  concealed  intention- 
ally. It  presents  many  mental  and  cerebral  and  vasomotor  symptoms, 
and  tremor  is  usually  very  marked.  These  patients  are  always  the 
victims  of  morbid  fears.  They  often  show  progressive  emaciation  and 
I  have  known  women  to  be  reduced  to  80  pounds.  Digestive  disturb- 
ances are  very  common.    All  the  symptoms  are  temporarily  benefited  by 


SPECIAL    TYPES    OF   NEURASTHENIA.  851 

a  drink  of  whiskey,  but  when  it  is  cut  off  these  patients  sink  into  a  state 
of  extreme  exhaustion  witli  great  depression  of  spirits.  These  cases 
are  particularly  obstinate  and  the  prognosis  is  worse  than  in  any  other 
type. 

Nicotine  Neurasthenia.  —  In  some  persons  the  long-continued  or  the 
excessive  use  of  tobacco  sets  up  a  nervous  condition,  the  special  symp- 
toms of  which  are  a  sensation  of  general  weakness  with  rapid  pulse 
and  palpitation  of  the  heart  on  exertion,  diffuse  pains  in  the  chest,  back 
and  limbs,  slight  numbness  and  awkwardness  of  the  fingers,  fine  tremor 
of  the  fingers,  and  disturbed  sleep  with  fearful  dreams  and  occasionally 
persistent  insomnia.  A  few  cases  of  this  kind  have  had  intermittent 
claudication  or  astasia-abasia.^  In  many  cases  of  neurasthenia  the 
temporary  or  permanent  cessation  of  the  use  of  tobacco,  tea,  and  coffee 
proves  of  great  service.  I  have  seen  a  case  in  a  tea-taster  who  pre- 
sented identical  symptoms  to  those  caused  by  tobacco,  and  who  recov- 
ered when  he  ceased  to  take  tea. 

Syphilitic  neurasthenia.  —  Syphilitic  neurasthenia  has  no  special 
features  excepting  for  the  fact  that  all  the  symptoms  are  worse  toward 
evening  and  insomnia  is  very  marked.  Many  patients  with  syphilis 
worry  themselves  into  a  neurasthenic  state  over  the  fact  of  having  the 
disease.  They  should  be  given  a  thorough  antisyphilitic  treatment, 
preferably  at  Hot  Springs,  Ark.,  and  then  with  the  assurance  that  when 
this  element  is  eliminated  the  treatment  for  the  neurasthenic  state  may 
be  successful. 

Climatic  neurasthenia.  —  Climatic  neurasthenia  is  a  rather  severe 
type  of  the  disease  which  develops  in  those  who  go  into  and  reside  in 
a  tropical  country.  Americans  in  the  Philippines  and  Panama,  and 
Englishmen  in  India  or  Africa  are  particularly  liable  to  develop  this 
type.  It  is  the  combination  of  heat  and  moisture  which  seems  to  affect 
the  nervous  system  and  when  this  is  kept  up  night  and  day  it  produces 
serious  effects.  There  is  a  feeling  of  general  lassitude,  an  unwilling 
ness  to  make  any  effort,  mental  or  physical,  a  complete  exhaustion 
when  such  efforts  are  made  ;  and  this  is  soon  followed  by  headache,  by 
insomnia  and  by  imperfect  memory  and  by  great  mental  depression. 
Cases  related  by  Woodruff^  may  be  quoted.  "  One  young  vigorous 
staff  officer  who  seemed  always  cool  complained  to  me  one  day  that  on 
a  previous  afternoon  at  five  o'clock  he  found  that  he  could  not  remem- 
ber anything  he  read.  When  he  got  to  the  end  of  a  sentence  he  had 
forgotten  the  beginning.  He  was  completely  disabled  until  the  next 
morning.  A  young  surgeon  informed  me  that  before  he  was  half 
through  his  rounds  he  daily  discovered  that  he  could  not  remember 
the  patient's  answers,  and  he  always  had  to  lie  down  thirty  or  forty 
minutes  before  the  brain  would  work  perfectly  again."  Such  condi-' 
tions  of  exhaustion  induce  men  to  take  stimulants  and  to  depend  too 
much  upon  them,  for  they  give  temporary  relief.  But  no  stimulation 
in  the  long  run  is  of  use  and  other  more  serious  effects  follow.     A 

I  Frankl  Ilocliwart.     Deut.  med.  Woch.,  Dec.  14,  1911. 

>  Woodrufi.     The  Soldier  in  the  Tropics.     Phil.  Med.  Jour.,  April  7,  1900, 


852        NEUBASTEENIA    AND    THE    OCCUPATION   NEUEOSES. 

state  of  mental  irritability  develops,  an  intolerance  of  criticism  or  of 
interference,  and  a  lack  of  self  control  of  which  the  victims  are  them- 
selves ashamed.  The  physical  symptoms  are  those  of  general  weak- 
ness of  the  limbs,  inability  to  endure  any  exercise,  irritability  of  the 
bladder,  sexual  impotence,  and  many  vasomotor  disturbances ;  a 
rapid  heart,  dyspnoea,  a  cyanosis  of  the  extremities,  oedema,  undue 
sweating  and  in  women  menorrhagia  or  metrorrhagia.  Such  a  neuras- 
thenic state  predisposes  one  to  all  the  dangers  of  a  tropical  climate,  to 
malarial  fever,  to  typhoid  fever,  to  dysentery  and  to  sunstroke. 
This  neurasthenic  state  has  been  ascribed  ^  to  an  ansemic  state  of  the 
central  nervous  organs  on  account  of  the  constant  dilatation  of  the 
arterioles  in  the  skin  due  to  heat.  The  body  is  kept  cool  by  the 
radiation  of  heat  and  by  the  evaporation  of  perspiration  secured  by 
dilatation  of  the  arterioles  but  this  removes  just  so  much  blood  from 
the  brain  and  cord  and  diminishes  their  nutrition  and  functional 
capacity.  Another  theory  to  account  for  the  neurasthenia  of  the 
tropics  has  been  advanced  by  WoodruflP,  namely,  that  it  is  due  to  the 
action  of  the  actinic  rays  of  the  sun  which  destroy  protoplasmic  life. 
Pigment  in  the  skin  protects  man  from  these  rays,  hence  negroes,  and 
brunettes  are  less  liable  to  neurasthenia  of  the  tropics  than  are  blond 
whites.  The  actinic  rays  are  supposed  to  stimulate  the  nerves  and 
especially  the  vasomotor  nerves,  causing  a  dilatation  of  the  vessels  ; 
they  are  also  supposed  to  stimulate  the  blood-forming  organs  ;  and 
owing  to  the  increased  flow  of  new  blood  cells  to  the  tissues  cell 
growth  is  excited  and  new  tissue  is  formed.  It  is  the  overstimulation 
of  the  constant  action  of  the  actinic  rays  which  is  supposed  to  cause 
the  neurasthenia.  The  remedy  suggested  is  to  wear  black  under- 
clothing next  the  skin  and  white  linen  outside  and  avoid  the  sun. 
The  only  effective  remedy  in  this  condition  is  a  change  of  climate. 
If  patients  can  get  to  a  high  mountainous  region  where  the  nights  are 
cool,  they  gradually  improve.  Thus  the  mountains  of  the  Philippines 
or  Simla  in  India  are  resorted  to  by  travellers  and  residents  when 
overcome  by  climatic  neurasthenia.  The  other  remedies  for  neuras- 
thenia, especially  strychnine  and  tonics  and  cool  packs,  are  of  use  in 
this  condition. 

Neurasthenia  in  Childhood.  —  While  the  disease  may  develop  at 
any  period  of  life,  attention  has  recently  been  called  to  its  appear- 
ance in  childhood.  There  are  many  nervous  children,  who  when 
over-stimulated  to  work  in  school,  or  when  subjected  to  too  much 
excitement  in  social  life  become  neurasthenic.  They  complain  of  head- 
aches, insomnia  and  indigestion  ;  are  unable  to  apply  their  minds  to 
study  ;  are  fretful  and  irritable  and  hard  to  manage,  and  may  even 
become  depressed  and  anxious  about  their  own  condition.  Chvostek 
has  noticed  that  in  nervous  persons  percussion  over  the  branches  of 
the  facial  nerve  causes  twitching  of  the  cheek  and  lip  muscles,  and 
this  sign  is  usually  to  be  found  in  nervous  children.  Unless  they  are 
allowed  to  stop  school  work,  and  are  sent  to  the  country  for  rest  and 
1  Fales.     Amer,  Jour,  Med.  Sci.,  April,  1907. 


mAmosts.  853 

play  these  symptoms  may  last  some  time.  But  under  proper  care,  and 
hygienic  surroundings  they  recover  more  rapidly  than  adults. 

Course.  — The  course  of  the  disease  is  always  slow  and  it  lasts  a 
long  time,  no  definite  limit  being  possible.  In  many  cases  a  few  cere- 
bral, some  vasomotor,  and  very  slight  spinal  symptoms  develop,  and 
the  patient  is  obliged  to  give  up  work  and  to  lead  an  idle  life.  If  he 
is  properly  directed  and  treated  he  improves  in  a  few  weeks,  and  after 
three  or  four  months  is  able  to  go  back  to  work  under  restrictions.  In 
some  cases  the  cerebral  symptoms  are  attended  by  very  great  mental  de- 
pression, and  melancholia  may  be  feared.  But  true  insanity,  with  de- 
lusions of  guilt  and  impulses  to  suicide,  rarely  if  ever  develops  from 
neurasthenia.  Many  of  these  patients  drift  into  a  condition  of  hypo- 
chondriasis, their  only  thought  being  of  their  symptoms,  and  their 
complaints  being  endless.  This  state  often  persists  several  months  or 
even  years,  not,  however,  becoming  worse,  or  leading  either  to  death 
or  to  insanity,  as  the  patient  fears.  In  these  cases  relapses  are  frequent 
if  recovery  occurs.  In  some  cases  the  spinal  symptoms  are  most 
prominent,  and  in  these  the  patient  is  often  bedridden  for  months  or 
even  years,  and  usually  drifts  into  a  state  of  hysterical  self-concentra- 
tion without  depression  of  spirits.  In  the  traumatic  cases  both  spinal 
and  vasomotor  symptoms  are  fully  developed,  and  the  hysterical  con- 
dition becomes  well  marked  as  the  case  goes  on.  These  cases  last  for 
years,  sometimes  improving,  especially  if  claims  for  damages  are  suc- 
cessful, but  rarely  recovering  entirely  for  several  years,  and  sometimes 
not  at  all. 

The  cases  in  which  mucous  colitis  develops  are  also  very  liable  to 
relapses,  especially  under  mental  worry. 

Diagnosis.  —  The  diagnosis  of  neurasthenia,  like  that  of  hysteria,  is 
to  be  made  by  exclusion,  and  not  hastily.  It  is  to  be  remembered  that 
neurasthenia  is  often  a  condition  secondary  to  some  other  serious  dis- 
ease, or  may  be  the  precursor  of  some  organic  affection.  Hence  the 
physician  should  be  on  the  alert  to  detect  a  train  of  symptoms  indica- 
tive of  such  diseases  and  should  never  neglect  to  make  a  careful  phys- 
ical examination. 

Since  paresis  begins  with  the  symptoms  of  neurasthenia,  it  is  well 
to  remember  this  fact ;  and  to  test  the  condition  of  the  pupils,  which 
are  rigid  in  paresis ;  the  state  of  the  knee-jerks,  which  are  lost  or  ex- 
aggerated in  paresis  ;  the  mental  state  of  the  patient,  which  is  hopeful, 
excited,  optimistic  with  lack  of  judgment,  and  errors  of  memory  in 
paresis  ;  and  the  power  of  speech,  which  is  tremulous  and  indistinct  in 
paresis.     Tremors  of  the  face,  eyelids  and  hands  occur  in  both  cases. 

Neurasthenic  symptoms  are  not  uncommon  in  many  organic  diseases, 
especially  in  multiple  sclerosis,  and  in  the  arterial  conditions  which 
precede  apoplexy.  Headache,  vertigo,  insomnia,  and  inability  to  work 
continuously,  or  to  remember  are  common  premonitory  symptoms  of 
apoplexy.  If  the  arterial  tension  is  high,  tlie  heart  hypcrtrophied,  the 
urine  of  h)W  specific  gravity  and  contains  a  trace  of  albumen,  and  if 
the  retinal  arteries  are  seen  by  the  o])hthalmoscope  to  be  tortuous  or 


854         NEURASTHENIA    AND    THE    OCCUPATION    NEUROSES. 

to  show  sclerotic  patches  the  patient  is  not  suffering  from  primary 
neurasthenia.  These  diseases,  however,  gave  rise  to  definite  symptoms 
and  physical  signs  of  their  own,  which  will  be  found  in  addition  to  the 
neurasthenic  symptoms,  and  thus  will  lead  to  a  correct  diagnosis. 

Neurasthenia  and  hypochondriasis  are  closely  allied,  but  the  pre- 
ponderance of  mental  symptoms  with  absurd  apprehensions  regarding 
some  particular  organ  or  function,  and,  the  absence  of  any  spinal  or 
vasomotor  symptoms  in  the  latter  usually  enables  a  diagnosis  to  be 
reached.  A  true  mental  and  physical  exhaustion,  and  attacks  of  sud- 
den morbid  fears  are  present  in  the  neurasthenic  cases,  and  absent  in 
hypochondria. 

Melancholia  is  distinguished  from  neurasthenia  by  the  intensity  of 
the  mental  depression,  by  the  development  of  delusions  of  self-accusa- 
tion, by  the  loss  of  weight,  persistent  insomnia,  especially  the  early 
morning  waking,  and  by  the  facial  expression  which  is  often  anxious, 
but  never  sad  in  neurasthenia. 

It  is  well  to  distinguish  between  neurasthenia  and  psychasthenia, 
though  they  are  often  associated.  In  the  latter  the  mental  symptoms 
are  much  more  prominent  than  the  physical  and  are  only  removed  by 
psychical  treatment.  In  the  former  though  mental  symptoms  may 
occur  they  are  of  minor  consequence  and  the  treatment  which  succeeds 
is  that  which  is  addressed  to  the  physical  condition.  The  character- 
istics of  psychasthenia  are  discussed  in  the  next  chapter. 

Prognosis.  —  It  is  evident  from  what  has  been  stated  in  regard  to 
the  causation  and  to  the  course  of  neurasthenia  that  the  prognosis  is  not 
very  favorable.  Recoveries  occur  after  some  time,  as  a  rule,  but 
relapses  are  very  frequent,  and  can  only  be  avoided  by  care  in  the  regu- 
lation of  life  and  of  work.  A  person  who  has  once  had  an  attack  of 
neurasthenia  should  therefore  be  particularly  on  his  guard  against  over- 
work or  anxiety.  The  less  the  evident  predisposition  to  the  disease, 
the  better  is  the  prognosis.  The  shorter  the  period  through  which  the 
exciting  cause  has  acted,  the  better  the  prognosis.  The  more  rapid 
the  onset  of  symptoms,  the  better  the  prognosis.  A  preponderance  of 
cerebral  symptoms  implies  a  shorter  duration  than  a  number  of  vaso- 
motor and  spinal  symptoms.  Vasomotor  symptoms  usually  last  longer 
than  others,  and  are  more  difficult  to  treat.  The  prognosis  in  trau- 
matic cases  is  never  good,  absolute  recovery  being  rare,  even  after 
damages  have  been  obtained  by  litigation.  The  anxiety  of  legal  pro- 
ceedings always  intensifies  and  lengthens  the  symptoms.  The  prog- 
nosis in  spinal  cases  is  almost  always  bad.  In  sexual  neurasthenia 
the  prospect  of  recovery  is  very  good,  as  the  patients  are  young  and 
suggestive  treatment  is  effectual.  The  longer  the  period  of  time  given 
up  to  rest  and  treatment,  the  better  the  chances  of  avoiding  a  relapse. 

Treatment.  —  A  thorough  examination  of  a  person  suffering  from 
neurasthenia,  with  a  view  to  eliminating  all  possibilities  of  organic 
disease,  is  not  only  necessary  for  the  physician,  but  does  much  to  obtain 
the  confidence  of  the  patient;  and  in  neurasthenia  his  confidence  is  the 
first  necessity.  His  mental  state  of  apprehension  can  be  met  only  by 
rational  encouragement,  based  on  frequent  examinations ;  hence  per- 


TREATMENT.  855 

sonal  influejace  is  a  factor  in  the  treatment.     The  effect  of  mental  sug- 
gestion upon  neurasthenic  and  psychasthenic  states  has  always  been 
appreciated    by    physicians    and    many    remedies    which    have    been 
employed  in  its  treatment  —  particularly  electricity  —  have  been  suc- 
cessful chiefly  in  proportion  to  the  expectation  of  cure  which  they  ex- 
cited.    Every  physician   appreciates  the  fact  that   he  has  a  certain 
moral  influence  over  some  of  his  patients  and  not  over  others.     He 
knows  that  his  statements  with  some  carry  conviction  and  that  this 
conviction  is  an  important  aid  in  the  effects  he  tries  to  produce.     This 
element  of  therapeutic  success  has  of  late  been  called  to  the  attention 
of  the  public,  and  there  is  now  some  danger  that  it  will   be  overesti- 
mated and  possibly  employed  to  the  exclusion  of  other  means  which 
are  also  needed.     Positive  statements  to  patients  are  necessary  in  the 
management  of  neurasthenia,  and,  confidence  once  secured,  hope  and 
comfort  may  be  instilled  directly  both  by  firm  persuasion  and  honest 
admonition.     The  object  sought  is  to  put  the  patient's  mind  into  a 
different  attitude,  to   displace  fear  by  confidence,   to  displace  despair 
by  hope,  to  displace  timidity  by  self-reliance.    It  is  well  to  enter  fully 
into  the   trains  of  thought  which  have  led   to   the  patient's   state  of 
mind,   for  if  they  are  discovered   the   means   of  counteracting   them 
may  be  clear.     The  analysis  of  the  origin  of  the  symptoms  may  show 
how  to  persuade  the  patient  that  they  are  foolish  and  can  be  overcome. 
Such    patients  must    be  listened    to   patiently,  must  be  argued  with 
constantly,  must  have  daily  support  and  comfort  and  must  feel  that 
they  are  being  continually  thought  of  and  worked  over.     The  physician 
who  can  live  with  a  neurasthenic, or  travel  with  him  and  give  him 
constant  mental  stimulus  will  do  more  in  a  week  than  one  who  sees 
him  once  daily  for  a  month.     One  reason  for  the  success  of  all  those 
movements  which  involve  the  calling  in  of  a  supernatural  element  in. 
psychotherapy  is  the  conviction  conveyed  to  the  mind  of  the  patient 
that  he  has  with  him  a  supernatural  assistance  which  is  always  present, 
"an  ever  present  help  in  time  of  trouble,"  one  to  which,  by  prayer, 
he  can  at  any  time  appeal.      It  is  easier  to  attain  success  by  treating 
such  patients  in  a  company  where  the  hope  of  others  and  the  confidence 
of  the  crowd  stimulates  the  individual  as  no  mere  personal  admonition 
can  :  hence  the  cures  of  Lourdes  and  of  the  Emmanuel  movement  in 
Boston  which  supply  all  these  elements  of  success  together.      For  the 
sceptical  patient  it  is  well  to  resort  to  more  philosophical  argument ; 
to  interest  him  in  his  own   psychological  problems  and  to  allow  him 
to  read  and  study  such  subjects  as  will  give  him  information  as  to  his 
own  state,  thus  helping  him  to  overcome  foolish  doubts,  fears,  and  im- 
pulses by  showing  him  their  absurdity.      For  the  ignorant  and  unedu- 
cated an  analogous  means  is  to  resort  to  some  agency  to  them  mysterious 
and  wonderful  —  like  the  sparks  of  frictional  electricity  —  accompany- 
ing the  treatment  by  assertions   that  it  will  cure.     There  is  usually  a 
tremendous  unconscious  waste  of  nervous  energy  when  a  person  is  suf- 
fering  from   nervousness.     The  muscles   are   held  rigid,  the  body  is 
seldom  relaxed,  and  the  mind  is  in  an  equally  tiresome  state  of  tension. 


856         NEURASTHENIA    AND    THE    OCCUPATION    NEUBOSES. 

The  cultivation  of  the  faculty  of  relaxation  of  both  mind  and  body  is 
to  be  commended  to  ueurastlienics.  Nothing  should  be  done  hastily, 
or  under  stress.     A  quiet  mind  will  help  a  restless  body.^ 

All  the  causal  factors  in  a  neurasthenic  patient  should  be  studied, 
and  so  far  as  possible  eliminated  at  the  very  outset.  All  work  should 
be  stopped,  and  any  source  of  anxiety  removed  if  possible.  Then  the 
patient  should  have  an  entire  change  in  his  physical  and  mental  sur- 
roundings and  habit  of  life.  For  this  reason  travel  in  a  foreign 
country,  or  a  sojourn  in  a  well-regulated  sanitarium  where  the  food 
is  good,  are  very  advisable.  If  these  are  impossible  residence  in  the 
country  on  a  farm,  with  a  routine  life  and  some  exercise,  is  to  be 
commended.  In  any  event  the  mind  of  the  patient  should  be  urged 
into  new  channels,  to  the  exclusion  of  those  worries,  fears  and  thoucrhts 
of  work,  which  have  induced  his  breakdown.  The  mind  cannot 
be  idle,  and  rest  of  one  set  of  ideas  is  to  be  attained  only  by  sub- 
stitution of  a  new  set  with  some  interests.  Hence  every  kind  of 
novel  occupation,  farming,  carpentering,  photography,  sketching,  the 
study  of  new  languages  or  of  art  or  of  science,  or  some  mechanical 
occupation  ;  the  pursuit  of  fishing,  or  hunting  ;  new  games,  both  out- 
door and  indoor  ;  unaccustomed  kinds  of  reading  ;  all  these  are  valu- 
able, as  pastimes  during  the  period  of  necessary  rest.  The  mere  fact 
of  leaving  home  and  getting  away  from  work  and  worry  is  sufficient 
in  many  cases  to  arrest  many  symptoms ;  and  a  complete  change  of 
scene  is  all  that  is  needed  to  secure  a  cure  in  the  course  of  three  or 
four  months.      It  is  rarely  of  use  for  a  shorter  period. 

Physical  treatment.  —  In  more  severe  cases  it  is  necessary  to  enforce 
a  rigid  regime  of  life  ;  to  make  the  patient  remain  in  bed  for  breakfast, 
rise  at  ten  and  take  a  warm  bath  (98°)  for  ten  minutes  followed  by  a 
cool  (80°)  shower  or  sponge,  and  a  brisk  rub  with  a  coarse  Turkish 
towel,  spend  an  hour  twice  a  day  in  physical  exercise  followed  by 
complete  rest  lying  down  ;  spend  another  hour  twice  a  day  driving  or 
riding  on  horseback  ;  rest  an  hour  after  meals  ;  keep  himself  entertained 
by  being  read  to,  or  by  playing  games  when  in  the  house ;  to  advise 
massage,  and  to  enforce  an  early  hour  of  retiring.  The  alternation  of 
effort,  or  some  form  of  treatment  and  rest  is  to  be  kept  up  for  a  number 
of  weeks. 

In  extreme  cases  the  rest  cure  of  Weir  Mitchell  (see  page  843)  may 
be  carried  out,  but  it  is  rarely  as  successful  in  neurasthenia  as  in 
hysteria  and  I  much  prefer  a  regime  which  affords  a  variety  of  occu- 
pation to  the  rest  cure. 

In  all  cases  a  careful  regulation  of  the  diet  is  to  be  observed,  plenty 
of  good  nutritious  food  of  every  variety  being  given,  simply  cooked, 
and  all  stimulants  being  excluded.  Coffee  and  tea  may  be  used  in 
moderation,  if  they  do  not  excite  the  patient,  and  tobacco  need  not  be 
forbidden  unless  it  evidently  disagrees.  Rich  food  and  too  much  food 
at  one  rneal  are  to  be  avoided.  When  milk  agrees  it  may  be  taken 
freely.  Occasionally,  a  simple  restricted  diet  of  milk,  eggs,  chicken 
1  See  Power  Through  Eepose,  by  Anne  Payson  Call. 


TREATMENT.  857 

and  vegetables  with  fruit,  for  a  few  days,  will  correct  a  state  of  indi- 
gestion witn  loss  of  appetite,  and  then  a  full  diet  can  be  added.  The 
object  of  all  treatment  being  to  increase  nutrition,  limited  diet  is  bad. 
In  all  cases  an  ample  supply  of  water  is  to  be  taken,  to  promote  metab- 
olism and  to  aid  in  the  elimination  of  waste  products. 

Various  forms  of  water  treatment  are  of  much  service  in  the  treat- 
ment of  neurasthenia,  as  whatever  promotes  the  flow  of  blood  and 
lymph  through  the  tissues  increases  nutrition.  A  warm  (98°)  bath, 
or  a  hot  (104°)  bath,  followed  by  a  cool  shower  or  spray,  is  to  be 
given  daily.  If  it  promotes  sleep  it  may  be  given  at  night.  Usually 
it  is  better  in  the  morning.  A  spray  douche  thrown  with  some  force 
upon  the  back  and  played  over  the  entire  body,  beginning  at  a  tem- 
perature of  90°,  being  run  up  to  105°  and  then  down  to  70°,  the 
duration  being  about  4  minutes,  is  very  stimulating  to  the  circulation. 
A  hot  box,  in  which  the  patient  sits  with  his  head  out,  the  air  being 
heated  to  150°  for  10  or  15  minutes,  followed  by  a  spray  douche  for 
1  minute,  at  first  warm,  then  cooler,  is  excellent. 

A  pack,  in  which  the  patient  is  enveloped  in  a  sheet,  wet  with  water 
at  80°,  each  limb  being  wrapped  separately,  and  then  rolled  in  three 
thick  blankets,  and  in  which  he  lies  for  15  minutes,  being  sponged 
off  when  taken  out  with  water  at  75°  and  then  rubbed  briskly,  is 
stimulating. 

Sponging  the  entire  back  with  very  hot  and  then  with  cold  water 
for  two  or  three  minutes  is  excellent. 

Salt  rubs,  in  which  the  surface  is  rubbed  with  cloths  wet  in  brine 
or  with  hot  or  cold  water  in  which  sea  salt  is  dissolved,  the  skin  being 
dried  rapidly  afterward,  are  very  refreshing. 

Massage  is  also  of  the  greatest  service  in  increasing  nutrition  by 
emptying  the  lymphatics  thoroughly  and  promoting  the  venous  circu- 
lation. It  may  be  combined  with  either  passive  or  active  exercises, 
and  these,  if  carefully  graduated  and  increased  daily,  will  restore  the 
muscular  tone. 

Some  of  these  forms  of  external  treatment  should  be  given  twice  a 
day  to  every  neurasthenic,  each  to  be  followed  by  a  period  of  rest  and 
a  little  simple  food.  The  time  may  be  determined  by  the  patient, 
morning  and  late  afternoon  being  the  best,  as  water  treatment  at  night 
often  prevents  sleep.     Massage  sometimes  induces  sleep. 

Electrical  treatment,  either  faradism,  galvanism,  or  static  currents 
have  never  seemed  to  me  to  be  of  direct  benefit,  but  may  act  by  sug- 
gestion, and  this  is  often  necessary.  General  faradization  is  of  service. 
The  patient  places  the  feet  on  one  electrode,  while  the  other  is  passed 
over  various  parts  of  the  body,  a  mild  current  being  applied.  Gal- 
vanization of  the  spine  may  be  given,  one  electrode  being  placed  on 
the  neck,  and  the  other  over  the  sacrum,  or  passed  up  and  down  the 
back,  no  interruption  being  made.  The  current  should  be  mild  (20 
milliamperes  with  electrodes  4  inches  in  diameter)  and  may  be  con- 
tinued 10  minutes.  Static  electricity  is  convenient,  as  it  can  be  ap- 
plied over  the  clothing,  and  the  smaller  sparks  or  the  breeze  are  often 


858         NEURASTHENIA  AND  THE  OCCUPATION  NEUROSES. 

agreeable.  It  is  my  experience  that  these  forms  of  treatment  are  only 
successful  when  accompanied  by  constant  positive  statements  that  they 
will  do  good,  and  will  relieve  certain  symptoms,  and  only  when  given 
by  some  one  in  whom  the  patient  has  confidence.  Hence  I  regard  the 
effect  as  chiefly  due  to  suggestion. 

A  change  of  climate  is  often  of  service  in  chronic  cases.  Anaemic  and 
poorly  nourished  patients  do  well  at  the  seashore,  in  mild  climates,  and 
should  avoid  the  cold  in  winter.  Southern  California  and  Egypt  are  par- 
ticularly suited  to  such  cases.  Robust  or  fat  patients  do  better  in  high 
altitudes,  and  for  them  Colorado  or  the  Engadine  may  be  advised. 

In  spinal  and  sexual  neurasthenia  a  light  application  of  the  Paquelin 
cautery  to  the  spine,  or  a  series  of  minute  blisters,  or  the  application 
of  strips  of  plaster  to  the  spine,  plain  or  medicated  with  capsicum  or 
belladonna,  may  give  relief.  Ice  bags  are  sometimes  of  service.  In 
the  sexual  cases  the  passage  of  cold  sounds,  or  the  insertion  of  medi- 
cated tampons  is  useful,  acting  chiefly  by  suggestion.  In  these  cases 
mental  occupation  is  necessary,  and  it  may  be  well  to  keep  such 
patients  at  work,  and  to  urge  exercise  in  the  open  air.  They  should 
also  be  put  on  a  rigid  diet,  excluding  all  foods  which  excite  the  sexual 
appetite,  viz. :  oysters,  meats,  condiments  and  cheese.  They  should 
avoid  alcohol,  and  never  be  given  strychnine. 

Thyroid  extract  in  small  doses  (1  to  3  grains  twice  a  day)  has  a  very 
beneficial  effect  in  the  cases  which  present  vasomotor  disturbances,  or 
where  the  skin  is  dry,  the  hair  brittle,  the  surface  subject  to  irregular 
swellings,  the  muscles  and  joints  painful,  and  where  chilly  sensations 
are  frequent.  In  these  cases  it  is  found  that  the  headache,  lassitude 
and  feeling  of  exhaustion  gradually  disappear  under  the  use  of  the 
extract,  but  return  of  it  is  discontinued,  hence  the  treatment  has  to  be 
kept  up  for  considerable  periods  of  time. 

The  use  of  drugs  in  the  treatment  of  neurasthenia  is  to  be  avoided. 
Patients  rely  upon  and  expect  much  from  them,  and  are  disappointed. 
The  disease  is  a  slow  one  and  no  drug  can  be  safely  kept  up  for  a  long 
time.  The  market  is  full  of  nerve  tonics  which  are  largely  alcoholic 
and  contain  strychnine.  These  do  harm  in  many  cases.  If  anything 
could  be  found  which  would  supply  nutrition  to  exhausted  nerve  cells, 
it  would  do  good.  Possibly  the  glycero-phosphates  of  lime  and  soda, 
or  the  cacodylate  of  soda,  used  either  hypodermically  or  by  the  mouth 
may  have  this  effect.  They  may  be  tried.  The  chief  use  of  drugs  is 
to  combat  certain  symptoms.  In  some  cases  quinine,  iron,  or  arsenic 
are  of  service  in  helping  nutrition.  When  indigestion  is  present,  bit- 
ter, non-alcoholic  tonics,  mineral  acids,  and  intestinal  antiseptics  are 
often  of  use.  The  bowels  should  be  kept  open,  and  the  kidneys  active 
by  the  use  of  water.  If  laxatives  are  begun  they  often  have  to  be 
kept  up  ;  while  massage  of  the  abdomen,  especially  by  a  heavy  ball 
of  rubber  filled  with  lead  shot,  and  an  occasional  large  enema  will  do 
equally  well,  especially  where  diet  is  regulated.  Great  nervous  irrita- 
bility with  intense  morbid  fear  may  be  benefited  by  moderate  doses  of 
bromide  of  sodium,  but  this  is  not  to  be  used  constantly.     The  vaso- 


TSE  OGCVFATION  neuboses.  S59 

motor  sympj^oms  are  sometimes  helped  by  ergot  or  adrenalin  if  the 
pulse  tension  is  low,  or  by  nitroglycerine  in  ^^^  grain  dose,  or  by  3 
grain  dose  of  chloral  hydrate  if  the  pulse  tension  is  high.  They  are 
always  benefited  by  water  treatment.  Headache  is  best  treated  by 
massage  or  shampooing,  by  cold  cloths,  or  by  static  electricity.  The 
coal  tar  products  may  be  tried,  but  usually  fail  in  neurasthenic  pain. 
Minute  doses  of  codein  gL-  grain,  or  of  ignatia  j-^q-^  grain  repeated 
every  5  minutes  for  10  doses  often  help  pain  and  restlessness  and 
fears,  but  both  are  to  be  used  seldom,  and  with  caution.  Hyoscyamus 
in  tincture,  used  in  dose  of  5  drops  every  15  minutes  for  3  hours,  may 
also  quiet  fears.  Tincture  of  lupulin  is  sometimes  of  service.  Sleep- 
lessness is  usually  due  to  fear  of  lying  awake  in  neurasthenia.  A 
warm  bath  at  night,  massage  at  night ;  or  warm  food  on  going  to  bed 
may  secure  sleep.  Sometimes  a  little  whiskey  at  bedtime,  in  milk,  or 
a  glass  of  beer,  will  have  this  effect.  Placebos  of  all  sorts  are  justifi- 
able. Occasionally  trional  or  veronal  may  be  needed  ;  if  so,  let  one 
large  dose  (10  grains)  be  given  once,  and  a  smaller  one  on  following 
nights,  the  patient  being  unaware  of  the  change.  These  drugs  should 
be  given  with  some  food,  hot  milk,  or  chocolate.  Bromide  may  be 
used  as  an  alternative.  No  hypnotic  should  be  used  continuously, 
lest  a  habit  be  fostered. 


THE   OCCUPATION  NEUROSES.     PROFESSIONAL   CRAMPS. 

The  occupation  neuroses  are  forms  of  functional  disturbance  whif.h 
arise  as  a  result  of  the  frequent  repetition  of  some  fine  act  of  coor- 
dination. 

Their  chief  characteristics  are  that  the  symptoms  are  confined  to  a 
grouj)  of  muscles  accustomed  to  act  in  harmony  for  the  performance 
of  some  special  professional  action,  and  that  it  is  only  in  the  perform- 
ance of  this  action  that  they  appear,  the  same  muscles  acting  perfectly 
when  called  on  to  perform  other  acts.  Any  finely  coordinated  act 
requires  the  orderly  and  adjusted  contraction  of  a  series  of  muscles  in 
proper  sequence,  and  this  is  secured  by  impulses  sent  out  from  the 
educated  set  of  interrelated  nerve  centres.  If  the  act  is  repeated  too 
often,  fatigue  results.  If,  in  spite  of  the  fatigue,  the  act  is  continued, 
structural  changes  in  the  mechanism  underlying  it  may  be  caused. 
These  changes  may  be  in  the  cortical  centres  of  direction  ;  in  the  sub- 
cortical tracts  of  transmission,  i.  e.,  the  motor  tracts,  the  spinal  neurons, 
or  the  nerves ;  or  in  the  muscles.  One  or  all  give  out  under  the 
excessive  use. 

Symptoms  of  exhaustion  follow,  attended  by  a  sense  of  fatigue,  a 
feeling  that  continuance  of  the  act  is  impossible,  and  finally  some  revolt 
in  the  mechanism,  occurs  such  as  a  spasm  of  the  muscles  concerned,  or 
a  temporary  weakness  in  them,  and  these  may  be  attended  by  pain. 

In  some  cases  the  spasm  or  cramp  in  the  muscles  is  the  main  symp- 
tom. In  other  cases  it  is  a  functional  exhaustion  which  is  felt  giving 
rise  to  a  sense  of  paralysis.     In  other  cases  still  it  is  discomfort  or 


860 


NEURASTHENIA    AND    THE    OCCUPATION    NEUROSES. 


even  pain  in  the  muscles  which  hampers  or  prevents  the  act  and  is 
attended  by  tremor.  Cases  have  been  classified  as  spasmodic,  paralytic, 
or  tremulous,  according  to  the  predominant  symptom. 

The  location  of  the  symptoms  will  depend  entirely  upon  the  mus- 
cles involved  in  the  act ;  though  as  the  majority  of  the  occupation 
neuroses  occur  after  repeated  acts  of  fine  co5rdination  done  by  the 
hands,  it  is  usually  the  upper  extremity  or  extremities  in  which  the 
symptoms  appear. 

While  the  most  common  occupation  is  that  of  writing,  and  writer's 
cramp  or  writer's  palsy  is  the  most  common   of  these  neuroses,  yet 

Fig.  310. 


Fig.  311. 


Writer's  cramp,  showing  the  spasm  lifting  the  pen  from  the  paper  and  arresting 
ail  motions  of  the  finger  and  thumb. 

almost  any  occupation  which  implies  the  constant  repetition  of  a  move- 
ment may  lead  to  the  disease.  Figs.  310  and  311  show  two  positions 
spasmodically  assumed  on  an  attempt  to  write,  the  pen  being  with- 
drawn from  the  paper;  at  the  same  time  pain  was  always  felt  at 
the  base  of  the  index  finger.  Thus  telegraphers,  typewriters,  pian- 
ists, violinists,  drummers,  machinists,  cigar  makers,  pressers  and  ironers, 
milkers,  plasterers,  those  who  use  a  hammer  or  brush,  or  scissors,  those 
who  sew  or  knit,  are  all  liable  to  develop  a  cramp  or  palsy  when 
they  attempt  to  do  their  special  work.     The  cramp  may  be  felt  in  a 


TRE    OCCUPATION    NEUEOSES.  861 

single  muscje,  in  one  or  two  fingers,  or  in  the  entire  hand  or  in  the 
wrist,  or  even  in  the  entire  arm  and  shoulder.  The  muscles  affected 
become  suddenly  stiff,  and  voluntary  motion  is  suspended  for  a  few 
moments.  If  the  accustomed  posture  of  the  hand  is  changed,  or  some 
other  motion  made,  the  cramp  relaxes,  but  it  returns  when  the  attempt 
is  made  to  resume  the  work.  There  is  a  distressing  sense  of  strain  and 
tension  in  the  muscles,  which  sometimes  is  painful,  and  in  many  cases 
there  is  a  fine  tremor  of  the  fingers.  In  many  cases  there  is  tingling 
and  numbness  in  the  extremity  affected  and  occasionally  this  is  attended 
by  actual  pain.  In  other  cases,  instead  of  a  cramp  there  is  a  sudden 
giving  out  of  power.  The  muscles  relax  in  spite  of  effort,  the  pen  or 
implement  falls  out  of  the  hand.  Yet  there  is  no  true  paralysis,  for 
any  other  motion  that  is  attempted  can  be  done  well.  The  entire  disease 
consists  of  a  suspension  of  an  overworked  function. 

Occupation  neuroses  in  the  legs  have  been  observed  in  those  who 
work  pedals,  as  in  turners,  polishers,  sewing  machine  workers.  Ballet 
dancers  who  stand  on  the  toes  are  occasionally  affected.  The  facial 
muscles  are  occasionally  the  site  of  occupation  spasms.  Musicians  who 
blow  the  horn,  trumpet  or  bassoon,  artisans  who  use  blowpipes  in  mak- 
ing glass  or  in  chemical  works  have  had  spasms  of  the  orbicularis  oris. 
A  cramp  of  the  eyelids  has  been  observed  in  microscopists  and  in 
watchmakers  who  use  a  lens  constantly. 

It  is  my  experience  that  occupation  neuroses  develop  only  in  those 
persons  who  are  subject  to  the  other  causes  of  neurasthenia  and  are  in 
some  degree  neurasthenic.  The  occupation  neuroses  may  then  be  the 
chief  active  symptom  of  the  general  affection,  which  will  be  found, 
however,  to  exist  when  it  is  searched  for.  Oppenheim  agrees  with 
this  view.  As  a  confirmation  of  this  it  may  be  noticed  in  every  case 
that  the  individual  is  extremely  sensitive  about  his  trouble,  greatly 
worried  over  it  and  presents  many  signs  of  extreme  nervousness. 
Hence  in  the  etiology,  prognosis  and  treatment  of  the  disease,  the  facts 
presented  in  the  foregoing  chapter  on  neurasthenia  may  be  referred  to. 

Roskan  has  found  fibrous  nodules  within  the  muscle  affected  near 
its  tendinous  insertion  in  a  number  of  cases  and  has  removed  these 
nodules  surgically  with  good  effect.  This  finding  is,  however,  excep- 
tional, and  in  the  cases  in  which  it  occurs  all  motions  of  the  muscle 
involved  are  interfered  with,  not  merely  those  involved  in  the  profes- 
sional act. 

In  quite  a  number  of  cases  which  have  been  traced  to  an  actual 
neuritis  in  a  single  nerve  the  same  fact  has  been  evident,  viz.,  that  the 
cramp  or  pain  is  not  limited  to  the  professional  action.  In  these  cases 
tenderness  along  the  nerve  and  some  paraesthesia  or  anaesthesia  have 
been  found. 

In  other  cases  a  general  arterial  sclerosis  has  been  found,  and  the 
onset  of  the  symptoms  suddenly  and  their  relief  when  the  position  of 
the  limb  is  changed,  has  been  ascribed  to  vascular  spasm,  such  as 
occurs  in  intermittent  claudication. 

These  three  conditions  then,  though  capable  of  producing  symptoms 


862         NEURASTHENIA    AND    THE    OCCUPATION    NEUBOSES. 

of  professional  cramps,  should  not  be  classed  with  occupation  neuroses, 
but  should  be  differentiated  from  them. 

The  prognosis  is  good  for  recovery,  provided  a  sufficient  period  of 
rest  can  be  enforced.  This  rest  must,  however,  be  absolute  rest  of  the 
function  affected.  If  it  is  writer's  cramp,  the  pen  or  pencil  should  not 
be  touched  or  the  fingers  placed  in  the  writing  position  for  two  years. 
Sometimes  patients  try  to  learn  to  write  with  the  left  hand,  but,  if 
they  do,  it  is  not  unlikely  that  the  cramp  will  develop  in  that  hand 
also.  Hence  absolute  cessation  of  writing  is  to  be  advised.  If  the 
rest  is  not  of  sufficient  duration,  a  relapse  is  likely  to  occur. 

Treatment.  —  In  addition  to  absolute  cessation  of  the  employment 
which  has  led  to  the  neurosis,  all  the  measures  already  described  in 
the  treatment  of  neurasthenia  are  to  be  employed.  Massage  of  the 
hands  and  arms  is  particularly  useful,  with  Swedish  movements  of  re- 
sistance. In  the  early  stage  of  writer's  cramp,  it  is  sometimes  possible 
to  give  relief  by  suggesting  unusual  methods  of  holding  the  pen  or  by 
wearinar  on  the  hand  or  wrist  some  form  of  bracelet  which  will  hold 
it.  Sometimes  if  the  pen  is  wound  with  twine  or  is  passed  through  a 
cork,  it  can  be  held  in  the  palm  of  the  hand  and  all  pressure  of  the 
fingers  can  be  obviated.  These  devices,  however,  only  delay  the  prog- 
ress of  the  affection,  which  must  be  treated  in  the  end  with  absolute 
rest.     When  writing  is  inevitable,  a  typewriter  can  be  used. 


CHAPTER  LI. 

PSYCHASTHENIA. 

PsYCHASTHENiA  is  a  Condition  of  abnormal  action  of  the  mind 
manifesting  itself  in  errors  of  judgment,  in  the  dominance  of  certain 
ideas  which  are  known  as  obsessions,  in  abnormal  emotional  states  lead- 
ing to  unwarrantable  fears  and  states  of  anxiety,  in  abnormal  impulses 
to  actions  which  are  known  to  be  purposeless  or  foolish  and  in  states 
of  mental  perturbation,  of  pain  and  of  disorders  of  consciousness  which 
render  ordinary  occupations  impossible.  The  absence  of  hallucinations, 
illusions  or  fixed  delusions  and  the  absence  of  continued  depression  or 
excitement,  separate  this  class  of  mental  symptoms  from  those  of  insanity 
in  its  well-recognized  forms. 

Etiology.  —  Psychasthenia  as  a  rule  develops  in  individuals  who  by 
their  inheritance  have  been  predisposed  to  a  weakened  nervous  system, 
or  who  have  acquired  it  by  any  of  the  causes  which  have  been  mentioned 
in  connection  with  the  etiology  of  neurasthenia.  In  fact,  to  discuss 
the  causes  of  psychasthenia  would  be  to  repeat  what  has  been  stated  on 
pages  838  to  840. 

Psychasthenia  may  develop  as  a  complication  of  neurasthenia,  and 
there  are  very  few  neurasthenics  who  do  not  present  some  symptoms 
of  psychasthenia,  but  they  are  not  necessarily  associated.  In  the 
majority  of  cases  of  psychasthenia  there  does  not  appear  to  be  any  real 
weakening  of  the  mind,  as  the  term  implies.  There  is  however  a  lack 
of  balance  between  the  various  powers  of  the  mind,  and  a  lack  of  judg- 
ment and  of  self-control.  In  a  normal  person  morbid  thoughts,  morbid 
impulses  and  morbid  feelings,  if  they  occur,  are  looked  upon  in  their 
true  value  and  are  controlled.  All  authors  agree  that  an  inherited 
weakness  of  the  nervous  system,  a  congenital  condition  of  degeneracy, 
an  inherent  instability  and  enfeebled  power  of  resistance  to  strain  is  to 
be  found  in  almost  all  psychasthenic  persons. 

Women  are  more  frequently  aflPected  than  men. 

The  symptoms  usually  appear  at  the  period  of  sexual  development, 
may  disappear,  but  recur  under  any  anxiety.  The  disease  usually 
develops  in  those  who  have  been  exposed  to  some  great  emotional 
strain  or  shock,  and  in  those  who  have  been  unduly  worried  by  family 
or  business  anxiety.  Long-continued  worry  is  as  potent  as  sudden 
shock  to  produce  it,  especially  if  that  is  attended  by  a  mental  conflict 
iK'tween  moral  dicta  and  impulses  toward  conduct  known  to  be  wrong. 
Women  contemplating  divorce,  clergymen  wishing  to  change  their  call- 
ing because  of  religious  doubts,  business  men  adopting  dishonest 
methods,  persons  who  have  entered  on  a  course  of  conduct  where  con- 

863 


864  PSYCHASTHENIA. 

cealment,  deception  or  deceit  are  constant,  are  subjected  to  such  a 
mental  conflict  as  to  produce  anxiety,  and  to  lead  to  psychasthenia. 

A  similar  anxiety  may  be  aroused  by  self-reproach  as  to  past  events. 
Many  cases  of  sexual  neurasthenia  are  largely  psychasthenic  in  origin. 
The  fears  produced  by  reading  advertisements  of  quack  remedies  for 
impotence,  together  with  the  self-reproach  at  past  abuse  or  excesses 
produce  a  state  of  psychic  impotence  much  harder  to  combat  than  any 
of  the  local  urethral  conditions  accompanying  the  sexual  neurasthenia, 
and  may  lead  to  psychasthenia. 

Symptoms.  Morbid  Fears,  —  One  of  the  most  prominent  symptoms 
of  psychasthenia  is  a  condition  of  the  mind  which  is  attended  by  a  sense 
of  alarm  and  a  constant  apprehension  destroying  all  sense  of  comfort. 
This  does  not  appear  to  be  reasonable  or  to  be  based  upon  any  definite 
idea.  It  is  an  indefinite  sense  of  fear  without  reason,  but  nevertheless 
dominates  the  mind  as  definitely  as  a  reasonable  alarm.  No  amount 
of  argument  as  to  the  unreasonable  nature  of  these  fears  appeals  to  the 
patient  or  succeeds  in  allaying  the  sense  of  distress.  As  examples  of 
these  morbid  fears  the  following  may  be  cited.  The  patient  will  be 
afraid  to  touch  certain  objects  for  fear  of  the  object  contaminating  him. 
He  will  fear  to  touch  a  knife  lest  he  may  cut  himself  or  lest  he  may 
be  siezed  with  an  impulse  to  hurt  himself.  He  may  fear  to  go  up  a 
flight  of  stairs  or  to  come  down  a  flight  lest  he  fall.  He  may  fear  to 
go  into  a  certain  room  in  the  house,  though  he  cannot  say  why  this 
room  is  distasteful.  He  may  fear  to  stay  in  a  chureh  or  theatre  with- 
out any  assignable  cause.  He  may  fear  to  walk  across  an  open  square 
and  in  consequence  will  go  a  roundabout  way  through  narrow  streets 
to  avoid  it,  or  he  may  fear  to  walk  through  narrow  streets  near  high 
buildings,  and  hence  make  a  long  detour  in  order  to  go  through  wide 
avenues.  He  may  be  beset  by  a  dread  that  anything  he  does  is  wrongly 
done.  Thus  one  patient  would  spend  hours  putting  on  and  taking  off" 
her  stockings,  because  of  an  indefinite  dread  that  she  had  not  put  the 
right  stocking  on  first.  Another  patient  would  go  to  the  gas  fixture 
forty  or  fifty  times  in  succession  and  feel  of  it,  fearing  that  it  had  not 
been  properly  turned  off".  A  postman  lost  his  job  because  he  feared 
that  he  had  not  locked  the  last  box  from  which  he  had  made  the  col- 
lection properly,  and  would  go  back  in  order  to  try  the  box  and  thus 
lost  time.  One  patient  whenever  she  sees  a  match-box  wonders  if  the 
matches  in  it  are  safe  ;  might  they  get  out  ?  might  they  be  lighted  and 
set  things  on  fire  ?  Hence  she  goes  to  the  match-box  and  makes  sure 
that  it  is  shut,  and  may  repeat  this  twenty  times.  She  cannot  reason 
with  herself  in  regard  to  this,  but  she  has  this  intense  fear  of  matches. 
If  she  has  a  medicine  she  fears  it  will  get  out  of  the  bottle  and  do  some- 
one an  injury.  She  goes  every  little  while  to  feel  if  the  bottle  is  corked. 
Another  patient,  in  reading,  reads  very  slowly  and  notices  each  word 
separately,  fearing  that  she  will  skip  a  word,  and  this  fear  is  so  constant 
that  it  destroys  the  pleasure  of  reading.  Another  patient  has  a  morbid 
fear  of  going  out  of  the  house  ;  he  feels  perfectly  comfortable  while  at 
home,  but  nothing  will  induce  him  to  venture  out,  though  there  is  no 
reason  whatever  that  he  should  not  go.     It  would  be  possible  to  go  on 


SYMPTOMS.  865 

citing  instances  of  these  morbid  fears  indefinitely.  Janet  and  Stanley 
Hall  have  tabulated  over  200  examples  of  such  fears,  and  Greek  names 
have  been  given  for  many  of  them,  such  as,  claustrophobia,  fear  of 
closed  places  ;  agoraphobia,  fear  of  open  places  ;  misophobia,  fear  of 
dirt ;  pyrophobia,  fear  of  fire,  etc. 

These  fears  are  not  continuous  but  occur  from  time  to  time  in  the 
mind  of  the  patient,  giving  rise  to  great  distress  while  they  remain,  but 
being  entirely  lost  sight  of  for  hours  when  the  mind  is  occupied  with 
other  things,  and  if  the  mind  can  be  very  suddenly  or  forcibly  turned 
into  a  different  channel  the  fear  suddenly  disappears  and  the  patient 
obtains  relief. 

The  origin  of  the  fear  is  never  clearly  explained  by  the  patient  and 
as  a  rule  is  not  known  by  him.  Many  of  the  fears  are  absurd  and  do 
not  appear  to  have  any  rational  explanation.  Others  are  manifestly 
connected  with  some  experience,  but  in  a  stronger  mind  would  be  com- 
bated or  ignored,  and  it  is  the  existence  of  the  fear  that  is  the  proof 
of  the  psychasthenic  condition. 

As  we  shall  see  a  little  later  the  origin  of  these  fears  is  probably  a 
subconscious  mental  process,  the  emotional  equivalent  of  which  appears 
in  the  domain  of  attention  while  the  underlying  processes  of  reasoning 
remain  below  the  threshold  of  consciousness,  and  in  proof  of  this  it  is 
urged  that  these  fears  can  be  quieted  sometimes  permanently  but 
always  temporarily  by  suggestion  in  a  state  of  hypnosis. 

Morbid  Impulses.  —  Another  of  the  symptoms  of  psychasthenia  is 
the  development  in  the  mind  of  a  sudden  morbid  impulse  to  do  some 
act  which  may  be  absurd  or  may  be  injurious  to  some  one,  and  which, 
though  contemplated,  is  not  necessarily  carried  out.  The  idea  of 
carrying  it  out  however  throws  the  mind  into  a  state  of  anxiety  which 
is  immediately  relieved  when  the  act  is  done,  and  which  remains  if 
the  act  cannot  be  accomplished.  Examples  of  these  morbid  impulses 
are  the  following.  The  patient  will  have  an  impulse  in  walking  on  the 
street  to  avoid  treading  on  the  cracks  between  the  paving  stones.  He 
will  have  an  impulse  to  count  his  steps.  He  will  have  an  impulse  to 
count  the  windows  of  the  houses  which  he  passes  or  to  count  the  people 
passing  him.  He  will  have  an  impulse  to  touch  certain  objects  in  the 
room  or  to  touch  a  certain  part,  for  example,  a  button  on  another 
person.  He  will  have  an  impulse  to  accentuate  certain  words  in  a 
sentence,  to  repeat  a  word  over  or  to  bring  a  certain  word  into  every 
sentence  which  he  utters.  He  will  have  an  impulse  to  conceal  certain 
objects  or  even  to  steal,  there  being  no  object  in  the  theft  excepting 
the  gratification  of  the  sudden  impulse  to  take  and  conceal  an  object. 
He  will  have  an  impulse  to  destroy  various  objects  or  to  set  fire  to 
things. 

It  will  be  noticed  that  all  of  these  impulses  are  things  which  would 
be  natural  in  a  little  child,  as  all  children  delight  in  touching,  conceal- 
ing, destroying,  burning,  etc. 

More  objectionable  impulses  are  those  which  involve  an  ilijury  to 
others. 
55 


866  PSYCEASTHENIA. 

Thus  one  patient  who  lives  with  two  aunts  has  a  constant  im- 
pulse to  push  these  old  ladies  down  stairs,  and  always  hopes  that 
when  they  step  upon  the  rugs  the  rugs  will  slip  so  that  they  will 
fall.  She  also  has  an  impulse  to  throw  things  at  them,  and  although 
she  has  never  done  anything  to  injure  the  old  ladies  or  given  them 
any  intimation  in  the  course  of  five  years  of  her  actual  feelings  towards 
them,  as  she  is  dependent  upon  them,  yet  her  life  is  made  miserable 
by  the  presence  of  these  constant  impulses.  I  have  known  a  mother 
who  had  a  constant  impulse  to  drown  or  to  kill  her  children  when  they 
were  infants,  and  who  has  been  much  perturbed  by  this  impulse,  though 
she  loves  her  children  and  has  never  done  them  harm. 

In  the  majority  of  cases  no  reason  can  be  assigned  by  these  patients 
for  the  existence  of  the  impulse.  It  comes  suddenly  into  the  mind 
with  a  desire  to  perform  the  act  and  is  at  once  relieved  when  the  act 
is  performed,  but  gives  rise  to  a  state  of  anxiety  if  the  act  cannot  be 
performed. 

Here  again  the  theory  of  subconscious  unrecognized  mental  proc- 
esses is  brought  forward  to  explain  the  condition.  In  normal  mental 
processes  every  act  is  the  outcome  of  a  preceding  logical  train  of 
thought.  It  is  supposed  that  in  the  case  of  a  morbid  impulse  the 
train  of  thought  occurs  below  the  level  of  consciousness  and  that 
only  its  conclusion  comes  within  the  domain  of  consciousness  as  an 
impulse.  The  basis  of  this  theory  is  the  fact  that  in  many  such 
cases,  if  the  patient  be  hypnotized  and  questioned  the  train  of  thought 
leading  to  the  impulse  can  be  discovered ;  and  if  in  the  hypnotic  state 
this  train  of  thought  be  combated  and  the  act  which  is  its  logical  out- 
come be  shown  to  be  either  useless  or  harmful  and  a  command  be 
given  to  abandon  the  thought,  the  patient  on  recovering  from  the 
hypnosis  will  be  temporarily  relieved  of  the  impulse  and  by  repeated 
hypnosis  will  be  cured. 

Morbid  Ideas.  —  Morbid  ideas  may  also  appear  as  a  symptom  in 
psychasthenia,  at  times  being  attended  by  morbid  fears  and  impulses, 
but  at  times  being  without  either  element.  One  patient  had  the  morbid 
idea  that  all  the  Christians  were  going  to  kill  all  the  Jews,  and  was 
then  perplexed  with  the  thought  of  what  Mr.  X,  who  was  a  Christian, 
would  do  when  all  the  Jews  were  killed,  his  wife  being  a  Jewess.  One 
patient  was  perplexed  with  the  idea  that  electrical  influences  were 
acting  upon  him  constantly  through  wireless  telegraphy  and  that  every 
thought  in  his  mind  was  controlled  by  a  telegraphic  message  some- 
where in  the  air.  A  morbid  idea  which  becomes  fixed  in  the  mind 
and  which  is  accepted  as  true  and  made  the  basis  of  action  is 
termed  a  delusion,  and  delusions  of  a  temporary  character  are  not 
infrequent  in  psychasthenia.  When  such  delusions  become  fixed  in 
the  mind  and  so  constantly  present  in  the  thought  as  to  be  made  the 
subject  of  constant  contemplation  and  of  action,  the  individual  is 
dominated  by  it  and  passes  from  the  border  land  of  psychasthenia  into 
the  admitted  territory  of  insanity  and  belongs  to  the  paranoiac  type. 
As  a  rule  such  fixed   delusions  are  rarely  curable  by  hypnotism^ 


SYMPTOMS.  867 

although  JTanet  has  reported  ^  several  cases  in  which  they  have  been 
relieved  by  suggestion.  It  is  not  certain  that  the  effects  of  his  treat- 
ment have  been  permanent,  as  the  cases  may  have  relapsed  after  the 
publication  was  made. 

Instability  of  conduct  is  so  characteristic  of  persons  suffering  from 
psychasthenia  that  it  may  be  mentioned  as  a  symptom  of  this  disease. 
The  ordinary  train  of  thought  presents  a  definite  order  and  succession 
of  ideas,  and  there  is  a  logical  connection  between  them.  Such  a 
logical  train  of  thought  leads  in  turn  to  appropriate  action  and  regu- 
lation of  conduct  which  is  manifestly  based  therefore  upon  adequate 
reason. 

In  a  psychasthenic  individual  there  is  to  be  found  a  tendency  to 
most  erratic  and  irregular  lines  of  thought.  Ideas  occur  to  the  mind 
without  any  apparent  logical  sequence  and  hence  consciousness  wanders 
from  subject  to  subject  just  as  the  eye  in  a  picture  gallery  travels  from 
picture  to  picture,  there  being  no  logical  connection  between  the  im- 
pressions. Hence  the  contents  of  the  mind  are  in  a  jumble  and  thought 
is  lacking  in  continuity.  A  continuous  conversation  on  a  definite 
subject  with  such  patients  is  impossible,  for  they  jump  from  subject 
to  subject  and  do  not  fix  the  attention  for  any  length  of  time  on  any 
one.  This  instability  of  thought  is  therefore  manifested  by  similar 
irregularity  of  conduct.  The  individual  is  liable  to  enter  upon  courses 
of  action  which  a  reasonable  judgment  would  condemn  and  which  are 
soon  abandoned  as  leading  nowhere  or  being  futile.  Continuity  of 
effort  in  the  conduct  of  business,  in  a  course  of  study,  in  professional 
life  or  any  definite  occupation  appears  to  be  impossible  and  the  indi- 
vidual will  vibrate  between  a  series  of  various  occupations,  going  from 
medicine  to  law  and  thence  to  theology  or  abandoning  the  original 
profession  for  business  or  a  business  man  may  change  his  occupation 
year  after  year  and  thus  in  the  end  accomplish  nothing.  Such  indi- 
viduals are  very  likely  to  enter  upon  foolish  schemes  and  unwise  under- 
takings and  cannot  be  convinced  of  their  foolish  nature  until  they  have 
squandered  time  and  money  needlessly. 

Psychic  pain  is  another  symptom  of  psychasthenia.  This  type  of 
pain  is  usually  gradual  in  onset,  persistent  day  and  night,  not  open  to 
any  relief  by  ordinary  sedatives  or  even  to  morphine,  and  frequently 
persists  for  months  or  years.  Its  location  may  be  such  as  to  be  inex- 
plicable on  the  basis  of  anatomical  structure,  as  for  example  in  a  band 
about  the  middle  of  the  arms.  The  most  common  pain  is  over  the 
eyes,  over  the  entire  head  or  at  a  point  over  the  occiput ;  but  it  may 
be  located  in  any  organ  or  limb.  The  exclusion  of  disease  in  the  part 
in  which  the  pain  is  felt,  the  character  and  duration  of  the  pain,  and 
the  fact  that  in  spite  of  complaint  the  patient  shows  none  of  the  inevit- 
able effects  of  actual  suffering  such  as  appear  in  organic  disease  or  in  in- 
cural)le  neuralgia,  force  tlie  idea  upon  the  physician  that  the  symptom 
is  really  an  hallucination  of  ])ain.  And  the  ])ro()f  is  afforded  in  such 
cases  by  its  immediate  relief  l)y  suggestion  in  hy[)nosis. 

'  N^vroses  et  Id^es  fixes,  p.  214,  Paria,  Alcan,  1898, 


868  PSYCEASTHENIA. 

States  of  double  consciousness  may  be  included  among  the  symptoms 
of  psychasthenia.  After  a  sudden  mental  shock,  severe  emotion  or 
anxiety  an  individual  may  suddenly  lose  consciousness  of  his  own  per- 
sonality and  pass  into  a  state  of  second  consciousness  in  which  his  per- 
sonality as  related  to  his  previous  mental  experience  is  lost.  He  is  to 
all  intents  and  purposes  for  the  time  being  another  person.  In  this 
state  he  has  no  recollection  of  his  previous  existence,  of  his  name, 
address  or  occupation.  He  does  not  recognize  places  or  persons,  for- 
merly familiar,  yet  he  has  no  difficulty  in  the  use  of  language  and  he 
acts  in  a  manner  in  accordance  with  normal  moral  restraint  and  in 
accordance  with  the  social  surroundings  to  which  he  is  accustomed. 
He  may  remain  in  this  condition  for  several  days  or  even  for  years, 
and  when  he  comes  out  of  this  state  into  his  previous  personality  the 
events  which  have  occurred  during  this  stage  of  second  consciousness 
are  wholly  obliterated  from  his  mind,  so  that  his  conscious  existence  is 
continuous  with  that  existence  before  the  occurrence  of  the  second  con- 
scious state. 

The  following  examples  of  this  condition  from  my  own  experience 
may  be  cited : 

A  healthy  young  man,  a  football  player  on  one  of  the  university 
teams,  while  playing  a  college  match  was  thrown  heavily  and  for  a 
few  moments  lay  unconscious  on  the  field.  Within  two  minutes,  how- 
ever, he  got  up  and  went  on  playing  and  during  six  minutes  following 
he  played  well,  he  answered  signals  of  the  captain  in  a  proper  manner, 
although  several  of  these  signals  involved  complicated  movements  and 
he  appeared  to  be  in  a  normal  state.  He  then  suddenly  became  conscious 
that  he  was  in  a  very  nervous,  trembling  condition,  and  unable  to  play, 
and  was  led  off"  the  field  in  a  state  of  great  nervous  excitability,  and  for 
several  hours  was  prostrated. 

When  he  came  to  himself,  he  had  a  recollection  of  the  fall  but  he 
had  no  recollection  whatever  of  the  plays  in  which  he  had  participated 
after  the  fall  and  which  he  had  carried  out  with  apparent  consciousness 
and  perfect  accuracy.  At  the  present  time  his  memory  extends  up  to 
the  time  of  the  fall  and  begins  at  the  point  where  he  felt  the  tremor 
and  prostration,  but  he  has  absolutely  no  recollection  of  the  six  minutes 
subsequent  to  the  fall  during  which,  as  already  stated,  he  played  a  good 
game. 

A  man  twenty-four  years  of  age,  who  had  been  for  three  months  in 
a  state  of  great  mental  anxiety,  during  which  time  he  was  decidedly 
overworked,  suffered  from  an  attack  of  the  grippe  in  March,  1903.  On 
the  24th  of  March,  just  as  he  was  recovering  from  this  attack,  he  went 
to  his  office  as  usual  in  the  morning  and  about  noon  left  the  office  to 
do  an  errand.  He  remembers  going  out  and  crossing  Broadway  and 
having  to  jump  suddenly  out  of  the  way  of  a  car,  the  motorman  shouting 
"jump  "  as  he  did  so.  From  this  time  for  the  subsequent  week  he  has 
no  recollection  whatever  of  his  actions  and  during  this  time  he  was  lost 
to  his  family,  who  were  correspondingly  alarmed  at  his  absence  and 
took  measures  through  the  police  to  try  and  trace  him.    These  measures, 


SYMPTOMS.  869 

however,  were  futile  and  no  trace  was  found  of  liim  until  March  31, 
when  a  friend  met  him  wandering  about  in  Harlem  and  recognized 
him  and  took  him  home.  On  arriving  at  home  he  recognized  his 
friends,  complained  of  intense  headache,  seemed  much  depressed  and 
very  nervous,  and  said  that  he  had  recovered  his  memory  at  about  ten 
o'clock  in  the  morning,  when  he  found  himself  on  the  elevated  railroad 
between  104th  and  116th  Streets,  much  to  his  own  surprise.  He  got 
off  at  his  regular  station,  125th  Street,  but  did  not  appear  to  recognize 
the  streets  and  was  much  relieved  when  his  friend  appeared  and  guided 
him  to  his  house.  He  remained  in  his  house  for  the  following  few 
days  suffering  much  from  headache,  but  gradually  recovered,  and  when 
I  saw  him  on  the  9th  of  April  was  in  his  ordinary  health.  He  had 
apparently  no  recollection  whatever  of  any  events  between  the  24th  of 
March  and  the  31st  of  March. 

On  his  return  he  presented  a  somewhat  dishevelled  appearance  and 
was  without  money  and  had  apparently  had  nothing  to  eat  on  that  day, 
as  he  was  quite  famished. 

On  cross-questioning,  it  was  found  that  he  had  a  dim  recollection  as 
of  a  dream  of  having  gone  to  a  hotel  down-town  for  the  night  and  of 
having  come  once  to  Harlem  with  a  view  of  finding  his  home,  but  being 
unable  to  do  so.  Subsequent  investigation  proved  that  he  had  been  one 
night  at  this  hotel,  as  he  was  registered  there  in  his  own  name.  He  was 
seen  once  during  the  week  by  a  policeman  whom  he  knows  down-town, 
but  this  man,  though  knowing  of  the  alarm  that  had  been  sent  out,  did 
not  think  it  worth  while  to  speak  to  him  or  take  him  home,  but  told 
him  of  the  fact  later  on  when  he  was  regularly  attending  to  his  business. 
What  his  movements  were  during  this  period  of  six  days  he  has  not 
been  able  to  ascertain.  He  did  not  go  to  his  office  and  he  did  not  come 
to  his  house. 

He  was  a  man  of  good  habits.  There  is  no  suspicion  that  he  was 
making  this  an  excuse  for  having  gone  on  a  spree,  and  nothing  in  his 
history  points  to  any  reason  for  such  a  sudden  disappearance.  He  has 
been  well  ever  since. 

It  seems  probable  that  the  sudden  shock  or  fright  at  the  danger  of 
being  run  over  by  a  car  threw  him  into  a  state  of  second  consciousness, 
from  which  he  emerged  spontaneously  at  the  end  of  a  week. 

A  perfectly  healthy  athletic  man  was  much  perturbed  in  his  mind 
by  a  business  transaction  involving  the  payment  of  a  mortgage.  He 
went  to  the  bank  and  drew  the  money  to  pay  the  mortgage  and  he 
remembers  leaving  the  bank.  Three  days  later  he  found  himself  in 
the  railway  station  at  St.  Louis,  and  looked  about  him  in  surprise,  not 
recognizing  his  surroundings.  He  inquired  where  he  was  and,  when 
informed,  had  no  recollection  of  how  he  had  reached  there.  Realizing 
that  he  had  come  from  New  York  and  being  told  what  day  of  the  week 
it  was,  he  immediately  realized  that  he  had  been  away  three  days  and 
that  his  wife  must  be  anxious  about  him.  He  went  to  the  telegraph 
office  at  the  station  to  send  her  word.  He  could  not  remember  her 
name  or  his  own  or  his  address.    He  sat  down  in  a  bewildered  manner 


870  PSYCHASTnENlA. 

and  after  fifteen  minutes  the  name  of  his  clergyman  in  New  York 
came  to  his  mind  and  he  immediately  sent  a  telegram  to  him,  announc- 
ing his  whereabouts,  asking  him  to  tell  his  wife  and  stating  that  he 
was  on  his  way  home.  The  transaction  at  the  bank  next  came  to  his 
mind  and  he  was  immediately  anxious  about  the  money  which  he  had 
drawn  and  much  relieved  when  he  found  the  greater  part  of  it  still  in 
his  pocket,  showing  that  he  had  not  been  attacked  or  robbed.  Within 
a  few  minutes  he  became  aware  of  all  his  previous  existence.  He 
returned  home  and  was  brought  immediately  to  me.  He  appeared  to 
be  in  perfectly  good  physical  condition,  but  was  much  perturbed  in  his 
mind  at  his  own  experience  and  was  incapable  of  doing  his  work  or 
fixing  his  attention  for  any  length  of  time  upon  it.  He  was  emotional 
and  apprehensive  and  fearful  that  this  was  the  beginning  of  a  mental 
breakdown.  In  the  course  of  a  month,  however,  under  a  good  general 
regime  he  had  entirely  recovered  from  all  these  symptoms  and  for  the 
past  year  and  a  half  has  been  in  his  usual  state  of  health  and  actively 
employed. 

I  have  seen  a  man  who  had  been  for  three  months  in  a  hospital  in  a 
peculiar  state  of  mind.  He  had  no  knowledge  of  his  history  previous  to 
the  time  he  had  been  taken  to  the  hospital  in  a  state  of  unconsciousness. 
On  recovering  consciousness  there  he  did  not  know  his  name  or  anything 
about  himself.  After  some  weeks  his  skill  in  the  use  of  tools  convinced 
us  that  he  must  have  been  a  carpenter.  He  has  been  set  to  work  in  the 
hospital  at  that  trade.  But  he  still  has  no  clue  to  his  previous  person- 
ality.    He  appears  to  be  a  fairly  educated,  good  natured,  moral  man. 

1  could  relate  eight  other  cases  within  my  own  experience  similar  to 
these.  Dr.  Morton  Prince  has  recorded  the  more  extraordinary  case 
of  Miss  Beauchamp,^  where  during  a  considerable  period  of  years  the 
individual  vibrated  between  three  different  personalities,  the  memories 
of  each  of  which  were  separate  from  the  others. 

Consciousness  and  the  Subconscious.  —  In  the  discussion  of  these 
psychasthenic  symptoms  the  term  subconscious  ideas,  emotions  and 
impulses  has  been  constantly  employed,  and  before  proceeding  to  discuss 
the  treatment  of  these  symptoms  by  suggestion,  it  may  be  wise  to  con- 
sider what  is  meant  by  the  term  subconscious.  Physicians  use  this  term 
much  more  freely  than  psychologists.  In  fact,  a  considerable  portion 
of  the  latter  refuse  to  admit  any  sharp  demarkation  between  the  two 
states  implied  in  the  term. 

MarshalP  says  that  the  word  consciousness  is  used  to  express  two 
diverse  meanings  :  first,  psychic  existence  and,  second,  awareness.  He 
likens  our  psychic  existence  (see  Fig.  312)  to  a  mass  of  fluid,  the  surface 
of  which  is  thrown  into  waves,  each  big  wave  having  its  few  minor  waves 
around  it,  with  ripples  on  their  surfaces,  but  each  wave  being  but  a 
small  mass  as  compared  with  the  greater  mass  of  fluid  of  which  it  is 
composed  and  by  which  it  is  surrounded.  Consciousness  in  the  sense 
of  psychic  life  is  represented  by  the  mass  of  fluid.     Consciousness  in 

^  Morton  Prince.     Dissociation  of  Personality,  Longmans,  Green  &  Co. 

2  Consciousness,  Henry  Eutgers  Marshall,  LL.  D.,  The  Macmillan  Co.,  1909,  p.  169 
et  seq.     I  am  indebted  to  Dr.  Marshall  for  the  use  of  his  figures. 


STmptOM^.  S7i 

the  sense  of  awareness  is  represented  by  the  wave  with  its  wavelets 
and  ripplef^ 

A  few  only  of  our  mental  experiences  are  clearly  present  to  the  mind 
at  one  time  ;  an  infinitely  small  number  in  comparison  with  the  number 
of  experiences  which  we  have  stored  in  memory.  The  reader^s  atten- 
tion may  at  present  be  fixed  upon  this  train  of  thought  and  he  may  be 
also  slightly  conscious  of  memories  of  psychological  discussions  or  of 
medical  cases  illustrating  some  of  the  points  thus  far  brought  out.  He 
may  also  be  dimly  conscious  of  the  surroundings  in  which  he  is  sitting, 
of  the  room  and  its  furniture,  of  the  state  of  his  body  as  it  rests  upon 
the  chair  in  comfort  or  discomfort.  But  he  is  not  conscious  of  the 
events  of  the  past  day  or  week  or  month  or  year,  though  they  lie  ready 
to  be  recalled  by  any  appropriate  associated  impulse.  In  that  mass  of 
conscious  or  unconscious  memories  there  are  mingled,  as  it  were,  all 
those  precepts  of  conduct  which  constitute  the  moral  system  and  which 
may  be  represented  for  the  sake  of  the  analogy  as  giving  a  specific 


Fig.  312. 


The  figure  represents  a  mass  of  fluid  thrown  into  waves,  each  wave  having  ripples  on  its  sides. 
(Courtesy  of  Henry  Rutgers  Marshall.) 

gravity  to  the  fluid,  thus  determining  in  a  way  the  height  of  its  wave. 

If  this  analogy  be  admitted  we  can  see  at  once  that  psychic  conscious- 
ness is  an  element  of  our  entire  existence,  while  awareness  is  the  quality 
of  the  thought  of  the  moment.  It  will  also  be  evident  that  the  sub- 
conscious, if  it  be  taken  to  be  coincident  with  psychic  consciousness  as 
contrasted  with  awareness  or  with  the  mass  of  fluid,  not  thrown  up  in 
the  wave,  is  enormous  in  its  extent  and  complexity  as  contrasted  with 
the  conscious  taken  in  the  sense  of  awareness  which  is  the  thought  of 
the  moment.  Those,  then,  who  ascribe  a  great  effect  upon  the  mind 
both  normal  and  diseased  to  subconscious  influences,  ideas,  fears  and 
impulses  have  certain  facts  in  their  favor.  For  it  is  clear  that  the 
mental  life  is  infinitely  wider  than  the  mental  state  of  the  moment  and 
must  determine  in  largv.  degree  that  state.  It  is  therefore  also  clear 
that  it  is  unwarrantable  to  make  a  sharp  distinction  between  the  con- 
scious and  the  subconscious. 

Marshall '  gives  another  diagram  (Fig.  313),  which  he  terms  a  neur- 
urgic  pattern  and  which  may  be  taken  to  represent,  in  a  way,  the 
physical  l)asis  of  mental  activity.  Various  groups  of  nervous  elements, 
representing  ideas  or  memories,  are  associated  with  one  anotller  in 
closer  or  looser  relation,  but  no  idea  exists  without  some  association. 

^  L.  c,  page  37. 


872  PSYCSASTHENIA. 

All  of  these  nervous  elements  are  in  some  measure  active  during  life 
and  they  are  so  bound  together  that  any  emphatic  activity  in  any  one 
element  must  directly  or  indirectly  influence  the  activities  of  all  the 
rest  of  the  elements  of  tlie  system  and  must  in  turn  be  influenced  by 
them.  If  the  activity  of  some  particular  system  of  elements  is  mark- 
edly emphasized  it  will  stand  away  in  contrast  from  the  whole  great 


The  figure  represents  the  contents  of  the  consciousness;  a  part,  under  A,  heing  present  in  attention, 
the  portion  P  representing  self-consciousness  ;  a  part,  under  B,  being  outside  the  range  of  attention 
and  hence  subconscious,  a  part,  under  C,  being  so  far  removed  from  consciousness  as  to  be  almost 
inaccessible. 

mass  of  unemphatic  activities.  This  condition  is  shown  in  the  figure 
under  the  line  A,  which  represents  the  ideas  of  which  we  have  an  acute 
sense  of  awareness  together  with  the  presentation  of  ourselves  as  think- 
ing of  these  ideas  and  hence  being  self-conscious  or  conscious  of  our 
own  personality  (shown  in  the  diagram  as  P). 

The  immensely  complex  nervous  system  is  represented  in  an  artifi- 
cially simplified  manner  as  the  groups  of  circles  under  line  B.  This 
corresponds  to  our  psychic  existence  or  general  consciousness,  which  is 
not  within  the  field  of  attention  and  of  which,  therefore,  we  are  not 
aware.  But  any  one  of  these  groups  of  circles  may  be  reached  by 
means  of  the  direct  or  indirect  lines  connecting  it  with  the  others,  and 
any  group  may  in  turn  be  separated  off  from  the  others  as  was  the 
group  under  line  A  and  thus  enter  the  field  of  attention. 

If  we  liken  our  memories  and  thoughts  to  the  circles  in  the  diagram 
it  is  easy  to  suppose  that  some  of  them  may  have  a  weaker  association 
with  the  rest  than  others.  These  are  shown  under  the  line  C.  Only 
by  means  of  a  few  lines  of  thought  can  we  reach  these  memories.  And 
it  is  conceivable  that  even  those  few  lines  may  be  severed,  temporarily 
or  permanently,  in  which  case  the  memory  cannot  be  reached.  In  the 
phenomena  of  aphasia  we  have  studied  permanent  loss  of  associations. 
It  is  conceivable  that  in  functional  conditions  a  temporary  suspension 
of  associations  may  occur.  And  this  may  be  taken  as  the  basis  of  the 
dissociation  of  ideas  which  is  hypothecated  to  explain  some  of  the 
symptoms  of  psychasthenia  and  of  hysteria.  The  suspension  of  the 
association  cuts  off  the  group  from  voluntary  recollection.  It  prevents 
the  "  awareness "  but  it  does  not  remove  the  group  from  "  psychic 


SYMPTOMS.  873 

existence  "  or  deprive  it  of  its  power  to  influence  that  part  of  conscious- 
ness which  IS  implied  in  that  term. 

To  bring  the  group  again  into  relation  with  the  other  elements  —  to 
reestablish  the  association  —  certain  artificial  means  may  be  employed. 
This  means  is  known  as  psycho-analysis.  In  this  process  a  series  of 
'  words,  nouns  and  verbs,  taken  more  or  less  at  random  are  mentioned, 
one  after  another  to  a  patient,  and  by  a  stop  watch  indicating  seconds, 
a  record  is  made  of  the  time  elapsing  between  the  mention  of  the  word 
to  him  and  his  utterance  of  the  first  word  that  comes  to  his  mind  when 
he  hears  the  word  mentioned. .  This  reveals  the  natural  association  in 
the  mind  between  the  two  words  and  when  this  associated  process  goes 
on  undisturbed  by  any  previous  emotional  association  with  either  word, 
it  takes  about  a  second  or  even  less.  If,  however,  the  word  uttered 
awakens  a  memory  that  is  connected  with  an  emotion,  either  conscious 
or  unconscious,  a  certain  perturbation  of  the  mind  ensues,  which 
hampers  the  rapidity  of  the  association  and  is  indicated  to  the  observer 
by  the  lengthening  of  the  process  of  association,  so  that  the  interval  is 
two,  three  or  even  five  seconds.^  The  words,  then,  at  which  such 
lengthened  processes  of  association  are  present  are  taken  as  a  basis  for 
a  possible  train  of  thought,  and  by  their  reiteration  to  the  patient  may 
stimulate  his  memory  so  that  it  recalls  an  event  of  his  life  which  he 
either  wishes  to  conceal  or  has  forgotten.  If  this  can  be  recalled  and 
can  be  described  to  the  examiner,  the  mere  act  of  confession  appears  to 
relieve  the  mind  of  a  tension  which  has  been  the  cause  of  the  morbid 
idea,  impulse  or  fear.  It  is  thus  that  psychic  analysis  is  not  only 
a  means  of  diagnosis,  but  a  therapeutic  measure  in  the  study  of 
psychasthenia. 

It  seems  as  if  a  group  of  ideas  which  have  been  separated  from  the 
mental  contents  of  the  moment,  but  which  nevertheless  remain  as  part 
of  the  psychic  life,  and  thus  have  a  sort  of  consciousness  of  their  own, 
are  by  this  method  reunited  to  the  mental  contents  and  are  thus  brought 
again  into  the  field  of  attention,  so  that  we  became  again  aware  of  them. 
Or,  to  recur  to  Fig.  313,  it  seems  as  if  the  lines  between  B  and  (7, 
which  for  a  time  had  been  impassable,  were  again  traversed  by  impulses 
and  thus  again  the  distant  circles  are  associated  with  the  central  mass 
of  nervous  elements. 

For  a  normal  action  of  the  mind,  therefore,  a  perfect  assemblage  of 
ideas,  memories  and  experiences  is  a  necessity  and  anything  which 
interferes  with  such  an  association  or  which  produces  a  dissociation 
causes  a  disturbance  in  the  process  of  thought,  and  disagreeable  sensa- 
tions of  discomfort.  We  are  all  familiar  with  the  mental  stress  which 
is  felt  when  we  see  a  face  that  is  known,  but  cannot  name  the  person. 
The  mind  makes  a  futile  effort  and  gives  it  up.  But  suddenly —  when 
we  are  thinking  of  something  wholly  foreign  to  the  face  —  the  name 
comes  to  the  mind.  An  activity  has  been  going  on  in  the  psychic  con- 
sciousness not  present  in  the  domain  of  awareness,  and  the  result  is  the 

'  See  Association  Tests,  by  Wood  worth  and  Wells.     Psychological  Monographs,  Dec, 


^74  PSYCHASfHENlA. 

forcing  into  the  field  of  attention  of  a  thing  apparently  without  natural 
association.  Many  persons  have  had  the  experience  of  going  to  sleep 
with  an  unsolved  problem  and  of  waking  to  find  the  solution  clear  to 
the  mind.  It  thus  appears  that  mental  processes  may  go  on  outside 
the  field  of  attention,  the  result  of  which  comes  spontaneously  into  that 
field,  to  the  surprise  of  the  individual. 

This  is  the  explanation  of  many  of  the  symptoms  of  psychasthenia. 
Some  act  of  the  psychic  life  occurs  outside  the  domain  of  awareness.^ 
It  leads  to  a  fear,  or  impulse,  or  idea.  That  suddenly  enters  the  field 
of  awareness  and  is  termed  morbid  because  it  is  unexplained,  unex- 
pected, not  associated  with  anything  else,  and  hence  surprises  and 
alarms  the  person.  If  the  origin  were  known,  if  the  process  of  thought 
leading  up  to  it  could  be  traced,  it  might  seem  a  natural  idea  or  it 
might  be  combated  as  either  baseless,  foolish  or  unreasonable.  But, 
like  a  tune  which  runs  in  the  head,  it  forces  itself  into  the  field  of  atten- 
tion and  cannot  be  voluntarily  combated  or  banished,  largely  because 
its  origin  is  unknown.  If  any  means  can  be  found  to  trace  the  origin 
the  mystery  is  solved,  the  alarm  and  mental  perturbation  vanish. 

Suggestion  and  Hypnosis.  —  The  only  known  means  of  tracing  the 
action  of  psychic  life  which  is  outside  the  field  of  awareness  is  to  banish 
the  element  of  awareness  and  get  below  it  at  the  underlying  psychic 
life.  This  can  be  done  by  putting  the  individual  into  the  state  of 
hypnosis  in  which  the  awareness  is  abolished  but  in  which  the  psychic 
life  appears  to  go  on  unhampered,  the  associations  formerly  suspended 
can  be  renewed,  dissociations  can  be  overcome  and  a  train  of  thought 
be  followed  up  from  beginning  to  end  or  from  end  back  to  beginning. 
The  mere  act  of  relaxation,  the  condition  of  dreamy  state  without  an 
entire  suspension  of  awareness  may  in  some  persons  suffice  so  that  sug- 
gestion and  questioning,  without  true  hypnosis,  may  be  possible.  In 
other  persons  a  deep  sleep  is  essential. 

To  hypnotize  it  is  well  to  place  the  patient  in  a  comfortable  position, 
where  relaxation  of  all  the  muscles  is  easy ;  then  to  direct  his  eyes  to  a 
bright  object  held  eight  inches  in  front  of  and  a  little  above  his  eyes, 
to  tell  him  to  fix  his  attention  on  the  object  and  what  is  said  to  him,  to 
assure  him  that  his  eyes  are  closing  as  he  gazes,  that  he  is  going  to 
sleep,  that  he  is  asleep,  and  as  he  goes  to  sleep  it  is  well  to  press  on 
the  eyes  with  the  fingers  and  to  stroke  the  forehead.  The  slight  change 
in  color,  the  deeper  respiration  and  the  evident  relaxation  of  the  body 
will  assure  one  that  the  subject  is  hypnotized.  Now  in  this  state  a 
person  will  answer  questions,  and  will  perform  acts  in  response  to 
commands.  If  morbid  fears,  impulses  and  ideas  have  been  present 
the  reason  for  them  will  be  related  in  response  to  questions.  If  states 
of  double  consciousness  have  occurred  the  patient  will  relate  in  detail 
all  that  occurred  in  the  state  of  second  consciousness.  If  some  symp- 
tom due  to  a  dissociation  of  ideas,  memories  or  feelings  has  occurred, 
such  as  a  fixed  idea  of  pain,  a  so-called  hysterical  pain,  or  a  functional 

'  For  a  discussion  of  Freud's  theory  that  these  acts  of  psychic  life  are  based  on  sexual 
experiences,  see  page  888. 


SYMPTOMS.  875 

ansesthesia,  *or  paralysis  or  blindness  or  deafness  has  been  present  the 
assurance  that  it  is  cured,  that  it  will  not  recur  when  the  person  wakes 
will  come  true  on  waking  —  for  the  association  has  been  reestablished 
in  the  dormant  state.  When  the  patient  is  wakened  he  has  no  recol- 
lection of  anything  which  has  occurred  during  the  hypnosis,  but  he 
acts  in  accordance  with  suggestions  and  commands  given  in  that  state. 
Thus,  if  he  has  been  told  that  he  will  remember  the  train  of  thought 
which  has  led  to  the  morbid  idea,  fear  or  impulse  and  will  see  that 
that  train  of  thought  is  unreasonable,  he  will  do  so  and  no  longer 
entertain  and  fear  the  morbid  thoughts.  Thus  the  hypnotic  suggestion 
cures  the  symptoms  of  psychasthenia.  To  ensure  a  permanent  cure, 
however,  the  process  must  be  repeated  from  time  to  time,  the  intervals 
being  made  longer  and  longer. 

In  cases  where  hypnosis  is  not  employed  a  similar  result  may  some- 
times be  attained  when  the  person  is  merely  put  in  a  state  of  relaxation 
and  is  talked  to  seriously,  being  assured  that  his  symptoms  are  curable 
and  will  disappear.  But  here  the  seance  must  be  longer,  it  must  be 
repeated  more  frequently  and  the  result  is  of  slower  attainment.  In 
this  respect  it  resembles  the  result  of  mere  argument,  persuasion,  or 
assurance,  which  is  used  by  every  physician  in  encouraging  a  timid 
person.  The  results,  however,  in  hypnosis  seem  more  definite  and 
positive  than  when  it  is  not  employed. 

To  awaken  an  hypnotized  person  it  is  only  necessary  to  say  "  wake 
up,"  or  to  tell  him  to  count  10  aloud  and  to  wake  as  he  says  5  ;  or 
to  stroke  the  forehead  upward,  telling  him  that  he  is  waking.  It  must 
not  be  forgotten  that  any  suggestion  made  during  sleep  will  be  retained 
on  waking.  Thus  if  when  hypnotized  the  patient  has  been  told  that 
his  arm  is  stiff  and  cannot  be  moved  and  spectators  are  shown  that  it 
is  so  for  the  time,  he  must  be  told  before  waking  that  the  stiffness  has 
gone  and  that  the  arm  is  perfectly  well  and  natural,  otherwise  on  wak- 
ing a  paralysis  or  rigidity  will  be  found  present.  And  careless  remarks 
made  by  the  hypnotizer  sometimes  lead  to  serious  mental  impressions 
which  remain  as  symptoms  when  the  person  comes  out  of  the  hypnosis. 
If  a  person  has  been  once  hypnotized  he  is  more  easily  hypnotized 
afterwards.  The  process  is  not  attended  by  exhaustion  and  seems  to 
do  no  harm.  It  should  be  done,  however,  for  a  definite  object  only, 
never  as  a  matter  of  amusement. 

While  hypnotic  suggestion  is  successful  in  some  of  these  cases  the 
majority  of  individuals  cannot  be  hypnotized  -and  very  few  physicians 
care  to  employ  this  method.  Even  in  France,  where  it  is  most  in 
vogue,  some  authorities — Babinski,  for  example — have  discarded  it  as 
either  harmful  or  needless. 

Under  these  circumstances  treatment  must  be  by  other  means.  The 
best  of  these  is  by  education  and  persuasion.  The  method  is  to  ex- 
plain fully  and  repeatedly  to  the  patients  the  import  of  the  symptoms 
present ;  to  ol)tain  their  confidence  by  patient  listening  to  and  interest 
in  tlieir  mental  states  of  doubt,  fear,  impulse,  or  perplexity  ;  to  argue 
out  the  imaginary  element  in  the  condition ;  to  urge  the  power  of 


876  PSYCHASTEENIA. 

controlling  the  thought  and  to  impel  the  patients  to  make  a  constant 
effort  to  combat  the  fear  or  impulse.  This  is  the  method  urged  by 
Dubois  aud  by  Babiuski.  It  awakens  the  belief  in  the  prospect  of  a 
cure  aud  stimulates  the  courage  of  the  patient.  It  implies  the  fact  that 
by  conscious  effort  the  subconscious  train  of  thought  may  be  controlled. 
That  effort  must  be  constant,  or  frequently  repeated,  and  must  be  aided 
by  the  encouragement  and  personal  direction  of  the  physician.  It  shows 
the  patient  the  difference  between  real  and  imaginary  symptoms  and 
shows  him  what  hope  can  do.  It  is  effective  in  many  cases  and  should 
always  be  employed  by  the  physician. 

The  psychasthenic  should  be  treated  not  only  mentally  but  her  mind 
should  be  given  constant  occupation.  This  is  to  vary  with  every  hour 
of  the  day,  and  should  involve  the  use  of  the  hands,  sketching,  wood 
carving,  moulding,  hammering  out  metals,  amateur  photography,  various 
kinds  of  farming,  carpentry,  mechanical  trades  as  well  as  games,  picture 
puzzles,  and  reading  aloud  and  committing  poetry  to  memory.  Any- 
thing which  fixes  the  attention  and  occupies  the  mind  will  distract  the 
thought  from  the  morbid  ideas,  fears,  and  impulses.  And  if  this  is 
combined  with  a  frequent  change  of  surrounding,  with  new  associations 
and  helpful  manners  of  life,  an  out-of-door  country  life  and  freedom 
from  care,  anxiety  and  effort,  the  symptoms  may  subside,  at  least  for  a 
time. 

Course.  —  Psychasthenia  as  a  rule  comes  on  very  gradually,  thougn 
occasionally  it  is  seen  to  appear  suddenly  after  a  shock.  As  a  rule 
the  individual  is  aware  of  the  fact  that  the  mind  is  not  acting  in  a 
proper  manner,  that  ideas  and  impulses  and  fears  little  by  little  are 
taking  possession  of  it,  and  that  the  control  of  the  mental  action  is  not 
what  it  should  be.  Finally  various  symptoms,  such  as  those  already 
mentioned,  develop  and  take  possession  of  the  thought,  so  that  all  occu- 
pations are  abandoned,  diversion  is  impossible,  and  the  time  is  occupied 
with  the  mental  worries,  anxieties  or  impulses.  The  fears  sometimes 
reach  a  point  where  they  are  attended  by  the  ordinary  physical  symp- 
toms of  a  normal  fear,  excessive  perspiration,  feelings  of  faintness  and 
rapid  heart.  The  same  physical  condition  of  anxiety  may  also  develop 
in  connection  with  impulses  that  are  not  gratified.  Thus,  if  a  patient 
restrain  his  impulse  to  touch  certain  objects  or  to  j)ut  on  a  garment  in 
a  certain  way  and  forces  himself  to  do  an  act  contrary  to  his  impulse, 
he  gets  into  a  state  of  greater  anxiety,  which  is  only  relieved  when  he 
carries  out  the  impulse  to  its  completion.  Thus  the  life  is  spent  in  a 
futile  effort  to  satisfy  the  impulses,  to  argue  out  the  ideas  or  to  avoid 
any  course  in  conduct  which  would  produce  a  fear. 

Sometimes  a  change  of  scene  and  travel,  sometimes  the  adoption  of 
a  course  of  treatment  involving  constant  occupation,  especially  with 
the  hands,  as  in  arts  and  crafts,  sometimes  a  sudden  grief  or  emotional 
shock  of  a  new  character  seem  to  alleviate  the  symptoms  and  result  in 
their  subsidence  and  patients  are  very  often  free  from  symptoms  for  a 
considerable  time.  But  they  are  very  prone  to  relapses  and  after  any 
renewed  emotional  strain  or  physical  illness,  all  the  former  symptoms 


COURSE.  877 

recur.  The  underlying  cause  being  a  weakened  state  of  the  nervous 
system,  it  is  hardly  to  be  expected  that  a  permanent  cure  can  be 
obtained.  Yet  these  patients  receive  great  help  from  suggestive  treat- 
ment or  from  persuasion  and  many  of  them  under  this  treatment  manage 
to  lead  a  reasonably  comfortable  life.  They  are  very  liable,  however, 
to  become  extremely  dependent  upon  anyone  who  has  succeeded  in 
treating  them  by  hypnosis  or  suggestion.  A  very  small  percentage  of 
these  cases  finally  drift  into  asylums,  it  being  impossible  for  their 
families  to  put  up  with  their  constant  eccentricities. 

For  other  means  of  treatment  the  reader  is  referred  to  the  Treatment 
of  Neurasthenia,  page  854. 


CHAPTER  LIL 

HYSTEEIA. 

Hysteria  is  a  functioDal  nervous  condition,  characterized  by  a 
permanent  mental  state,  which  may  be  termed  the  hysterical  tempera- 
ment, and  by  sudden  temporary  attacks  of  mental  or  emotional  or 
physical  kind. 

1.  The  hysterical  temperament  is  manifested  by  an  abnormally  keen 
sensibility  to  all  external  impressions  and  sensations,  by  a  high  grade 
of  imaginative  power,  by  a  susceptibility  to  suggestions,  by  an  unusual 
desire  for  attention  and  notice,  by  variations  in  mood  not  due  to  appar- 
ent causes,  by  a  lack  of  judgment,  by  a  manifest  incapacity  to  exercise 
control  over  thought,  emotion  and  action,  and  by  a  tendency  to  act  on 
sudden  impulses.  These  characteristics  lead  to  a  mental  state  which 
is  so  distinct  as  to  be  recognized  both  by  the  laity  and  by  physicians. 
The  former  consider  it  as  evidence  of  wilfulness,  of  foolish  fancies,  of 
imagination,  or  of  moral  obliquity,  and  base  this  notion  on  the  fact 
that  every  one  has  the  possibility  of  showing  such  manifestations  when 
a  relaxation  in  self-control  is  permitted.  They  point  to  the  fact  that 
under  intoxication,  or  in  the  state  of  mental  exhaustion  following  pro- 
longed anxiety  or  grief,  or  after  sudden  shock  from  fright,  the  mental 
characteristics  of  the  hysterical  temperament  may  suddenly  appear,  and 
they  hold  that  as  this  implies  a  condition  of  loss  of  self-control,  it  is 
merely  necessary  to  urge  the  importance  of  such  control  and  to  punish 
those  who  will  not  exert  it.  The  physician  and  psychologist,  on  the 
other  band,  regard  the  hysterical  temperament  as  evidence  of  some  de- 
fective interaction  of  those  mechanisms  of  the  nervous  system  through 
which  mental  and  physical  acts  are  harmonized.  These  mechanisms 
are  located  in  the  cortex  of  the  brain,  and  are  made  up  of  cortical 
centres  and  their  association  tracts.  Sensory  impulses  reaching  the 
cortex  normally  cause  mental  perceptions  which  are  recognized,  are 
related  to  other  perceptions  or  to  actions,  and  awaken  an  orderly  train 
of  thought.  In  the  hysterical  person  the  sensory  impulse  may  cause 
an  abnormally  keen  perception ;  a  slight  sound,  or  light,  or  touch,  or 
pain  being  felt  as  almost  too  intense  to  be  endured  (hysterical  hyper- 
esthesia), or  it  may  awaken  no  perception  whatever  [hysterical  anaes- 
thesia). Or  if  perceived,  the  perception  may  awaken  no  recognition 
(hysterical  amentia),  the  patient  manifesting  the  same  symptoms  as 
those  who  are  mind  blind,  or  mind  deaf.  (See  page  122.)  Or  the 
perception,  though  recognized,  may  fail  to  awaken  a  natural  train  of 
ideas  or  lead  to  natural  acts.  It  may  even  start  an  unrelated  series 
of  mental  responses,  which  like  our  dreams  bewilder  ai7d  distress  the 

878 


HYSTERIA.  879 

mind  by  their  lack  of  continuity  (hysterical  insanity).  And  these 
illogical  ideas  may  in  turn  lead  to  illogical  actions  as  confused  and 
absurd  as  the  chaotic  ideas  of  which  they  are  the  outcome  (hysterical 
morbid  impulses').  Thus  in  an  hysterical  patient  the  ordinary  external 
impression  does  not  produce  the  effect  one  would  expect.  It  may, 
however,  produce  unusual  effects.  The  capacity  of  imitation,  which  is 
normally  inherent,  is,  in  these  persons,  frequently  excessive.  If  they 
see  anyone  doing  something  unusual  (twitching,  running,  posturing, 
etc.),  they  have  an  intense  desire,  or  an  irresistible  impulse  to  do  the 
same.  If  a  sufficiently  impressive  statement  is  made,  accompanied  by 
a  command,  they  accept  it  without  question,  and  obey.  The  power  of 
judgment  and  of  critical  reason  seems  to  be  at  times  suspended,  so 
that  they  are  almost  automatic  instruments  in  the  control  of  a  stronger 
mind.  This  is  termed  suggestibility,  the  dictates  of  the  stronger  mind 
leading  to  acquiscence  without  reason. 

With  this  temperament  there  is  invariably  associated  a  hypersensi- 
tive emotional  state.  Things  which  should  cause  emotional  effects 
may  cause  none.  Or  things  which  should  cause  very  little  effect  may 
awaken  most  intense  reactions.  And  the  emotional  effect  may  be  un- 
duly prolonged,  as  well  as  intense,  so  that  a  smile  will  run  into  a  fit  of 
laughter  or- a  perfect  paroxysm  of  merriment,  or  a  slightly  depressing 
circumstarice  may  cause  long  weeping,  or  these  may  alternate.  It  is 
in  the  emotional  sphere  that  the  lack  of  self  control  is  most  apparent. 
It  seems  as  if  the  mechanisms  of  the  brain  which  are  called  into  play 
in  emotional  acts,  once  started  run  their  own  course  and  cannot  be  ar- 
rested, just  as  in  the  mental  confusion  the  thoughts  appear  to  be  beyond 
control.  It  is  true  that  a  second  emotional  shock  may  serve  to  stop 
the  action  first  started,  in  some  unknown  way ;  and  in  fact  there  ap- 
pears to  be  no  other  means  of  controlling  these  emotional  storms  than 
by  some  very  intense  impression  calculated  to  arouse  another  emotion. 
Other  evidences  of  a  lack  of  control  are  found  in  the  excessive  temper, 
the  vanity  and  desire  for  notice,  in  abnormal  shyness,  or  in  morbid 
fears  of  an  intense  kind  which  these  patients  manifest.  It  may  also 
be  shown  in  a  lack  of  power  to  arrest  some  reflex  bodily  function ;  thus 
the  act  of  coughing,  or  of  swallowing,  once  begun  cannot  be  arrested ; 
or  a  conscious  and  voluntary  motion  once  begun  has  to  be  kept  up, 
like  the  flexion  or  extension  of  a  limb  (hysterical  tic),  or  the  mainte- 
nance of  some  peculiar  position  (hysterical  contractures).  Even  so-called 
automatic  acts  of  the  body,  respiration,  the  heart  beat,  the  peristalsis  of 
the  stomach  and  intestines,  may  become  conscious  and  may  be  affected 
by  the  attention,  either  being  hampered  or  hastened.  And  finally,  the 
entire  motor  mechanism  of  the  body  may  be  thrown  into  action,  as  in 
hysterical  convulsions. 

Persons  of  hysterical  temperament  are  usually  very  introspective. 
They  watch  their  symptoms,  remember  keenly  all  their  variations  of 
feeling,  and  exaggerate  their  suffering.  They  refuse  to  allow  their 
attention  to  be  directed  to  matters  of  interest  not  connected  with  their 
own  state,  and  thus  generally  narrow  their  mental   horizon,  until  all 


880  HYSTERIA. 

thoughts  are  self-centred.  This  very  attention  to  sensations  serves  to 
intensify  the  sensations,  hence  their  suffering  is  usually  out  of  all 
rational  proportion  to  the  objective  symj)toms.  This  fact  once  appre- 
ciated by  friends  results  in  a  lack  of  sympathy  on  their  part,  which  is 
resented  by  the  patient ;  who  in  turn  seeks  to  regain  that  sympathy  by 
further  claims  to  attention,  even  to  the  extent  of  simulating  symptoms. 
Self-inflicted  wounds,  with  consequent  sores  that  will  not  heal,  the 
vomiting  of  blood  sucked  from  a  tooth  and  swallowed,  the  high  tem- 
perature obtained  by  friction  on  the  thermometer  either  in  the  mouth 
or  rectum,  emaciation  from  supposed  starvation,  and  vomiting  of  all 
food,  are  examples  of  such  symptoms. 

The  hysterical  temperament  is  not,  however,  always  manifested  by 
emotional  outbursts.  Some  individuals  will  not  permit  of  any  emo- 
tional expression.  They  repress  all  such  tendencies,  are  unusually 
reserved  and  self-contained,  yet  they  suffer  from  grief,  anxiety,  or 
fright,  equally  with  others,  and  have  not  the  natural  relief  which  comes 
from  emotional  expression.  In  these  persons  hysterical  symptoms 
often  appear  suddenly,  though  their  friends  declare  that  they  are  not 
hysterical.  It  is  often  more  difficult  to  deal  with  these  suppressed 
hysterical  persons  than  with  those  subject  to  emotional  outbreaks. 

The  vasomotor  system  is  also  in  an  abnormal  state  in  persons  of 
hysterical  temperament,  as  is  shown  by  the  pallor  or  flushing  which 
attends  their  mental  processes,  and  by  the  increased  excretion  of  urine 
which  follows  a  paroxysm  of  any  kind. 

The  hysterical  temperament,  while  a  chronic  condition,  may  not  be 
always  in  evidence.  It  is  at  times  in  abeyance  or  under  control,  and 
sometimes  it  is  outgrown  as  life  goes  on.  It  is  likely,  however,  to 
persist,  and  under  conditions  of  mental  or  physical  strain  to  reappear, 
often  complicating  the  symptoms  of  other  diseases. 

2.  The  hysterical  temperament  rarely  remains  for  any  length  of  time 
without  leading  to  sudden  temporary  attacks  of  a  mental,  emotional,  or 
physical  kind,  which  are  termed  hysterical  paroxysms.  These  are 
usually  caused  by  some  emotional  strain,  but  may  occur  without  any 
ascertainable  reason.  They  vary  so  greatly  in  their  intensity  as  to  be 
divided  clinically  into  attacks  of  hysteria  minor  and  hysteria  major. 
The  latter  are  quite  rare  in  this  country. 

(a)  Hysteria  Ilinor.  —  The  attacks  consist  of  sudden  choking  sensa- 
tions in  the  throat,  as  if  the  pharynx  contracted  (globus  hystericus),  of 
a  tendency,  which  soon  becomes  imperative,  to  cry  or  to  laugh,  or  to 
do  both  in  succession,  or  of  a  violent,  unreasonable  outburst  of  temper. 
There  is  a  feeling  of  great  nervousness,  apprehension  or  fear,  a  desire 
for  air,  a  heat  or  chilliness  throughout  the  body,  and  a  restlessness  or 
tendency  to  shout.  The  body  is  kept  in  motion  as  the  patient  wanders 
about  or  tosses  in  bed.  Soon  intense  pain  is  felt  in  the  top  or  back  of 
the  head,  or  in  the  eyes,  or  in  the  spine.  Usually  pallor  or  flushing 
accompany  the  attack.  Sometimes  sexual  excitement  occurs.  The 
mental  distress  and  excitement  may  lead  to  a  condition  like  delirium. 
Sometimes  the  patient  falls  into  a  light  slumber,  and  wakens  in  a  nor- 


HYSTERIA.  881 

mal  state.  More  often  the  attack  subsides  gradually  under  the  influ- 
ence of  remedies.  Occasionally  it  ceases  suddenly,  and  the  patient  is 
at  once  quite  well.  In  all  cases  a  large  flow  of  urine  coincides  with 
recovery.  It  is  light  in  color,  of  very  low  specific  gravity,  and  often 
contains  an  excess  of  phosphates.  A  sense  of  exhaustion  and  fatigue 
lasts  for  some  hours  or  even  days  after  the  paroxysm.  One  attack  may 
follow  another  for  several  days,  during  which  the  exhaustion  increases, 
and  recovery  is  then  very  gradual.  A  state  of  mental  distress  and  of 
depression  of  spirits  is  sometimes  observed,  but  usually  the  patient  does 
not  appear  to  regret  the  attack,  though  she  rather  dreads  its  return. 
After  an  attack  the  sleep  is  often  disturbed  for  many  nights,  being  rest- 
less and  disturbed  by  dreams  of  an  unpleasant  kind.  Occasionally 
somnambulism  occurs. 

(6)  Hysteria  Major. — The  attack  begins  with  symptoms  similar  to 
those  of  hysteria  minor,  but  in  much  greater  intensity  and  longer  dura- 
tion. The  crying  or  laughing  is  violent  and  loud,  or  the  outburst  of 
temper  so  extreme  as  to  be  maniacal.  After  this  the  patient  passes 
into  a  general  convulsive  seizure  which  consists  of  a  series  of  struggles 
with  those  about  her,  kicking  with  the  feet,  seizing  objects  and  push- 
ing them  away  with  the  hands,  throwing  the  head  and  body  about, 
arching  the  back  till  the  body  rests  only  on  the  head  and  feet  in  bed,  or 
rolling  about  on  the  floor.  In  all  these  movements  it  would  seem  to 
the  bystander  as  if  there  was  some  conscious  motive  in  every  act,  and 
as  a  rule  nothing  is  done  which  causes  any  injury  of  herself.  She  may 
bite  others,  but  does  not  bite  her  tongue  or  hurt  herself  in  her  motions. 
Yet  these  patients  rarely  show  evidence  of  consciousness  in  an  attack, 
and  usually  deny  any  memory  of  its  details.  They  cry  and  scream 
and  fight.  In  such  an  attack  the  pupils  are  dilated,  the  eyes  are 
rolled  about  or  turned  far  up,  the  eyelids  are  often  open,  yet  they  do 
not  seem  to  see.  The  respiration  may  cease  for  a  time  and  the  face 
become  cyanotic.  The  limbs  are  alternately  flexed  and  extended,  but 
sometimes  remain  rigid  for  a  time,  and  then  again  are  set  in  motion. 
The  tonic  spasm  is  followed  by  clonic  spasms  as  in  epilepsy,  and  this 
by  a  series  of  contortions  and  extraordinary  postures,  the  body  resting 
on  heels  and  head,  in  opisthotonos.  The  convulsive  attack  may  last 
an  hour  or  more,  or  recur  in  series  for  several  hours,  and  this  fact 
alone  suffices  to  distinguish  it  from  epilepsy.  In  a  few  cases  delirium 
coincides  with  or  follows  the  attack,  the  patient  talking  wildly  and 
appearing  to  be  subject  to  hallucinations.  Usually  the  attack  stops  as 
suddenly  as  it  began,  the  patient  falls  asleep  and  wakes  in  a  normal 
state.  Sometimes  the  attack  subsides  slowly.  That  consciousness 
is  not  wholly  suspended  is  proven  by  the  fact  that  strong  external 
impressions  often  arrest  the  attack.  Thus  painful  pressure  on  a 
nerve  or  sensitive  organ,  or  sharp  counter-irritation  of  the  skin,  or 
an  emetic  will  arrest  an  attack.  Sometimes  a  patient  who  is  sub- 
ject to  these  attacks  finds  that  a  strong  effort  of  the  will  prevents  their 
occurrence. 

3.  Attaciks  of  either  minor  or  major  hysteria  are  sometimes  followed 
56 


882  HYSTERIA. 

by  conditions  of  loss  of  power  of  sensation,  or  of  motion,  which  appear 
suddenly  and  disappear  as  suddenly. 

(a)  Anaesthesia  to  pain  is  the  most  common  symptom,  touch  and 
temperature  .sensations  being  often  but  slightly  aifected.  This  may  be 
limited  to  one-half  of  the  body,  and  is  always  far  more  complete  than 
in  organic  hemianalgesia.  In  hysterical  cases  no  pain  is  felt.  In 
organic  cases,  as  a  rule,  some  sensation  can  be  aroused.  The  anaes- 
thesia may  be  limited  to  the  peripheral  part  of  one  limb  or  several 
limbs.  In  such  cases  it  never  resembles  the  anaesthesia  of  a  peripheral 
nerve  lesion  in  its  distribution,  and  its  upper  limit  is  sharply  defined, 
which  is  not  the  case  in  multiple  neuritis.  (See  Plate  IV.,  page  40.) 
The  anaesthesia  occasionally  is  found  in  irregular  shaped  areas,  not  cor- 
responding to  any  anatomical  distribution  of  nerves.  Usually  it  is 
difficult  to  draw  blood  in  the  anaesthetic  area,  pin  pricks  not  bleeding 
as  in  other  parts.  Sometimes  the  analgesia  can  be  removed  by  static 
electricity,  sometimes  by  applying  magnets  or  metals  to  the  surface, 
sometimes  by  mere  suggestion.  It  may  be  transferred  by  these  agents 
to  the  other  side  of  the  body.  The  mucous  membranes  of  the  nose, 
mouth,  and  throat  are  occasionally  analgesic.  The  skin  reflexes  are 
usually  normal. 

(6)  Anaesthesia  of  the  retina  to  light  and  color  is  also  observed.  This 
causes  a  concentric  diminution  of  the  visual  field  for  color  and  for 
light,  and  in  some  cases  an  apparent  blindness  of  one  eye.  The  blind- 
ness rarely  takes  the  form  of  hemianopsia,  and  this  fact  serves  to  dis- 
tinguish it  from  a  state  due  to  organic  brain  disease.  Usually  one 
eye  is  much  more  affected  than  the  other  though  total  blindness  has 
been  observed.  The  normal  color  fields  may  be  reversed  or  altered. 
Blindness  of  one  eye  usually  coincides  with  hemianaesthesia.  The  pupil 
reflex  is  never  abolished. 

(c)  Hearing  may  be  affected,  and  total  deafness  in  one  ear  usually 
coincides  with  hemianaesthesia,  though  occasionally  it  occurs  alone. 
Sometimes  it  is  bilateral.  High  pitched  or  low  pitched  tones  may  be 
inaudible  when  ordinary  sounds  are  heard.  Bone  conduction  is 
affected  equally  with  air  conduction  —  a  condition  rather  rare  in  non- 
hysterical  deafness. 

(d)  Loss  of  taste  and  smell  on  one  side  have  also  been  observed, 
and  often  accompany  hemianaesthesia. 

(e)  Loss  of  motor  power,  or  hysterical  paralysis,  is  a  very  common 
symptom.  This  may  occur  either  as  a  hemiplegia,  or  a  monoplegia, 
or  a  paraplegia.  It  comes  suddenly  and  is  a  total  paralysis.  The 
patient  does  not  move  the  paralyzed  part  at  all,  and  seems  incapable 
of  trying.  The  degree  of  the  paralysis  is  much  greater  than  in  cen- 
tral brain  disease,  where  as  a  rule  some  slight  response  to  effort,  ex- 
cept at  the  very  outset,  is  the  rule.  The  face  is  rarely  affected.  The 
muscles  are  usually  relaxed.  There  is  no  increase  of  muscular  tone, 
there  is  no  rigidity,  there  is  no  increase  in  tendon  reflex,  and  no  change 
in  electrical  excitability.  There  is  no  tendency  of  the  arm  to  assume 
the  flexed  posture,  as  in  hemiplegia.     It  hangs  like  a  flail.     The  leg  is 


HYSTERIA. 


883 


Fig.  314. 


dragged  in  srttempts  to  walk,  or  the  knee  refuses  to  give  any  support 
to  the  body.  (See  Fig.  314.)  In  organic  hemiplegia  the  knee  is  stiff 
in  walking.  The  muscles  con- 
trolling the  voice  may  be  affected 
alone,  so  that  whispers  only  are 
possible.  This  is  called  aphonia. 
If  the  paralysis  is  paraplegic  in 
kind,  it  is  never  attended  by 
paralysis  of  the  sphincters,  or  by 
bed  sores  and  cystitis.  A  true 
clonus  is  not  obtained,  though  a 
slight  exaggeration  of  the  knee- 
jerk  is  often  present.  Associated 
with  hysterical  paraplegia,  but 
sometimes  occurring  without  it, 
is  a  symptom  described  by  the 
French  as  astasia-abasia.  This 
is  a  temporary  loss  of  power  to 
stand  or  to  walk,  so  that  the 
patient  collapses  and  is  unable  to 
rise.  The  attack  may  come  on 
unexpectedly,  and  it  seems  as  if 
the  function  of  the  cerebellum 
were  suddenly  suspended.  It 
may  be  cerebellar  hysteria,  as 
other  symptoms  are  clearly  of 
cerebral  origin. 

Sometimes  a  complete  hysteri- 
cal paralysis  is  preceded  by  a 
series  of  temporary  attacks  of 
weakness  in  one  arm  or  leg, 
which  alarm  the  patient  and  tend 
to  bring  on  a  complete  paralysis, 
much  intensified  by  fear. 

Sometimes  the  paralysis  appears  in  the  form  of  a  fixed  contracture 
of  some  muscles,  a  limb,  or  a  part  of  a  limb,  being  constantly  held  in 
a  rigid  position.  Voluntary  motion  is  thus  suspended  by  the  rigidity. 
The  postures  assumed  by  the  hand  or  foot  in  this  condition  are  always 
peculiar,  and  do  not  resemble  those  which  appear  in  organic  disease. 
The  contracture  increases  when  attempts  are  made  to  overcome  it.  The 
contractured  limb  is  often  anaesthetic.  Any  muscle  of  the  body  may 
1)6  thus  affected. 

Sometimes  tremor  of  one  hand  or  one  foot  suddenly  develops,  either 
with  or  without  paralysis.  The  tremor  is  usually  coarse,  the  move- 
ments l>cing  of  a  jerky  kind.  They  are  intermittent,  and  sometimes 
have  a  tendency  to  increase  when  an  effort  is  made  to  control  them, 
thus  resembling  the  tremor  of  multiple  sclerosis.  A  form  of  spasm 
which  is  not  uncommon  is  hysterical  singultus.     The  patient  swallows 


Hysterical  paralysis  of  the  leg.    (Icon,  de  la 

Salpetrifere. ) 

I  have  seen  an  organic  hemiplegia 


884 


HYSTERIA. 


frequently,  and  as  the  saliva  fails,  air  is  swallowed  till  the  stomach  is 
distended  with  gas ;  this  is  then  belched  up,  and  a  part  is  expelled 
into  the  intestines.  Hence  the  peristalsis  produces  rumbling  noises 
which  alternate  with  the  belching  and  excite  notice  and  cause  distress. 
Respiratory  spasms  are  sometimes  noticed.     Deep  sighing,  repeated 


Fig.  315. 


Fig  316. 


Hysterical  contracture  of  the  great  toe. 
(Icon,  de  la  Salpetri&re. ) 


Hysterica]  contracture  of  both  feet. 
(Icon,  de  la  Salpetriere. ) 


yawning,  or  irregular  agitated  breathing  may  occur,  with  or  without 
a  true  hysterical  attack.  Hysterical  cough  is  loud,  barking,  constant 
and  very  annoying,  and  often  leads  to  irritation  of  the  larynx,  when  it 
is  not  started  by  such  irritation.  Blepharospasm  of  hysterical  nature 
is  occasionally  seen.  Any  of  these  symptoms  of  sensation  or  motion 
may  come  on  suddenly  after  a  shock,  or  may  follow  an  hysterical 
attack. 

Hysterical  pain  is  another  symptom  which  is  very  common.  It  may 
be  anywhere,  but  is  usually  in  the  head  or  spine,  or  in  a  joint.  It  is 
very  acute  and  the  patients  appear  to  suffer  intensely.  It  is  out  of 
proportion  to  any  apparent  affection  of  the  organ  involved.  Thus  in 
the  spine  it  is  far  greater  than  that  due  to  meningitis.  In  a  joint  it  is 
far  more  severe  than  that  of  rheumatism.  If  these  pains  are  carefully 
studied,  they  will  always  be  found  to  be  true  hallucinations  of  pain, 
rather  than  true  pain.  They  are  never  affected  by  analgesic  remedies, 
even  by  hypodermics  of  morphine.  On  the  other  hand,  mental  sug- 
gestion often  cures  them  at  once.     They  are  mental  pains  and  are  only 


HYSTERIA. 


885 


to  be  reached  by  mental  agents.  Pain  in  the  head  may  be  either  on 
top,  as  if  a  nail  were  being  driven  into  the  head,  or  it  may  be  in  the 
back  of  the  head  and  neck,  or  it  may  be  a  general  headache.  Some- 
times it  simulates  migraine  or  neuralgia.  It  is  usually  attended  by 
hypersesthesia  of  the  scalp.  Sometimes  hysterical  girls  complain  of 
steady  constant  pain  in  the  head  and  eyes,  which  is  not  alleviated  by 
any  remedy  and  differs  from  all  other  forms  of  headache  in  its  uniform 
character  and  duration. 

Pains  simulating  angina  pectoris  are  not  uncommon  in  hysteria,  but 
are  to  be  distinguished  from  the  true  disease  by  the  absence  of  arterial 
sclerosis  or  myocarditis  and  their  frequent  recurrence  without  the 
intense  agony  and  vascular  spasm  seen  in  true  angina. 

Fig.  317. 


Hysterical  spasm  of  the  hand  and  fingers.     Icon,  de  la  Salpetriere,  IV.,  100. 

Pains  in  a  joint,  especially  in  the  knee  or  elbow,  wrist  or  ankle, 
which  are  hysterical  in  nature,  are  not  attended  by  any  heat  or  redness 
or  swelling,  but  are  usually  associated  with  a  spasm  of  the  muscles 
when  an  attempt  is  made  to  move  the  joint.  I  have  known  a  hyster- 
ical pain  in  the  knee  to  keep  a  patient  in  bed  for  three  years  and  to 
appear  in  the  other  knee  when  the  leg  had  been  amputated  as  a  last 
resort. 

Hysterical  patients  usually  are  found  to  have  some  small  circum- 
scribed areas  of  the  skin  which  are  very  sensitive  to  touch  or  pressure, 
and  irritation  of  wiiicli  may  bring  on  a  true  hysterical  attack.  These 
lie  under  the  l)reaHt  and  ov(,'r  the  ovaries  in  women,  and  on  the  S(!ro- 
tum  in  men.     They  may  also  be  found   in  the  epigastrium  or  on  the 


886  HYSTERIA. 

spine.     They  sometimes   disappear   after  counter-irritation,  or  after 
electrical  applications,  or  on  strong  suggestion. 

Catalepsy  sometimes  develops  in  hysterical  persons.  This  is  a  state 
of  plastic  rigidity  of  the  limbs.  They  can  be  put  in  any  posture  and 
will  remain  so  for  a  longer  time  than  is  possible  by  an  effort  of  the 
will.  Such  a  state  may  ensue  on  a  general  hysterical  attack,  or  it  may 
appear  after  a  mental  shock,  or  it  may  develop  suddenly  without  cause. 
The  patient  as  a  rule  seems  to  be  in  a  semi-conscious  state,  is  able  to 
hear  and  see  but  takes  no  notice,  and  is  unable  to  move  or  to  speak. 
Sometimes  there  is  a  general  anaesthesia  in  this  condition.  The  facial 
expression  is  blank,  or  the  eyes,  if  opened,  seem  not  to  see,  and  no 
response  is  made  to  questions.  The  condition  may  persist  for  hours 
or  even  for  days,  during  which  the  patient  has  to  be  fed  and  kept 
clean,  as  a  baby. 

Hysterical  sornnolence  is  a  peculiar  symptom  occasionally  observed. 
It  is  a  sleepy  state  in  which  the  patient  remains  for  days,  being  aroused 
only  with  difficulty  to  be  fed,  and  relapsing  into  a  semi-stupor.  Some- 
times it  is  difficult  to  elicit  even  motions  of  swallowing,  and  the  sleep 
resembles  coma,  respiration  being  slow  and  irregular  or  almost  imper- 
ceptible, and  the  heart  action  slow  and  feeble.  This  is  the  state  called 
trance,  in  which  a  patient  may  lie  apparently  dead  for  days,  and  then 
recover  consciousness.  The  duration  is  usually  only  a  few  hours,  but 
Krauss  has  described  a  case  lasting  thirty-two  days. 

Narcolepsy  is  a  temporary  condition  of  sleep,  which  comes  as  sud- 
denly as  an  epileptic  attack  and  passes  off  as  suddenly,  lasting  only  a 
few  minutes  or  at  most  two  or  three  hours.  It  cannot  be  distinguished 
from  sleep,  as  the  patient  can  be  awakened  by  irritation.  It  appears 
commonly  in  hysterical  persons. 

Gastric  symptoms  sometimes  occur  in  hysteria.  There  is  often  a 
loss  of  appetite,  a  loathing  of  food,  a  craving  for  unusual  articles  of 
diet,  and  possibly  an  arrest  of  secretion  of  the  gastric  juice,  and  nausea. 
Occasionally  vomiting  of  an  obstinate  kind  occurs,  all  food  being 
rejected,  and  rapid  emaciation  follows. 

Intestinal  peristalsis  may  cease,  leading  to  obstinate  constipation,  or 
in  other  cases  is  excessive  with  great  evolution  of  gas,  causing  tym- 
panites and  borborigmi.  Phantom  tumors  of  the  abdomen  are  due  to 
gas  in  the  intestines.  They  usually  simulate  pregnancy,  but  disappear 
under  ether. 

Suppression  of  urine  for  twenty-four  or  forty-eight  hours  occasion- 
ally occurs  in  hysterical  patients,  but  does  not  lead  to  symptoms  of 
uraemia  as  might  be  supposed.  The  menses  are  usually  irregular  in 
hysterical  women.  They  may  be  excessive  and  lead  to  sensations  of 
great  prostration,  which  are  really  due  to  fear  :  or  they  may  be  very 
scanty  or  even  suspended.  In  hysterical  women  the  fear  of  pregnancy 
may  stop  menstruation  for  months.  It  is  well  known  that  all  these 
vasomotor  and  sympathetic  disturbances  can  be  produced  or  cured  by 
hypnotic  suggestion,  and  in  hysteria  it  often  seems  as  if  many  of  the 
symptoms  Avere  produced  by  auto-suggestions  of  hallucinatory  origin. 


THEOBIES   OF   THE  DISEASE.  887 

It  is  not  trije,  however,  that  hypnosis  can  be  more  easily  produced  in 
hysterical  individuals. 

Theories  of  the  Disease. — We  know  very  little  regarding  the 
origin  and  mechanism  of  hysteria,  and  for  this  reason  many  theories 
have  been  advanced  to  explain  the  sumptoms. 

Babinski  ^  defines  hysteria  as  a  psychical  state  rendering  the  subject 
capable  of  autosuggestion,  and  manifesting  itself  both  by  primary  and 
by  secondary  symptoms.  The  characteristic  of  the  primary  symptoms 
is  that  it  is  possible  to  reproduce  them  by  suggestion  with  a  rigorous 
exactness  in  certain  persons  and  to  remove  them  only  by  means  of 
persuasion.  The  characteristic  of  the  secondary  symptoms  is  that  they 
are  directly  subordinated  to  the  primary  symptoms.  Suggestion  is  an 
act  by  which  one  attempts  to  enforce  upon  another  the  acceptance  of 
an  idea  in  itself  unreasonable.  Persuasion  is  an  act  of  enforcing  the 
acceptance  of  an  idea  which  is  true.  For  example,  to  tell  a  normal 
person  that  his  arm  is  paralyzed  and  that  he  cannot  move  it  constitutes 
a  suggestion,  for  it  is  contrary  to  common  sense.  To  declare  to  a 
person  attacked  with  a  psychical  paralysis  that  his  trouble  is  purely 
imaginary,  that  it  can  disappear  instantaneously  by  an  effort  of  his 
will  and  thus  to  obtain  a  cure  is  to  act  by  persuasion.  If  this  defini- 
tion is  accepted  it  makes  hysterical  symptoms  quite  definite  and  gives 
them  a  character  which  organic  symptoms  do  not  have,  for  these  cannot 
be  reproduced  by  suggestion.  Thus  facial  palsy  of  the  peripheral  type 
where  the  eye  is  always  open,  or  radial  palsy  with  paralysis  of  the 
supinator  lougus  in  flexion  of  the  arm  could  never  be  p;  oduced  by  sug- 
gestion. On  the  other  hand,  hysterical  contractures  or  paralysis  or 
fears  can  only  be  removed  by  persuasion,  other  means  being  useless. 
This  definition  makes  hysteria  a  purely  mental  malady  under  the  con- 
trol of  psychic  phenomena,  suggestion,  autosuggestion  and  persuasion. 
It  has  been  quite  universally  accepted  and  not  only  explains  many  of 
the  symptoms,  but  also  the  remarkable  cures  by  means  which  could  act 
only  through  mental  influence.  If  it  is  adopted  it  points  the  way  to 
the  only  means  of  successful  treatment,  viz.,  mental  persuasion. 

According  to  Janet  hysteria  is  a  purely  mental  disease  and  the  physi- 
cal symptoms  that  are  present  are  wholly  the  result  of  mental  processes. 
The  fundamental  idea  of  Janet  is  that  in  a  hysterical  individual  certain 
groups  of  ideas  belonging  to  the  psychic  life  are  dissociated  from  the 
total  mass  of  ideas,  so  that  they  are  no  longer  brought  into  the  range 
of  consciousness  by  the  ordinary  process  of  association,  but  when  they 
are  brought  into  the  field  of  attention  by  some  apparently  spontaneous 
internal  energy,  take  possession  of  the  field  of  attention  to  the  exclusion 
of  everything  else  and  cannot  be  voluntarily  banished  any  more  than 
they  can  be  voluntarily  recalled.  He  calls  attention  to  the  fact  that 
the  common  factor  in  all  hysterical  manifestations  of  whatever  nature 
is  the  existence  of  a  state  of  absent-mindedness  or  amnesia,  and  he 
characterizes  the  state  in  which  the  hysterical  patient  is  as  a  state  of 

'Soc.  de  Neurolof^.,  Nov.  7,  1901.  Revue  de  Neurologie,  1001.  Art.  Hysteric. 
Diitil  ;  Trait<5  de  Medicine,  vol.  x.,  p.  668,  1905. 


888  htstehia. 

somnambulism.  In  this  state  of  somnambul ism  the  individual  is  entirely 
abstracted  from  his  normal  surroundings  and  his  mind  is  centered  upon 
some  idea  or  scene  or  action  to  which  he  gives  himself  up  entirely  and 
be  goes  through  a  series  of  performances,  just  as  a  somnambulist  does, 
which  seem  to  the  onlooker  to  be  v/ithout  reason  but  which  are  really 
the  normal  following  out  of  the  idea  which  is  present  to  him  in  his 
state  of  somnambulism.  In  other  words,  his  mind  is  occupied  for  the 
time  being  by  a  single  idea,  thought,  or  motive,  which  absorbs  his 
consciousness  and  dominates  his  action,  but  this  is  in  no  way  correlated 
with  his  normal  state,  and  when  the  normal  state  is  resumed  he  has  no 
recollection  whatever  of  what  has  occurred  in  the  abnormal  condition. 
The  minor  attacks  of  hysteria  Janet  would  consider  as  examples  of 
imperfect  somnambulism.  The  phenomena  of  double  consciousness  he 
would  consider  the  most  perfect  exhibition  of  an  hysterical  state.  The 
retraction  of  the  field  of  personal  consciousness  is  with  Janet  the  essence 
of  the  hysterical  condition. 

If  we  recur  to  the  discussion  of  the  subconscious  already  given  on 
page  870,  and  the  diagram  of  Mr.  Marshall,  we  can  picture  to  our- 
selves the  hysterical  patient  of  Janet  as  having  a  mass  of  ideas  forcibly 
thrust  into  his  consciousness  like  the  high  rising  of  a  wave  above  the 
level  of  the  fluid  by  some  force  beneath  (see  Fig.  312) ;  or  as  having 
a  group  of  circles  (see  Fig.  313)  forcibly  detached  from  the  remaining 
groups  and  thrust  involuntarily  into  the  range  of  consciousness  and 
dominating  the  personality,  the  connections  between  the  circles  under 
A  and  B  being  severed  temporarily. 

This  theory  certainly  explains  many  of  the  symptoms  of  hysteria  in 
an  admirable  way  and  has  been  found  satisfactory  by  many  authors. 
Its  practical  outcome  is  found  in  the  method  of  treatment  by  hypnosis. 
In  the  hypnotic  state  Janet  finds  that  by  questioning  he  can  penetrate 
to  the  dissociated  group  of  ideas  and  by  suggestion  in  that  state  he  can 
bring  these  into  their  normal  relation  to  the  mental  content,  can  link 
the  groups  together,  can  reestablish  the  broken  connection  and  in  that 
way  arrest  the  condition  of  dissociation  which  prevents  a  normal  con- 
trol of  thought,  idea  and  impulse.  Thus  the  hypnotic  treatment  cures 
the  hysterical  symptoms. 

A  theory  of  hysteria  proposed  by  Breuer  and  Freud  is  different  from 
the  two  already  described.  Freud  ^  has  called  attention  to  the  fact 
that  our  ideas,  thought,  emotions,  experiences,  and  so  forth,  are  natur- 
ally grouped  in  our  minds  into  certain  complexes,  each  of  which  is 
somewhat  independent  of  the  rest  and  yet  influences  the  others.  For 
example,  all  the  thoughts  and  feelings  connected  with  one's  religious 
education  or  with  one's  political  affiliations  or  with  one's  family  life 
may  be  termed  the  religious  complex  or  the  political  complex  or  the 
family  complex.  Every  one  of  these  complexes,  even  when  not  appar- 
ently within  the  field  of  attention,  has  an  influence  upon  the  subject 
within  the  field  of  attention  and  tends  to  modify  any  resulting  con- 
clusion which  would  follow  from  the  consideration  of  that  subject.  One 
1  Hysteria  and  the  Psychoneuroses.     Trans,  by  A.  Brill,  1909. 


THEOBIES   OF   THE  DISEASE.  88& 

may  be  wholly  unaware  that  the  fact  of  such  a  complex  is  determining 
the  thought  or  the  conclusion  arrived  at,  yet  the  complex  nevertheless 
has  its  continuous  and  permanent  effect.  We  express  this  by  saying 
that  a  person  is  "  unconsciously  biased  "  in  his  thought.  The  mind, 
then,  never  acts  freely,  but  is  always  influenced  by  these  underlying 
complexes  to  a  greater  or  less  extent.  The  more  fundamental  and  primi- 
tive the  complex  affecting  it  the  more  dominating  will  be  the  effect  of 
that  complex  upon  the  mental  activity. 

According  to  Freud  the  most  fundamental  of  all  complexes  is  the 
sexual  complex,  which,  he  believes,  originates  very  early  in  infancy 
and  maintains  its  dominating  influence  through  life.  He  holds  that 
all  love,  even  the  love  of  the  child  for  its  parent,  is  primarily  sexual  in 
origin.  It  is  the  tendency,  however,  of  general  education  to  repress 
the  discussion  of  this  sexual  complex.  Many  ideas  connected  with  it 
are  unpleasant,  mortifying  or  shameful,  and  the  tendency  of  training 
is  to  combat  its  expression.  For  Freud,  sexual  abuse  or  sexual  trauma 
exists  in  everyone  at  some  early  age.  The  constant  effort  to  ignore  or  to 
repress  these  disagreeable  and  shameful  experiences  connected  with  the 
sexual  complex  produces  a  state  of  mental  perturbation  and  this 
in  turn  causes  a  very  morbid  stress  of  mind,  attended  by  unpleasant 
emotions.  The  group  of  ideas  is  being  pushed  into  the  background, 
is  being  detached  from  the  conscious  life  by  an  effort  attended  by  an 
emotion,  but  the  emotion  is  one  of  anxiety  to  conceal  the  sexual  com- 
plex, or  of  fear  lest  it  be  discovered,  or  of  shame  at  its  existence,  and 
hence  any  thought  which  suggests  the  sexual  complex  tends  to  awaken 
the  emotions  connected  with  it. 

And  if  the  repression  is  successful  the  complex  after  a  time  no 
longer  consciously  influences  the  course  of  thought.  It  is  not  thereby 
obliterated  from  the  mind  but  remains  in  the  domain  of  the  subcon- 
scious. From  the  subconscious  Freud  imagines  that  there  is  a  constant 
tendency  for  the  complex  to  emerge  and  hence  the  process  of  repression 
is  kept  up  all  the  time  by  an  act  of  the  mind  which  he  calls  "  censure." 
Such  an  act  is  attended  by  more  or  less  emotion  and  hence  the  indi- 
vidual may  be  constantly  under  an  emotional  strain.  This  emotion 
may  then  be  transferred  from  the  unconscious  train  of  thought  to  some 
conscious  train  of  thought,  lending  an  emotional  character  to  some 
mental  act  that  otherwise  would  be  without  emotion.  Freud  thus 
explains  the  existence  of  morbid  fears,  that  is  the  existence  of  a  fear 
connected  with  some  act  which  normally  would  go  on  without  fear. 
If  this  complex  energy  with  its  emotion  is  diverted  into  a  physical 
channel,  Freud  terms  the  process  "  conversion  "  and  believes  that  it  is 
the  basis  for  many  hysterical  physical  symptoms,  such  as  spasms,  con- 
vulsions or  paralysis  and  anaesthesia.  This  hypothesis  of  "  conversion  " 
seems  unreasonable,  forced  and  artificial.  He  holds  that  every  case 
of  hysteria  is  the  result  of  some  preceding  psychic  trauma ;  that  such 
a  trauma  may  be  produced  by  any  experience  which  produces  fear, 
anxiety  or  shame.  And  in  his  opinion  such  experiences  are  always  in 
the  sexual  sphere. 


S90  HysteMA. 

The  treatment  of  hysteria  which  follows  from  this  theory  is  neces^ 
sarily  a  psychic  treatment.  The  object  is  to  stop  the  mental  processes 
of  censure  and  repression  by  recalling  to  consciousness  the  experiences 
which  have  produced  the  psychic  trauma,  for  he  claims  that  when  these 
are  consciously  considered  and  confessed  the  confession  is  attended  by 
an  emotional  outburst  which  relieves  the  tension  under  which  the  mind 
labors.  To  elicit  this  confession  may  require  hours  of  laborious  self- 
examination  under  cross-examination — a  process  he  terms  "  catharsis." 
He  affirms  that  when  this  is  complete  the  symptoms  disappear. 

There  are  elements  of  truth  in  this  hypothesis  and  there  are  many 
errors,  hence  a  criticism  must  be  offered.  It  is  admitted  that  mental 
conflicts  between  natural  impulses  and  moral  ideas  as  acquired  by  edu- 
cation go  on  all  the  time  in  every  mind.  At  times  these  cause  very 
intense  emotions  and  stress  of  mind,  and  frequently  produce  neurotic, 
psychasthenic  and  hysterical  symptoms  by  apparently  arresting  normal 
inhibition,  the  susceptibility  to  suggestion  being  increased  thereby. 
That  all  or  even  the  majority  of  such  mental  conflicts  originate  in  the 
sexual  sphere  experience  contradicts.  For  the  most  fundamental 
mental  comjDlex  or  instinct  is  that  of  self-preservation,  rather  than  of 
reproduction.  And  the  modern  American  is  much  more  concerned 
with  success  in  business,  society,  profession  or  politics,  etc.,  than  in 
his  sexual  life,  though  this  may  not  be  true  in  the  country  where  this 
hypothesis  originated.  Nor  do  I  believe  that  sexual  ideas  exert  much 
influence  before  the  age  of  puberty,  while  the  idea  that  the  child's  love 
for  its  parents  is  based  on  sexual  feeling  is  contrary  to  general  accep- 
tation and  is  an  offensive  notion ;  according  to  it  a  child  should  show 
aifection  for  the  parent  of  the  opposite  sex  only  —  which  is  not  true. 

Another  objection  to  the  hypothesis  is  the  method  of  treatment.  In 
the  search  for  forgotten  shameful  sexual  experiences  it  is  unavoidable 
that  a  set  of  immoral  ideas  should  be  installed  or  if  latent  awakened 
and  made  a  subject  of  thought  and  discussion.  I  kno\y  of  patients 
who  have  been  turned  into  confirmed  hypochondriacs  by  self-examina- 
tion. Freud  himself  says  that  the  treatment  is  applicable  only  to  a 
small  fraction  of  hysterical  patients,  those  of  the  higher  intelligence, 
with  no  trace  of  weakmindedness,  and  those  with  whom  the  physician 
is  in  intimate  sympathy.  But  hysteria  is  not  common  among  those  of 
strong  mind  or  high  intelligence.^  Hence  its  scope  is  very  limited,  as 
the  majority  of  hysterical  patients  are  not  open  to  it  at  all.  Nor  is  its 
success  in  the  few  cases  a  proof  of  the  hypothesis.  It  is  really  an 
adaptation  of  the  Catholic  confessional,  which  is  more  successful  in 
relieving  mental  stress  and  is  open  to  all  classes,  but  the  evils  of  Avhich 
have  been  long  recognized.  It  is  actually  a  method  of  suggestion,  like 
Christian  Science  and  many  other  processes,  in  which  an  intimate  rela- 
tion of  a  confidential  kind  is  established  between  the  patient  and  the 

^  "  I  could  not  conceive  myself  entering  deeply  into  the  psychic  mechanism  of  a 
hysteria  in  a  person  who  appears  to  me  common  or  disagreeable  and  who  would  not  be 
able  to  awaken  in  me  human  sympathy.  The  process  is  especially  inapplicable  below 
a  certain  niveau  of  intelligence.  It  is  rendered  difficult  wherever  there  is  the  least  tinge 
of  weakmindedness."     Hysteria  and  the  Psychoneuroses,  p.  84. 


ETIOLOGT.  891 

healer  untij  the  latter  is  able  by  personal  influence  to  accomplish  almost 
any  result  by  decided  commands  or  direct  persuasion. 

The  treatment  then  does  not  differ  essentially  from  other  methods 
of  successful  suggestive  treatment,  as  it  confessedly  depends  on  an 
intimate  sympathy  between  patient  and  physician,  but  is  objectionable 
on  account  of  its  character.  And  furthermore  it  has  to  be  admitted  by 
Freud  himself  that  a  certain  proportion  of  his  patients  leave  him  in 
disgust  without  benefit,  while  others  have  required  years  of  treatment 
to  reach  a  cure.  The  criticism  is  inevitable  that  the  method  has  a  very 
limited  scope  and  has  many  objectionable  features  not  shared  by  the 
direct  persuasion  of  Babinsky  or  the  hypnosis  of  Janet.^ 

Etiology.  —  The  disease  is  much  more  common  among  females  than 
among  males.  The  age  of  maximum  liability  is  from  twelve  to  thirty, 
but  children  are  often  affected  and  women  may  develop  hysteria  at  any 
age.  Next  to  the  time  of  puberty,  the  time  of  the  menopause  is  the 
period  in  which  the  symptoms  most  commonly  appear.  The  Hebrew 
race  is  peculiarly  liable  to  develop  the  disease.  The  Latin  races  are 
more  susceptible  than  the  Teutonic.  The  disease  is  rarely  seen  in  its 
extreme  forms  in  this  country,  but  appears  to  be  increasing  in  fre- 
quency in  cities  under  the  stress  of  modern  life. 

Heredity  is  the  most  important  factor  in  its  causation.  A  nervous, 
hysterical  mother,  or  an  alcoholic  father,  are  particularly  liable  to  pro- 
duce hysterical  children.  Blood  relation  between  parents  is  often  the 
cause  of  hysteria  in  the  progeny.  Exhausting  disease  of  long  stand- 
ing in  the  parents  is  a  factor.  Charcot  held  that  every  case  was  based 
on  a  bad  heredity. 

Mental  or  emotional  shock  is  the  chief  exciting  cause.  Such  shocks 
come  to  every  one,  but  in  those  who  are  predisposed  to  hysteria  they 
have  a  surprising  effect,  often  starting  a  long  train  of  symptoms  which 
may  last  a  lifetime.  Thus  the  fright  occasioned  by  a  railway  or  auto- 
mobile accident,  by  a  stroke  of  lightning,  by  a  sudden  fall,  maybe  fol- 
lowed by  hysterical  symptoms  either  at  once  or  after  an  interval  of 
some  days.  Another  cause  is  long  continued  anxiety  or  care.  This 
acts  like  a  series  of  little  shocks  continued  with  a  summation  of  effect 
under  which,  as  under  a  great  shock,  the  weak  nervous  system  gives 
way.  Occupations  which  involve  tense  mental  strain,  the  life  of  an 
engineer  on  a  locomotive,  that  of  a  broker  on  the  stock  exchange,  that 
of  a  responsible  leader  of  politics,  or  banking,  or  any  profession  or 
business,  may  lead  to  nervous  exhaustion,  which  shows  itself  in  hys- 
terical symptoms.  It  appears  to  be  the  worry  and  emotional  strain 
rather  than  the  work  itself,  which  induces  the  collapse. 

Any  exhausting  disease  long  continued,  which  saps  the  vital  energy, 
especially  diseases  of  the  genital  organs  which  involve  much  anxiety 
over  the  potency  of  the  indivi<lual,  may  produce  hysteria.     Masturba- 

»The  reaxier  is  referred  to  articles  in  Brain,  Jan.,  1911,  by  Ormerod  and  by  Wilson 
on  Modem  C(jnceptions  of  Hysteria,  and  by  Hart  on  Freud's  Conception  of  Hysteria. 
Also  to  II.  S.  IMunro,  .Suf,'Kestive  Tliera|)euticH  and  Hypnotism,  1!»12,  and  toBondurant, 
N.  Y.  Med.  Record,  March  U,  1912.  In  Germany  Freud's  theories  are  condennied  by 
Striimixjll,  Anton  and  others.     See  Arch,  fiir  Psych.,  Bd.  xlix.,  5,  G52,  1912. 


892  HYSTEHIA. 

tion  and  the  consequent  seminal  emissions  and  fear  of  Impotence  in 
boys,  or  uterine  and  ovarian  diseases  in  women  who  live  without  sex- 
ual gratification  or  in  married  women  who  fear  sterility,  are  frequent 
causes  of  hysteria.  Excessive  sexual  indulgence  in  either  sex  may  in- 
duce it.  The  old  idea  that  the  disease  is  of  uterine  origin  is,  however, 
a  mistaken  one.  I  have  never  seen  much  benefit  in  hysterical  cases 
from  operations  upon  the  uterus  or  ovaries. 

In  young  people  the  tendency  to  imitation  is  strongly  developed, 
and  one  hysterical  child  in  a  school  or  convent  is  often  the  cause  of  an 
outbreak  of  hysterical  symptoms  in  many  others.  Epidemics  of  hys- 
teria, so  common  in  the  middle  ages,  are  now  infrequent  because  the 
necessity  of  seclusion  of  such  patients  is  widely  appreciated. 

I  have  seen  hysteria  develop  after  repeated  hypnosis,  either  by  auto- 
suggestion, or  by  direct  suggestion.  It  may  also  develop  after  anaes- 
thesia from  chloroform  or  ether  in  the  same  way. 

It  is  said  to  follow  chronic  intoxication  by  alcohol,  lead,  opium, 
mercury,  coal  gas,  and  by  the  products  of  the  infectious  diseases. 

Prognosis.  —  The  prognosis  is  always  a  good  one,  so  far  as  the 
recovery  from  any  form  of  hysterial  affection  goes.  The  symptoms 
invariably  pass  away  either  after  proper  treatment,  or  spontaneously  in 
time,  even  though  they  may  last  for  many  years.  It  is  to  be  remem- 
bered, however,  that  the  hysterical  temperament  remains  through  life, 
and  hence  the  prognosis  as  to  recurrence  of  similar  attacks,  or  of  the 
appearance  of  other  forms  than  the  one  first  shown,  is  a  bad  one. 

Diagnosis.  —  This  is  usually  made  without  any  difficulty  when  the 
history  of  the  individual,  especially  her  heredity  and  temperament,  is 
fully  understood.  The  diagnosis  is  always  to  be  reached  by  excluding 
every  form  of  organic  disease  which  might  cause  analogous  symptoms ; 
by  the  facts  that  hysterical  never  wholly  resemble  organic  affections, 
and  that  they  always  present  peculiar  features  which  serve  to  distin- 
guish them.  Thus  the  distribution  of  anaesthesia  and  of  pain,  or  the 
character  and  distribution  of  paralysis  are  always  typical  and  are  easily 
distinguished  from  those  due  to  nerve,  cord  or  brain  lesions.  The 
only  disease  which  produces  symptoms  that  cannot  be  differentiated 
from  hysteria  is  multiple  sclerosis.  But  when  the  physical  signs  of 
that  affection  appear,  nystagmus,  intentional  tremor,  and  scanning 
speech,  no  doubt  remains.  Until  they  do  appear  no  diagnosis  is  pos- 
sible. The  variability  of  symptoms  in  hysteria,  their  great  change  in 
degree  or  extent  from  day  to  day,  are  to  be  remembered  as  inconsistent 
with  lesions  of  the  nervous  system.  The  particular  points  of  differen- 
tiation have  been  already  alluded  to  in  discussing  the  symptoms. 

Treatment.  —  The  key  to  success  in  the  treatment  of  hysteria  is 
the  susceptibility  of  these  patients  to  suggestion.  If  the  physician 
obtains  the  confidence  of  the  patient,  and  has  the  power  to  impress 
upon  her  the  necessity  of  obeying  his  commands,  and  the  expectation 
of  definite  results,  he  will  succeed  in  his  efforts  to  instil  a  more  health- 
ful mental  state  and  to  relieve  special  symptoms.  If  he  is  met  by  a 
spirit  of  opposition  or  of  doubt,  his  efforts  will  be  useless. 


TBEATMENT.  893 

It  is  neeessary  as  a  rule  to  produce  an  entire  ehange  in  the  mental 
attitude  and  occupation  of  these  patients.  If  they  can  be  taken  away 
from  home  and  from  their  ordinary  surroundings  and  associates,  if  they 
can  be  given  rest  of  body  and  of  mind,  and  especially  if  anxiety  and 
care  can  be  removed,  time  alone  will  cure  them.  In  mild  cases,  there- 
fore, travel  without  fatigue,  with  a  cheerful  companion,  and  constant 
mental  occupation  of  a  pleasant  kind,  are  to  be  recommended.  In 
severe  cases  the  Weir  Mitchell  rest  cure  is  to  be  enforced.  This 
involves  separation  from  friends ;  the  companionship  of  doctor  and 
nurses  who  are  not  foolishly  sympathetic  and  cannot  be  alarmed  by 
symptoms  ;  a  relief  from  responsibility  and  dependence  upon  a  person 
who  can  be  trusted.  The  person  acquires  a  faith  in  the  physician,  and 
then  his  persuasion  begins  to  have  an  effect  and  improvement  and  cure 
follow. 

The  means  used  by  the  physician  to  enforce  and  aid  these  suggestions 
should  be  those  devised  by  Weir  Mitchell  to  improve  nutrition.  A 
regular  systematic  regime  with  extra  feeding,  with  massage  to  assist 
assimilation,  with  exercises,  water  treatment  and  electrical  applications 
to  occupy  the  time  and  to  keep  the  mind  of  the  patient  busy,  are  the 
methods  employed.  Weir  Mitchell  insists  upon  absolute  rest  in  bed 
for  four  or  six  weeks,  forced  feeding  by  milk  and  simple  food  every 
two  hours,  massage  and  electricity  to  give  the  exercise  needed  for  the 
assimilation  of  food  and  absolute  seclusion.  In  many  severe  cases  this 
method  is  the  only  one  which  will  be  successful.  It  must  however  be 
carried  out  with  the  full  consent  and  cooperation  of  the  patient,  by  a 
physician  in  whom  she  has  confidence,  and  with  the  aid  of  nurses  whose 
personality  is  both  agreeable  and  inspiring.  If  these  last  named  factors 
do  not  enter  into  the  rest  cure  it  usually  fails. 

In  mild  cases  a  less  rigorous  regime  is  to  be  employed  which  com- 
bines rest  with  occupation  and  diversion,  and  involves  water  treatment 
in  place  of  electricity.     The  following  regime  is  an  example  : 

8  A.  M.  Small  cup  of  coffee  with  hot  milk ;  or  black  coffee  if 
preferred.     Hunyadi  if  needed. 

8.15  A.  M.     Bathe  hands  and  face  and  brush  teeth. 

8.30-9  A.  M.  Breakfast :  fruit,  cereal  with  cream,  eggs,  bacon  or 
fish,  hot  milk  or  cocoa. 

9-10  A.  M.  Quiet  and  rest ;  letters  read  by  nurse  or  patient  after 
9.30  ;  glass  of  water. 

10.30-11  A.  M.  Bath:  large  bath  towel  under  patient;  blanket 
over  patient.  Nurse  to  bathe  each  part  with  soap  and  tepid  water ;  or 
give  patient  a  salt  rub  (1)  ;  or  pack  (2)  in  place  of  bath,  with  salt 
water  affusion. 

11-11.30  A.  M.  Glass  of  milk,  or  kumyss,  or  hot  broth,  or  cocoa. 
Rest. 

11.30  A.  M.     Get  up  and  dress  for  the  day. 

12  Noon.     Go  out  for  drive  or  walk. 

1.30  P.  M.  Lunch  :  soup  ;  steak  or  chops  with  vegetables  ;  salad  ; 
baked  apple  or  fruit. 


894  EYSTEBIA. 

2-3  P.  M.  Rest  quietly,  lying  down,  relaxed  but  not  undressed  ; 
glass  of  water. 

3-4  P.  M.  Go  out  for  walk  or  drive,  or  see  friends.  Glass  of 
milk,  or  beef-tea.     Undress. 

6  P.  M.     Massage  (3),  or  pack  (2),  or  physical  culture  exercises  (4). 

6-6.30  P.  M.     Rest  alone,  lying  down. 

6.30  P.  M.     Dress  for  dinner  ;  glass  of  water. 

7-8  P.  M.  Dinner :  oysters,  soup,  fish,  game  or  chicken,  vege- 
tables of  any  kind,  salad,  cheese  or  fruit.     No  wine,  no  coffee. 

8-8.30  P.  M.     Rest. 

8.30-10  P.  M.     Reading,  or  games. 

10  P.  M.  Bed,  preceded  by  spinal  douche  (5),  or  drip  sheet  (6). 
Cascara  tablet,  5  minims  of  fluid  extract.  Glass  of  hot  milk  without 
or  with  trional  if  needed. 

The  various  means  mentioned  may  be  described  as  follows  : 

1.  Salt  Rub.  —  Coarse  bath  towel  soaked  in  brine,  (1  to  20  solution 
of  Dittman's  sea  salt)  and  allowed  to  drip,  then  rolled  and  kept 
twelve  hours,  but  not  dried.  Rub  with  this  salt  damp  towel,  wet 
with  hot  water. 

2.  Paek.  —  Wrap  patient  in  wet  sheet,  temperature  85°  ;  cover  this 
with  three  dry  blankets;  put  wet  towel,  95°,  to  forehead;  hot 
bottle  to  feet.  Lie  thus  for  twenty  minutes.  Followed  by  affusion 
all  over  with  water,  temperature  75°,  followed  by  brisk  rub  with 
hot  towel. 

3.  Massage.  —  To  be  given  alone  during  the  first  two  weeks,  the  force 
of  massage  being  increased  daily,  and  after  two  weeks  followed  by 
Swedish  movements,  exercises  against  resistance. 

4.  Exercises.  —  These  are  to  consist  of  flexion  and  extension  of  the 
arms  and  legs  while  standing,  also  of  rising  from  a  sitting  posture, 
also  of  stooping  forward,  backward,  and  turning  laterally,  and  fin- 
ally, of  respiratory  movements  deep  and  forcible.  Five  such  move- 
ments of  each  kind  to  begin  with,  and  each  day  the  number 
increased. 

5.  Spinal  Douche.  —  The  back  to  be  sponged  with  very  hot  water, 
from  the  line  of  the  shoulders  to  the  tip  of  the  spine,  while  sitting 
on  the  edge  of  the  bath-tub,  temperature  of  water  102°  to  105°,  for 
three  minutes,  followed  by  one  affusion  from  a  pitcher  of  cold  water 
at  temperature  of  75°,  and  quick  rub  down  with  hot  towel. 

6.  Drip  Sheet.  —  Stand  in  bath-tub  in  hot  water  up  to  ankles,  have 
sheet  wet  in  water,  temperature  80°,  thrown  about  body  and  brisk 
friction  made  with  hands  over  the  sheet  for  one  minute,  followed 
by  a  brish  rub  with  hot  towels. 

In  many  cases,  indigestion,  imperfect  nutrition,  and  emaciation  have 
been  active  factors  in  producing  the  symptoms,  and  when  these  have 
been  cured  the  symptoms  disappear.  In  some  cases  nutrition  is  per- 
fect, and  in  these  it  is  the  mental  influence  rather  than  the  medical 
treatment  which  does  the  good.  In  fact  I  believe  that  the  rest  cure 
is  successful  in  those  cases  only  where  the  personality  of  the  doctor  is 


TREATMENT.  895 

able  to  impress  and  control  the  patient.  Hence  the  success  of  Weir 
Mitchell  and  Playfair,  and  the  failure  of  their  methods  in  other  hands. 
Hysterical  patients  respond  promptly  to  any  method  which  excites 
their  expectation  of  relief  and  which  suggests  a  cure.  Hence  charla- 
tans of  all  sorts,  mental  healers,  "  Christian  Scientists "  (who  are 
neither  Christians  nor  scientific),  osteopaths,  often  succeed  when  phy- 
sicians fail.  The  success  of  suggestive  treatment  in  the  hands  of  the 
clergy  has  been  proven  at  the  Emmanuel  Church  in  Boston  and  else- 
where. It  is  the  personality  of  the  leaders  in  this  medico-religious 
movement  which  accounts  for  its  success.  In  feebler  hands  similar 
methods  fail. 

Hypnotic  suggestion  is  of  great  use  in  the  treatment  of  hysterical 
symptoms.  In  the  dreamy  state  produced  by  hypnosis  the  persuasion 
of  the  physician  is  accepted  without  question ;  his  positive  assertion 
that  this  or  that  symptom  is  passing  away  and  will  not  return  when 
the  patient  awakes  carries  a  double  conviction,  and  remains  as  a  sub- 
conscious persuasion  and  the  result  is  the  disappearance  of  the  malady 
for  a  time.  Bepeated  treatments  are  needed  for  a  permanent  cure,  the 
intervals  between  them  being  gradually  increased,  and  the  persuasion 
should  be  enforced  each  time  that  the  symptoms  will  remain  away  for 
a  longer  period.  Hypnotic  treatment  is  not  harmful  unless  the  idea 
of  dependence  upon  the  hypnotizer  is  awakened  in  the  patient  by  per- 
suasion in  hypnosis.  The  hypnotizer  should  not  fail  to  persuade 
the  patient  of  her  own  capacity  to  control  herself  and  of  the  perma- 
nence of  the  cure. 

The  remarkable  results  achieved  by  hypnotic  suggestion  are  equalled 
by  the  miracles  of  Lourdes  and  of  St.  Anne  de  Beaupr^.  Messner 
and  Braid  succeeded  in  curing  by  inducing  a  firm  expectation  of 
relief,  though  the  means  which  they  employed  were  not  really  what 
they  claimed.  The  same  thing  is  true  to-day  of  the  many  patent 
remedies  widely  advertised.  The  advertisement,  and  not  the  remedy, 
cures  the  patient,  for  it  awakens  her  belief  and  expectation,  and  sug- 
gestion does  the  rest.  These  facts  are  not  to  be  neglected  by  a  phy- 
sician in  treating  hysterical  patients.  Positive  statements  regarding 
the  effect  to  be  produced  by  a  remedy  should  always  be  made,  and 
should  be  reiterated.  Active  measures,  whether  electrical  applications, 
water  treatment,  counter-irritation,  cupping,  massage,  should  be  accom- 
panied by  frequent  assurance  that  they  will  relieve  the  symptom  pres- 
ent, and  when  a  positive  belief  is  developed  the  result  will  follow. 
In  the  employment  of  drugs,  the  same  is  true.  It  matters  little  what 
drug  is  used,  or  in  what  strength,  for  it  is  the  mental  rather  than  the 
physiological  effect  which  is  desired.  For  this  reason  it  is  far  better 
to  give  remedies  in  very  small  doses,  but  very  frequently  repeated,  in 
hysterical  conditions.  For  the  repeated  suggestion  every  five  or  ten 
minutes  that  something  is  being  done  to  give  relief  will  do  more  than 
a  single  dose  given  at  long  intervals.  And  it  is  an  extraordinary  fact 
that  in  acute  hystcTical  states  drugs  are  not  readily  absorbc^l  by  the 
stomach  until  the  attac^k  is  over.     Thus  I  have  seen  no  eifect  what- 


896  HYSTEBIA. 

ever  from  large  doses  of  bromides  given  during  a  convulsive  attack 
but  a  cumulative  effect  the  next  day.  On  the  other  hand,  I  have  seen 
immediate  effects  from  very  small  doses  of  some  pungent  but  inert 
remedy  absorbed  in  the  mouth,  when  enforced  by  positive  statements 
that  it  will  cure. 

The  only  explanation  of  these  facts  is  that  hysterical  symptoms  are 
wholly  mental  and  must  be  met  by  mental  suggestion  rather  than  by 
physical  remedies.  It  is  for  this  reason  probably  that  counter-irrita- 
tion is  of  much  service,  especially  if  the  actual  cautery  is  employed. 
The  relief  of  pain  from  slight  applications  is  often  instantaneous. 
Static  electricity  is  also  of  much  use. 

The  effect  of  remedies  calculated  to  increase  the  vascular  tone  and 
to  stimulate  the  circulation  is  usually  of  use  in  hysterical  conditions. 
Thus  strychnine,  digitalis  and  ergot  are  often  of  much  apparent  benefit. 
The  relaxed  arterial  tension  and  the  ansemic  state  of  the  surface  of  the 
body  have  suggested  the  theory  that  in  some  cases  a  general  relaxation 
of  the  large  abdominal  veins  in  the  domain  of  the  splanchnic  nerves, 
and  a  consequent  anaemia  of  the  arterial  system,  is  a  factor  in  the  pro- 
duction of  hysterical  states.  If  this  theory  is  correct,  the  benefit  of 
vascular  stimulants  is  easily  explained.  It  is  certain  that  cold  water 
treatment,  which  is  usually  helpful,  has  a  tonic  effect  on  the  circula- 
tion. And  all  hysterical  patients  admit  that  a  cold  shower  bath  makes 
them  feel  much  better. 

All  cases  are  not  benefited  by  the  rest  cure.  There  are  many  hys- 
terical persons  for  whom  active  exercise  in  the  open  air  is  of  more  use 
than  rest,  and  after  a  rest  cure  such  exercises  are  to  be  commended. 

The  remedies  usually  employed  in  hysteria  are  asafoetida,  valerian, 
valerianate  of  ammonia,  or  of  zinc,  simulo,  turpentine,  or  the  bromides. 
They  may  be  prescribed  in  small  doses  frequently  repeated  during  an 
attack,  but  are  not  to  be  continued  long.  And  it  is  important  that 
hysterical  patients  should  not  be  encouraged  to  rely  upon  drugs,  but 
rather  upon  external  remedies. 

In  hysterical  attacks  an  application  of  cold  water  to  the  face,  the 
inhalation  of  ammonia  or  of  amyl  nitrite,  or  a  drink  of  spirits  will 
usually  stop  the  attack.  In  case  it  goes  on  to  a  convulsion,  counter- 
irritation  by  mustard  leaves  may  be  used  or  ice  may  be  applied  to  the 
back.  In  the  severe  cases  a  hypodermic  of  apomorphine  -^^  grain  will 
cause  emesis  and  stop  the  attack. 

I  know  of  no  remedy  for  hysterical  pain,  and  strongly  deprecate  the 
use  of  morphine,  which  only  has  an  effect  when  given  in  narcotic 
doses,  and  even  then  gives  temporary  relief  only.  The  ordinary  anal- 
gesics may  be  tried,  but  are  rarely  of  service.  Ignatia  in  minute 
dose  yqVo"  grain  repeated  frequently  sometimes  gives  relief. 

For  hysterical  anaesthesia,  the  application  of  the  faradic  brush  or  of 
static  electricity  is  often  useful.  If  the  application  of  metal  discs  to 
the  surface  restores  sensation,  this  may  be  done  to  various  parts  of  the 
limbs. 

For  hysterical  paralysis,  the  application  of  strong  faradic  currents 


TBEATMENT.  897 

which  movfe  the  limbs,  accompanied  by  the  encouragement  that  so 
long  as  the  muscle  acts  to  faradism  voluntary  effort  may  move  it,  will 
usually  be  successful.  Immediate  cure  is  not  to  be  hoped  for  but 
daily  improvement. 

For  hysterical  spasms  and  contractions  massage  and  long  continued 
hot  baths,  followed  by  cold  effusions  are  the  best  remedies. 

The  vasomotor  symptoms  are  to  be  combated  by  the  use  of  strych- 
nine in  small  doses  frequently  repeated. 

There  is  no  disease  in  which  the  ingenuity  of  the  physician  is  more 
constantly  called  into  play  than  in  hysteria.  To  devise  new  means 
which  are  legitimate  but  harmless,  and  to  instil  a  faith  in  their  effect 
is  his  duty. 


57 


CHAPTER  LIII. 

VERTIGO. 

Vertigo  is  such  a  common  symptom  that  it  is  well  to  classify  its 
causes  in  order  to  reach  a  diagnosis. 

I.  Vertigo  from  affections  of  the  auditory  apparatus. 

1.  Vertigo  from  disease  in  the  outer  and  middle  ear.  This  form  is 
moderate,  is  attended  by  some  staggering  or  inability  to  stand,  and  by 
roaring  in  the  ear  and  deafness.  Any  disease  of  the  ear  may  cause  this 
type  of  vertigo  in  greater  or  less  degree.  Thus  a  plug  of  wax  or  any 
irritation  in  the  ear  from  sea  water,  picking  the  ear,  or  boils  in  the 
auditory  canal  may  produce  giddiness.  Such  affections  are  visible. 
Any  form  of  otitis  media  is  attended  by  vertigo,  which  may  be  one  of 
the  most  distressing  symptoms.  It  is  attended  by  pain  in  the  ear,  by 
changes  in  the  appearance  of  the  drum  membrane,  and  eventually  by  a 
discharge  of  pus.     It  is  somewhat  relieved  by  inflation. 

Treatment  should  be  directed  to  the  ear  disease. 

2.  Vertigo  from  disease  of  the  inner  ear — Meniere's  disease.  This 
form  is  very  intense  and  distressing,  begins  with  a  loud  report  in  the 
ear,  is  attended  by  prostration,  inability  to  stand  and  pallor,  coldness 
and  sweating,  and  vomiting,  and  is  always  associated  with  tinnitus  and 
deafness.  There  may  be  nystagmus,  great  mental  distress,  and  a  loss 
of  consciousness.  It  may  come  on  suddenly,  and  then  constitutes 
the  symptom-complex  named  after  Meniere,  who  first  described  it. 
Meniere's  disease  is  due  to  hemorrhage  in  the  semilunar  canals,  and 
comes  like  an  apoplectic  stroke,  but  is  not  accompanied  or  followed 
by  paralysis.  It  may  come  upon  a  person  in  perfect  health,  or  it  may 
be  preceded  by  ear  disease  or  by  arterio-sclerosis.  If  the  attack  is 
recovered  from  it  is  usually  followed  by  permanent  deafness  and  by 
other  attacks,  and  any  exertion  or  mental  excitement  may  bring  on  an 
attack.  The  patients  dread  such  attacks  and  live  in  terror  of  them. 
The  attacks,  at  first  lasting  a  few  minutes  only,  become  more  and  more 
frequent  and  severe,  as  a  rule,  and  finally  the  patient  suffers  from  con- 
stant dizziness,  becomes  extremely  nervous  and  apprehensive,  and  dies 
in  an  attack.  Occasionally  the  first  attack  is  not  followed  by  recur- 
rence and  all  symptoms  excepting  deafness  pass  off. 

The  treatment  of  vestibular  vertigo  as  a  symptom  is  by  rest  in  bed, 
applications  of  ice-packs  to  the  ear  or  blisters  behind  the  ear,  and  the 
free  use  of  bromide,  chloral,  and  nerve  sedatives,  or,  in  case  these  fail 
to  relieve,  by  hypodermic  injections  of  morphine. 

Babinski  and  Putnam^  have  recently  advocated  lumbar  puncture  in 

1  Jour,  of  Nerv.  and  Ment.  Dis.,  Jan.,  1908. 


VERTIGO.  899 

the  treatment  of  aural  vertigo  and  report  success.  From  10  to  15  c.c. 
of  spinal  fluid  is  withdrawn  and  after  a  week  this  is  to  be  repeated  if 
necessary. 

Voltolini  has  described  an  aflFection  which  he  considers  due  to  pri- 
mary inflammation  of  the  labyrinth.  This  is  known  by  his  name. 
The  disease  occurs  in  children,  but  adults  are  not  exempt.  It  resem- 
bles acute  meningitis.  It  begins  suddenly  with  high  fever,  general 
cerebral  symptoms,  of  which  vertigo  and  vomiting  are  the  most  promi- 
nent, and  consciousness  soon  becomes  obscured.  After  a  few  days 
these  symptoms  subside,  but  the  child  still  suifers  from  dizziness, 
staggers  in  walking,  and  is  found  to  be  deaf.  The  staggering  grad- 
ually passes  off,  but  some  degree  of  deafness  remains.  The  lesions 
found  have  been  plastic  exudations  with  destructive  processes  in  the 
semilunar  canals.  It  is  practically  impossible  to  differentiate  Meniere's 
disease  from  Voltolini's  disease  in  the  early  stage,  and  even  in  the  later 
stage  it  is  very  difficult.  In  M^niSre's  disease  the  attacks  of  vertigo 
recur.  The  treatment  should  be  by  counter-irritation  behind  the  ears, 
hot  baths,  antifebrile  remedies,  purgatives,  and  bromides  in  large 
doses. 

3.  Vertigo  from  disease  in  the  vestibular  nerve  is  attended  by 
symptoms  quite  like  those  in  Meniere's  disease,  but  is  often  accom- 
panied by  forced  movements  or  by  staggering  or  falling  in  some  defi- 
nite direction. 

Thus  in  a  case  of  a  fireman  seen  at  the  New  York  Hospital,  who 
by  a  fall  had  fractured  the  base  of  his  skull  and  torn  the  left  auditory 
nerve,  the  most  marked  symptoms  during  the  three  days  preceding 
his  death  were  an  absolute  deafness  in  the  left  ear,  a  constant  agon- 
izing sensation  of  rotation  of  the  body  in  its  longitudinal  axis,  and 
consequently  a  constant  turning  of  the  body  in  the  opposite  direction 
in  the  endeavor  to  correct  the  subjective  sensation.  Unless  firmly 
held  this  man  constantly  revolved  upon  his  bed,  and  when  held  com- 
plained of  the  most  intense  distress.  In  this  case  the  vertigo  was 
attended  by  extreme  exhaustion,  vomiting,  utter  prostration,  rapid 
pulse,  and  death  from  heart  failure. 

Raymond  ^  in  his  lectures  has  described  similar  cases. 

4.  Vertigo  is  a  frequent  symptom  in  disease  of  the  pons  Varolii. 
Tumors  upon  the  base  of  the  brain,  syphilitic  exudations  or  vascular 
lesions  in  the  pons,  and  abscess  or  tumors  in  the  cerebellum  compres- 
sing the  pons  irritate  or  destroy  the  vestibular  nerve  or  its  nuclei.  In 
these  cases  the  vertigo  is  usually  mild  in  degree.  It  is  accompanied 
by  a  staggering  gait,  the  tendency  being  to  stagger  to  one  side,  in  the 
majority  of  the  cases,  but  not  invariably,  toward  the  side  of  the  lesion. 
The  existence  of  other  general  symptoms  of  tumor,  abscess,  syphilis 
of  the  brain,  sclerosis,  or  vascular  lesicms,  and  the  presence  of  other 
cranial  nerve  symptoms  enable  a  diagnosis  to  be  reached.  Such  a 
tumor  is  shown  in  Fig.  240,  page  575. 

II.   Vertigo  from  affections  of  the  ocular  apparatus. 

'  Clinique  des  MaladicH  du  Syst6me  Nerveux,  1900,  IVifirae  s^rie. 


900  VERTIGO. 

Any  sudden  attack  of  diplopia  due  to  paralysis  of  an  ocular  muscle 
is  liable  to  be  attended  by  vertigo.  Thus  lesions  of  the  third  or  sixth 
nerve  or  ophthalmoplegia  externa  produce  giddiness  and  cause  uncer- 
tainty of  gait.  This  form  of  vertigo  is  often  attended  by  nystagmus. 
It  ceases  when  the  eyes  are  closed  and  when  the  patient  does  not  use 
the  affected  eye. 

III.  Vei'tigo  from  affections  of  the  muscular  sense. 

While  staggering  gait  is  a  frequent  symptom  in  locomotor  ataxia, 
vertigo  is  less  constant.  It  occurs,  however,  with  such  frequency  as 
to  require  mention.  Charcot  pointed  out  that  it  develops  in  those 
cases  whose  general  sensibility  and  muscular  sense  are  profoundly 
aifected.  It  may  be  due  in  some  ataxics  to  a  primary  atrophy  of  the 
vestibular  nerve. 

TV.  In  the  three  types  of  vertigo  thus  far  studied  it  is  evident  that 
the  lesion  interferes  with  the  conduction  to  the  cerebellum  of  those 
impulses  which  are  necessary  to  an  appreciation  of  one's  position  in 
space.  Lesions  in  the  cerebellum  itself,  the  organ  of  equilibrium,  are 
almost  always  attended  by  vertigo.  This  is  particularly  true  of  lesions 
of  the  vermis  or  central  lobe  and  by  lesions  of  the  flocculus,  the  small 
polyp-like  lobes  on  the  base.  It  is  thought  that  lesions  of  the  vermis 
in  its  anterior  part  cause  staggering  forward,  and  in  its  posterior  part, 
backward.  Such  staggering  is  due  to  a  sensation  of  falling  in  the 
opposite  direction.  Any  form  of  lesion  may  cause  this  symptom,  but- 
it  is  particularly  constant  in  tumors  of  the  cerebellum.  The  diagnosis 
can  be  reached  only  by  observing  the  general  signs  of  cerebellar 
disease  in  addition  to  vertigo.  In  congenital  defective  development  of 
the  cerebellum,  vertigo,  nystagmus,  and  staggering  appear  early.  In 
Marie's  disease  they  are  permanent  symptoms. 

Neurasthenia  and  hysteria  often  produce  vertigo  of  a  mild  and 
transient  variety,  which  is  probably  cerebellar.  The  so-called  "  essen- 
tial vertigo,"  consisting  of  attacks  of  vertigo  with  no  other  symptoms, 
is  probably  a  functional  neurosis  of  the  cerebellum,  and  occurs  in 
attacks  quite  like  epileptic  attacks,  and  is  usually  to  be  treated  as  a 
form  of  epilepsy.  Epileptic  attacks  are  almost  uniformly  preceded  or 
attended  by  vertigo. 

Gertier,  of  Geneva,  has  described  a  peculiar  disease,  which  has  been 
seen  in  Switzerland  and  France,  and  is  known  as  Gertier's  disease  or 
vertige  paralysant.  It  also  occurs  in  Japan,  where  it  is  known  as 
kubisagari.  It  is  characterized  by  a  series  of  sudden,  short  attacks  of 
vertigo,  with  ptosis  and  somnolence,  pain  in  the  neck,  with  paralysis 
of  the  neck  muscles,  allowing  the  head  to  fall  forward,  or  general 
weakness  or  even  general  paralysis,  with  thickness  of  speech  but  no 
loss  of  consciousness.  The  attacks  last  a  few  minutes,  recur  every 
few  minutes  for  several  hours,  leaving  the  patient  fatigued  but  well  in 
the  interval.  They  increase  in  frequency  during  the  hot  weather,  but 
cease  in  winter.  The  disease  lasts  from  one  to  five  months.  In  the 
intervals  the  patients  appear  to  be  in  good  health.  Gertier  believes 
that  exhalations  of  marshes  and  stables  have  something  to  do  with  its 


VEMIQO.  90i 

occurrence^  He  also  finds  it  more  common  in  those  who  work  in  a 
bowed  position,  and  in  those  who  are  much  fatigued  and  are  neurotic 
and  emotionah  It  is  observed  in  laborers  on  farms.  If  has  been  seen 
in  epidemics  about  Geneva,  but  no  fatal  cases  have  been  observed.  In 
the  cases  in  Japan,  Miura  found  an  exaggeration  of  the  knee-jerks. 
Dejerine  thinks  that  the  peculiar  fits  to  which  cats  are  liable  are  of 
this  same  nature. 

V.  Vertigo  may  occur  as  a  general  symptom  in  disease  of  any  kind 
within  the  cranium.  It  is  especially  frequent  in  diseases  causing  a 
sudden  change  in  intracranial  pressure.  Thus  the  effort  of  straining 
at  stool,  lifting  a  heavy  weight,  or  running  fast  may  cause  vertigo. 
Apoplexy  is  usually  preceded  or  attended  by  vertigo,  and  brain  tumors 
or  abscesses,  sclerosis  or  syphilis,  no  matter  what  their  location,  fre- 
quently cause  vertigo.  It  is  probable  that  in  all  these  conditions  some 
irritation  is  set  up  in  the  cerebellum  or  some  defective  circulatory  con- 
dition is  produced  in  it  sufficient  to  disturb  its  function.  This  form 
of  vertigo  in  not  very  intense  and  never  causes  forced  movements,  nor 
is  it  usually  sufficient  to  cause  unsteadiness  of  gait.  It  is  often  attended 
by  vomiting  and  sometimes  by  convulsions.  It  is  not  usually  asso- 
ciated with  deafness.  It  is  not  relieved  by  lying  down.  It  is  worse 
on  waking  after  sleep.     It  may  be  relieved  by  hot  baths. 

VI.  Vertigo  may  also  be  due  to  irritation  in  the  cerebral  centres 
produced  by  conditions  of  the  blood.  Thus  certain  toxic  agents,  alco- 
hol, nicotine,  coffee,  opium,  quinine,  the  salicylates,  the  coal-tar  pro- 
ducts, and  many  other  drugs,  cause  vertigo.  Vertigo  is  an  early  symp- 
tom in  auto-intoxication  from  stomach  or  intestinal  fermentation  and  in 
the  early  stage  of  almost  all  infectious  diseases ;  gout,  uraemia,  and 
diabetes  may  produce  vertigo. 

VII.  Vertigo  may  be  due  to  anaemia  of  the  brain  from  heart  failure., 
chronic  valvular  disease,  particularly  aortic  obstruction,  and  mitral 
regurgitation,  and  from  arteriosclerosis.  It  attends  a  fainting  fit  and 
severe  hemorrhage.  It  is  also  produced  by  venous  congestion  of  the 
brain,  as  after  violent  exertion,  straining  at  stool,  or  lifting  heavy 
weights. 

VIII.  There  are  some  forms  of  vertigo  which  are  reflex  in  origin. 
These  are  so-called  stomach  vertigo  and  laryngeal  vertigo.  The  nuclei 
of  the  vagus  nerve  lie  close  to  that  of  the  vestibular  nerve.  Any  irri- 
tation reaching  one  may  easily  extend  to  the  other  or  be  conveyed  to 
the  other  by  association  fibres ;  hence  vertigo  and  vomiting  are  com- 
monly associated  symptoms.  Stomach  vertigo  is  a  form  of  vertigo 
occurring  in  sudden  attacks  associated  with  belching  of  gas,  or  raising 
of  acid  fluid,  with  pain  in  the  stomach  and  with  various  symptoms  of 
dyspepsia,  and  relieved  by  vomiting.  It  may  attend  any  ingestion  of 
food  or  it  may  follow  a  meal  after  two  or  three  hours.  It  is  not  infre- 
quently a  symptom  in  dilatation  of  the  stomach,  but  rarely  in  organic 
disease  sucli  as  ulcer  or  cancer.  It  is  more  frequent  in  patients  who 
are  anaemic,  poorly  notirislKid,  or  neurasthenic!.  It  not  uiuionmionly 
develops  in   old    persons.      It  usually  yields  promptly  to  treatment 


902  VERTIGO. 

directed  to  the  stomach.  Large  doses  of  bicarbonate  of  soda,  bismuth, 
or  salol  relieve  it  at  once,  especially  if  taken  in  hot  water ;  and  care 
in  the  diet,  with  proper  treatment  for  indigestion  prevents  a  recurrence 
of  attacks. 

Laryngeal  vertigo  is  a  symptom  occurring  usually  in  the  course  of 
tabes,  especially  when  other  laryngeal  symptoms  have  been  present. 
It  occurs  in  a  sudden  attack,  with  cough,  sense  of  suffocation,  dyspnoea, 
and  feeling  of  strangling.  It  lasts  only  a  few  moments,  but  may  recur 
several  times.  It  may  be  so  intense  as  to  cause  asphyxia  and  loss  of 
consciousness,  and  it  has  been  fatal  in  some  cases. 

Nasal  vertigo  may  also  be  mentioned,  though  the  reflex  irritation 
here  arises  in  the  fifth  nerve.  It  occurs  in  cases  of  polyps  and  obstruc- 
tive mucous  thickening  in  the  nose,  and  is  relieved  by  the  removal  of 
the  original  disease. 

Other  diseases  supposed  to  excite  vertigo  by  reflex  irritation  are 
uterine  and  ovarian  affections. 

IX.  Psychical  disturbances  sometimes  produce  vertigo.  The  dizzi- 
ness one  feels  when  on  a  height  is  an  example ;  and  as  any  mental 
experience  may  be  revived  in  memory  with  an  intensity  equal  to  its 
perception,  hallucinatory  vertigo  is  a  common  symptom.  It  occurs  in 
many  cases  of  neurasthenia  and  hysteria,  in  hypochondria,  and  in  trau- 
matic neuroses.  This  form  of  vertigo  is  rarely  attended  by  staggering ; 
it  belongs  to  a  class  of  symptoms  known  as  phobias  or  morbid  fears, 
the  fear  of  falling  being  often  attended  by  vertigo.  In  such  cases  sug- 
gestion is  of  great  use  in  treatment.  Thus  in  one  case  so  severe  as  to 
keep  the  patient  in  bed  a  cure  was  produced  by  convincing  her  that 
if  her  head  was  held  steady  she  would  feel  no  dizziness.  The  head 
was  held  at  first  by  the  hands,  later  by  a  firm  head  splint  attached  to 
the  back,  and  after  two  or  three  days  the  symptom  disappeared. 


CHAPTER   LIV. 

DISOEDEES  OF  SLEEP. 

There  are  several  different  types  of  insomnia.  There  is  first  the 
type  in  which  the  individual  on  going  to  bed  is  unable  to  get  to  sleep 
for  an  hour  or  more. 

There  is  secondly  a  type  in  which  the  individual  falls  asleep  easily 
but  wakes  repeatedly,  falling  asleep  again,  and  so  has  a  very  restless 
sleep. 

There  is  thirdly  a  type  in  which  the  individual  gets  to  sleep  easily 
and  sleeps  heavily  but  wakes  at  three  or  four  o'clock  in  the  morning 
and  cannot  get  asleep  again. 

There  is  fourthly  a  type  in  which  the  individual  has  a  sense  of  fear 
of  sleeplessness  and  worries  all  the  night  through,  only  falling  asleep 
in  the  early  morning  hours  from  exhaustion  and  then  sleeping  rather 
heavily  late  into  the  day.  Each  of  these  requires  a  separate  con- 
sideration. 

Insomnia  as  a  symptom  of  other  organic  and  nervous  affections  or 
of  general  diseases  is  very  common,  and  when  it  is  recognized  as 
merely  a  symptom  and  its  origin  is  well  understood,  it  may  or  may 
not  awaken  any  special  anxiety  or  require  any  special  treatment.  Thus 
the  insomnia  of  an  acute  infectious  disease  or  the  insomnia  connected 
with  the  pain  of  neuralgia  or  tumor  of  the  brain  or  of  locomotor  ataxia 
or  the  insomnia  of  paresis  or  acute  mania,  are  conditions  which  have 
to  be  met  as  they  arise.  Some  form  of  insomnia,  however,  of  the 
types  just  recounted,  is  often  the  chief  symptom  which  has  to  be  dealt 
with  and  may  be  the  only  symptom  which  annoys  the  patient.  Hence 
a  brief  consideration  of  the  treatment  of  insomnia  as  a  symptom  is 
warranted  and  the  various  types  will  be  considered  in  the  order  named. 

I.  First.  Wakefulness  on  retiring.  The  essential  to  falling  to 
sleep  is  the  gradual  suspension  of  mental  activity,  the  checking  of  the 
course  of  thought  and  a  state  of  repose  of  mind  and  of  body.  Any- 
thing therefore  which  excites  the  mental  activity,  such  as  a  course  of 
study,  active  excitement  produced  by  a  state  of  worry  and  anxiety, 
increased  interest  after  a  theatre  or  an  active  game  of  chance,  or  the 
artificial  excitement  produced  by  the  use  of  coffee,  tea  or  alcohol,  or  the 
excessive  use  of  tobacco,  will  produce  a  state  of  mind  in  no  way  con- 
ducive to  sleep  ;  and  until  the  train  of  thought  and  excitement  is  over 
or  the  effects  of  the  artificial  excitement  have  passed  away,  the  mind 
cannot  sink  into  that  state  of  inactivity  which  is  essential  to  sleep. 

While  the  exact  mechanism  of  sleep  is  at  present  still  undetermined, 
there  are  very  many  facts  which  point  to  the  probability  that  the  act 

903 


904  DISOBDEBS    OF   SLEEP. 

of  sleep  Is  preceded  by  a  diminution  in  the  amount  of  blood  in  the  brain 
and  it  is  pretty  well  admitted  that  anything  which  increases  the  flow 
of  blood,  such  as  a  state  of  functional  activity  in  the  brain,  prevents 
sleep.  In  order  therefore  to  secure  sleep  it  is  necessary  to  arrest  the 
dominant  train  of  thought  and  if  possible  reduce  the  functional  hyper- 
seraia  of  the  brain. 

The  dominant  train  of  thought,  whatever  its  character,  may  be 
counteracted  by  forcing  the  attention  into  other  lines  for  a  longer  or 
shorter  period,  until  the  tendency  to  recur  to  the  train  of  thought  which 
it  is  sought  to  break  has  been  arrested.  Many  artificial  means  have 
been  suggested  to  secure  this,  such  as  the  repetition  of  familiar  poetry, 
the  recalling  to  the  mind  one  after  another  of  familiar  scenes,  such  as 
have  followed  each  other  on  a  trip  or  in  some  period  of  one's  life ; 
counting ;  repeating  nonsense  syllables  ;  repeating  the  multiplication 
table  or  similar  methods.  The  functional  hypersemia  may  be  counter- 
acted by  applying  cold  cloths  to  the  head  ;  by  a  general  long-con- 
tinued hot  bath  ;  by  putting  the  feet  in  hot  water ;  by  going  through 
a  series  of  physical  exercises  stripped  ;  by  taking  a  series  of  long  res- 
piratory exercises  at  an  open  window  ;  by  taking  a  short  brisk  walk 
before  attempting  to  retire ;  by  applying  cold  applications  such  as  an 
alcohol  spray  to  the  back  of  the  neck  which  by  its  evaporation  causes 
a  coolness  and  at  the  same  time  produces  a  slight  irritation  in  the 
cervical  region  which  is  found  experimentally  to  produce  a  contraction 
of  the  blood  vessels  in  the  head ;  or  by  general  massage,  which  will 
often  produce  sleep  when  other  methods  fail.  If  the  object  to  be 
obtained  by  these  measures  is  explained  to  the  individual  and  meets 
with  his  concurrence  and  if  he  is  urged  to  exert  a  certain  amount  of 
voluntary  effort  to  go  to  sleep,  accompanying  it  by  a  process  of  auto- 
suggestion produced  by  the  repetition  in  the  mind  over  and  over  again 
of  the  words,  "  I  am  going  to  sleep,"  "  I  am  feeling  drowsy,"  "  my 
muscles  are  all  relaxed,"  "  I  am  quiet,"  "  I  am  sleeping,"  it  is  quite 
possible  that  sleep  will  be  induced  without  any  resort  to  drugs. 

If  these  methods  fail  and  drugs  are  to  be  used  a  number  of  hyp- 
notics are  at  our  disposal.  The  simplest  of  these  is  bromide  of  sodium 
or  potassium  which  may  be  given  well  diluted  in  water  in  doses  vary- 
ing from  10  up  to  50  grains  just  as  the  individual  is  going  to  bed.  In 
the  administration  of  drugs  for  this  type  of  wakefulness  it  is  well  to 
give  a  large  dose  on  the  first  occasion  and  then  when  the  expectation 
of  a  prompt  effect  has  been  awakened,  on  subsequent  nights  to  give  a 
lesser  dose  without  the  patient's  being  aware  that  the  dose  has  been 
reduced.  Another  remedy  in  this  type  of  wakefulness  is  a  combina- 
tion of  bromide  of  sodium  20  grains  with  chloral  hydrate  5  or  10 
grains  according  to  the  weight  of  the  patient,  a  heavy  person  requiring 
a  larger  dose,  though  chloral  should  never  be  given  if  there  is  any 
cardiac  weakness.  If  this  form  of  sleeplessness  does  not  yield  to 
bromide  or  chloral,  a  remedy  of  some  efficacy  is  the  tincture  of  hyos- 
cyamus.  This  is  to  be  given  in  5-drop  doses  repeated  every  ten 
minutes  for  an  hour  before  retiring,  so  that  a  drachm  will  be  taken  all 


DISORDERS   OF  BLBBP.  905 

together,  in  order  to  produce  the  effect.  In  cases  which  resist  these 
remedies  regC)rt  may  be  had  to  trional  in  5-  or  10-grain  dose,  to  veronal 
in  7|^-grain  dose,  or  to  medinal  in  5-grain  dose,  or  to  sulphonal  in  10- 
grain  dose.  Any  of  these  should  be  given  in  the  form  of  powder  trit- 
urated with  an  equal  amount  of  sugar  of  milk  and  should  be  given  in 
or  with  a  glass  of  milk  or  with  some  food  half  an  hour  before  retiring. 
In  this  way  the  drug  will  be  digested  by  the  stomach  soon  after  taken. 
If  the  stomach  is  not  stimulated  to  digestion  by  food  when  these  drugs 
are  taken  they  may  lie  undissolved  all  night  and  have  their  effect  only 
after  breakfast,  when  they  make  one  drowsy  through  the  entire  day. 
In  giving  these  drugs  it  is  also  well  to  reduce  the  initial  dose  after  two 
or  three  nights  and  this  can  be  done  by  increasing  the  amount  of  sugar 
of  milk  in  the  powder  without  the  patient's  knowledge  that  the  drug 
is  reduced.  Many  patients  will  go  to  sleep  if  they  have  a  remedy 
that  they  believe  to  be  efficacious  at  their  bedside,  and  have  permission 
to  take  it  in  case  they  do  not  go  to  sleep  ;  and  many  patients  having 
once  been  put  to  sleep  by  a  powder  of  veronal  or  medinal  will  sleep 
equally  well  when  they  are  given  a  powder  of  sugar.  For  this  reason 
it  is  well  to  conceal  the  taste  of  the  drug  by  means  of  the  sugar  or  if 
this  is  not  sufficient  a  small  amount  of  pulverized  ginger,  which  con- 
ceals the  taste  effectively  may  be  added,  or  the  drug  can  be  given  in 
capsule  form  and  subsequently  sugar  can  be  put  in  the  capsules  in  its 
place.  Where  a  patient  is  of  an  impressionable  nature  and  yet  has  the 
fear  of  sleeplessness,  and  where  it  is  essential  to  give  a  remedy,  it  is 
well  that  these  measures  should  be  used,  as  the  habit  of  taking  a  strong 
drug  every  night  to  induce  sleep  is  a  very  bad  one. 

II.  The  second  type  of  insomnia  is  the  type  in  which  the  individual 
gets  to  sleep  easily  but  wakes  easily  and  lies  awake.  Many  persons 
are  exceedingly  sensitive  to  sounds  and  are  awakened  very  easily,  and 
if  one  goes  to  sleep  with  the  expectation  of  waking  at  a  light  sound  he 
is  extremely  liable  to  do  so.  Special  sounds  may  waken  one  when 
ordinary  sounds  do  not.  Thus,  a  mother  will  go  to  sleep  by  the  side 
of  an  infant  and  will  awaken  at  the  least  sound  coming  from  that 
infant  though  much  louder  sounds  about  her  will  fail  to  awaken  her. 
Similarly,  if  a  person  goes  to  sleep  with  the  fear  that  he  may  be 
awakened  by  the  passing  of  an  automobile,  or  the  sound  of  a  train,  or 
by  the  cry  of  an  animal,  that  particular  sound  will  often  waken  him 
when  sounds  which  are  much  louder  will  fail  to  do  so.  It  is  necessary 
to  mention  these  facts  to  persons  who  are  wakeful,  as  it  is  not  impos- 
sible by  a  certain  discipline  of  the  mind  to  influence  the  individual  and 
thus  secure  proper  rest.  When  this  type  of  wakefulness  therefore  is 
due  to  hyper-sensitiveness  to  sounds  and  cannot  be  voluntarily  con- 
trolled the  quietest  possible  surroundings  should  be  secured  and  the 
patient  may  even  be  allowed  to  place  cotton  in  the  ears  for  the  night 
in  order  to  deaden  sounds. 

Another  reason  for  wakefulness  is  discomfort  from  indigestion.  The 
processes  of  digestion  go  on  more  slowly  in  some  persons  than  in  others 
and  the  stomach  frequently  contains  food  six  hours  after  the  last  meal 


906  BISOEDEES    OF   SLEEP. 

if- there  is  any  atony  of  its  wall.  Under  these  circumstances  an  excess 
of  acidity  may  be  produced  which  is  sufficient  to  wakeu  a  person  after 
he  has  gone  to  sleep.  It  is  uncertain  whether  it  is  the  nervous  impulse 
from  the  walls  of  the  stomach  or  whether  it  is  the  chemical  change  in 
the  blood  which  produces  the  irritation  of  the  brain  and  wakes  the 
patient.  But  the  practical  remedy  for  such  cases  is  to  take  an  alkali 
into  the  stomach  on  waking.  If  therefore  a  patient  wakes  with  gas 
in  the  stomach  or  with  an  acid  rising  in  the  throat  or  with  a  reflex 
stomach  cough  the  administration  of  10  grains  of  bicarbonate  of  soda 
or  the  taking  of  five  soda  mint  tablets  or  the  drinking  of  a  half  glass  of 
vichy  in  which  soda  has  been  dissolved  will  soon  be  followed  by  a  sense 
of  comfort  and  sleep  will  follow.  Any  case  of  this  kind  will  yield  to 
this  alkaline  remedy  when  hypnotics  under  the  circumstances  will 
produce  no  effect. 

In  other  cases  irregularities  of  the  circulation  appear  to  produce  a 
wakefulness  after  the  initial  sleep.  These  patients  will  wake  with  cold 
feet  or  general  chilliness  or  possibly  with  a  sense  of  extreme  heat  and 
burning  of  the  body.  In  the  former  case  a  hot  water  bag  placed  in  the 
bed  at  night  will  prevent  the  patient  from  waking,  or  a  small  dose  of 
nitroglycerine  1/200  of  a  grain  or  a  2-grain  tablet  of  thyroid  extract 
taken  at  bed  time  may  prevent  the  spasm  of  the  vessels  which  causes 
the  cold  and  the  wakefulness.  When  the  patient  wakes  in  a  state  of 
heat,  with  the  body  burning  or  the  feet  burning,  it  is  a  sign  of  a 
general  gouty  diathesis  and  this  should  be  immediately  treated  by  a 
course  of  alkaline  remedies,  after  which  the  sleep  will  be  more  quiet. 
To  use  light  bed  clothes  or  even  to  get  up  and  walk  about  a  cool  room, 
exposing  the  body  to  cold  air,  will  be  a  measure  which  the  patient  will 
spontaneously  adopt  and  will  be  successful.  Irritability  of  the  bladder 
frequently  interferes  with  sleep  and  under  these  circumstances  if  the 
underlying  condition  can  be  cured  sleep  will  be  resumed.  If  not,  the 
administration  of  alkaline  remedies,  citrate  of  potash  or  urotropin  com- 
bined with  bromide  and  given  at  intervals  before  bedtime  without 
much  water  may  prevent  the  wakefulness. 

In  these  cases  of  the  second  type  small  doses  of  bromide,  10  grains 
given  every  two  hours  for  four  times  prior  to  going  to  sleep,  will  often 
when  kept  up  for  a  week  or  more  produce  such  a  quieting  effect  upon 
the  nervous  system  as  to  counteract  permanently  the  tendency  to  wake 
on  slight  provocation.  If  bromides  are  found  to  be  insufficient  in 
there  cases  resort  may  be  had  to  trional,  veronal  or  medinal  given  as 
described  above. 

III.  The  third  type  of  insomnia  is  one  in  which  the  person  goes  to' 
sleep  easily,  sleeps  well  for  a  time,  but  wakes  at  two  o'clock  or  soon 
after  and  is  unable  to  get  to  sleep  again.  This  is  a  type  most  frequently 
met  with  in  neurasthenia  of  toxic  origin ;  and  is  always  present  in 
melancholia  and  in  manic  depressive  insanity.  It  may  be  the  first 
symptom  in  these  mental  states.  It  seems  as  if  in  this  condition  some 
chemical  process  went  on  during  the  sleeping  hours  which  resulted  in 
the  production  of  some  poison  in  the  blood ;    and  this  poison,  like 


DISOBDEES    OF    SLEEP.  907 

caffeine,  caused  wakefulness.  This  type  of  insomnia  is  the  most  diffi- 
cult type  to  treat.  It  is  useless  to  give  sleeping  potions  at  bedtime, 
for  the  patient  has  a  natural  tendency  to  go  to  sleep,  and  if  they  are 
given  they  seem  merely  to  prolong  the  natural  sleep  an  hour  or  two 
and  do  not  prevent  the  waking.  JSTor  are  sleeping  medicines  of  much 
avail  if  taken  when  the  patient  awakes.  He  is  usually  restless,  anxious, 
fearful  of  not  sleeping,  and  often  much  depressed  in  spirits  on  waking. 

The  same  type  of  insomnia  is  likely  to  be  present  in  conditions  of 
arterial  sclerosis  in  old  people  and  in  cases  of  senile  dementia. 

It  is  well  to  advise  patients  suffering  from  this  form  of  insomnia  to 
get  up  when  they  wake,  move  about  the  room,  read  awhile  and  take 
some  food,  such  as  hot  milk  or  cocoa  or  bouillon,  which  has  been  kept 
ready  in  a  thermos  bottle.  They  may  remain  up  for  a  half  hour  or 
more  and  then  on  going  back  to  bed  may  go  to  sleep  again. 

If  medicines  are  used,  10  grains  of  chloral  hydrate,  or  1  drachm  of 
paraldehyde  or  strychnin  1/100  grain  will  often  prove  of  service. 
Tincture  of  hyoscyamus  is  a  good  remedy,  also  fluid  extract  of  celery 
in  one-drachm  dose.  In  some  cases  a  little  whiskey  will  put  the 
patient  to  sleep.  If  the  hour  of  waking  is  very  early  (3  a.m.)  veronal 
or  trional  may  be  given  with  some  food. 

IV.  The  last  type  of  insomnia  to  be  mentioned  is  the  type  in  which 
an  individual  retires  with  a  fear  of  not  going  to  sleep  and  has  a  great 
dread  of  lying  awake  and  is  unable  to  compose  the  mind  or  the  body 
in  such  a  way  as  to  induce  sleep  or  permit  of  its  natural  occurrence. 
Some  of  these  individuals  are  really  in  the  early  stage  of  mania  and 
this  type  of  insomnia  is  then  but  a  symptom  of  a  serious  mental 
disease.  The  majority  of  such  patients,  however,  are  suffering  merely 
from  hysteria  and  the  morbid  fear  of  sleeplessness.  No  amount  of 
persuasion  or  argument  will  suffice  to  convince  these  patients  that 
they  can  or  will  sleep,  and  although  all  the  measures  that  have  been 
proposed  in  dealing  with  the  first  class  of  cases  of  insomnia  may  be 
tried,  yet  as  a  rule  it  is  necessary  to  compel  sleep  in  these  patients  by  a 
very  large  dose  of  some  powerful  hypnotic  and  thus  put  them  to  sleep 
in  spite  of  their  determination  not  to  go  to  sleep.  Having  once,  how- 
ever, put  them  to  sleep  in  this  manner  and  overcome  their  fear  of 
insomnia  it  is  wise  to  continue  a  placebo  on  successive  nights  without 
giving  a  strong  remedy,  as  the  persuasion  that  the  remedy  will  have 
the  effect  is  capable  of  overcoming  the  fear.  With  this  in  mind  it  is 
right  to  select  such  a  remedy  for  the  first  dose  as  can  be  concealed  by 
some  powder  or  syrup  which  will  have  the  same  taste  as  the  placebo 
so  that  the  patient  will  not  be  aware  that  the  second  dose  is  in  any 
way  different  from  the  first.  For  that  reason  I  prefer  a  large  dose  of 
su]j)honal  or  trional  or  veronal  mixed  with  powdered  ginger  and 
powdered  sugar  of  milk,  and  in  the  second  dose  give  the  sugar  of  milk 
and  ginger  without  the  drug.  If  the  case  is  one  of  beginning  mania 
no  drug  will  produce  sleep.  If  the  case  is  one  of  hystc^ria  this  method 
will  occasionally  succ(!od.  In  hysterical  insomnia  many  nonsensical 
measures  meet  with  success   purely  because  they  appeal   to  the  imagi- 


908  Pisobdebs  of  sleep. 

nation  of  the  patient  and  produce  an  auto-suggestion  that  induces 
sleep.  Such  remedies  are  hop  pillows,  the  inhalation  of  stramonium 
cigarettes,  electrical  belts  worn  about  the  body  or  the  numerous  fraud- 
ulent ostensibly  electrical  devices  which  are  placed  beside  the  bed  and 
attached  to  the  arm  by  bands  which  are  supposed  to  convey  electricity. 
There  are  many  fraudulent  devices  of  this  kind  in  the  market  which 
patients  will  praise,  as  they  have  apparently  brought  them  relief.  If 
drugs  are  used  to  produce  sleep  in  hysteria  it  is  my  experience  that  the 
best  remedies  are  very  minute  doses  of  strychnin  frequently  repeated; 
thus  a  I  drop  of  nux  vomica  or  tincture  of  ignatia  or  a  1/1000  of  a 
grain  of  strychnin  given  every  five  minutes  with  the  assurance  that 
after  a  few  doses  the  patient  will  be  sleepy,  but  also  with  the  direction 
to  keep  the  remedy  up  until  the  patient  can  no  longer  keep  awake,  will 
sometimes  produce  a  sufficient  suggestion  to  conquer  this  form  of 
insomnia. 


CHAPTER   LV. 

REFLEX   NEUROSES. 

The  active  part  of  the  nervous  system  is  the  gray  matter.  The 
white  fibres  merely  carry  impulses  to  and  from  it.  This  gray  matter 
is  collected  into  three  quite  distinct  masses  :  (1)  the  spinal  and  medul- 
lary centres  —  i.  e.,  those  from  which  the  spinal  and  cranial  nerves 
arise  ;  (2)  the  basal  ganglia  ;  (3)  the  cortex.  In  the  entire  vertebrate 
species  it  is  possible  to  assign  to  these  distinct  masses  different  func- 
tions :  to  the  spinal  and  medullary  centres  reflex  action,  to  the  basal 
ganglia  automatic  action,  to  the  cortex  conscious  and  voluntary  action. 
In  dealing  with  refl!ex  disturbance  it  would,  therefore,  at  first  appear 
that  we  were  to  be  limited  in  our  discussion  to  affections  of  the  spinal 
cord  and  medullary  centres.  These  must  certainly  be  considered. 
Yet  we  are  not  thus  limited.  For  each  lower  system  is  under  constant 
control  from  the  one  above  it ;  hence  the  spinal  (reflex)  activity  being 
the  lowest  is  dominated  both  by  the  basal  (automatic)  activity  and  by 
the  cortical  (voluntary)  activity.  Hence,  disturbances  of  reflex  action 
may  be  brought  about  by  anything  which  either  increases  the  normal 
inhibitory  action  of  the  higher  centres  or  removes  that  action,  as  well 
as  by  disease  of  these  reflex  centres  themselves.  A  prompt  motor 
response  to  a  sensory  stimulus  is  the  action  which  should  normally  take 
place  when  the  reflex  centres  are  called  into  play.  Sneezing,  wink- 
ing, swallowing,  coughing,  the  secretion  of  alkaline  saliva  in  the  mouth, 
and  acid  gastric  juice  in  the  stomach,  when  any  foreign  body  is  put  into 
these  cavities,  the  act  of  vomiting  when  that  body  is  an  active  irritant, 
or  of  increased  peristalsis  of  the  intestines  if  the  foreign  substance 
reaches  it ;  the  flushing  of  the  skin  with  blood  when  exposed  to 
changes  of  temperature,  the  regular  respiratory  and  cardiac  movements, 
and  the  vascular  tone ;  these  as  well  as  the  so-called  tendon  and  skin 
reflexes  are  examples  of  reflex  activity.  Without  such  reflex  action 
the  balance  of  supply  and  demand  in  the  human  economy  would  be 
impossible  and  life  itself  would  cease.  A  power  of  quick  and  appro- 
priate response  to  normal  or  abnormal  irritation  is  a  natural  power  of 
the  lowest  masses  of  gray  matter.  I  cannot  find  any  evidence  from 
the  study  of  disease  that  the  exercise  of  this  power  is  in  any  way  detri- 
mental. This  power  may  be  interfered  with  in  several  ways.  Strych- 
nine and  its  coefficient  drugs  will  increase  it.  The  result  is  manifest 
(1)  l)y  an  unnaturally  strong  response  to  a  weak  irritation  ;  (2)  by  an 
abnormally  long  and  contitnied  response  to  ordinary  irritation.  The 
spasms  on  slight  touch  and  the  opisthotonos  with  rigidity  in  a  guinea- 
pig  poisoned   by  strychnine  are  examples  of  both.     In   man  tetanus 

909 


910  REFLEX    XEUROSES. 

furnishes  a  good  illustration  ;  but  the  extraordinary  state  of  nervous 
irritation,  the  starting  and  jumping  at  little  noises,  the  flushing  on 
slightest  irritation,  the  cramps  produced  by  heat,  the  extreme  uneasi- 
ness and  hypersesthesia  present  in  spinal  meningitis  are  equally  good 
examples  of  increased  reflex  activity  due  to  direct  irritation.  Reflex 
activity  may  be  suspended  by  violent  and  sudden  irritation.  Injuries 
to  the  abdomen  or  head  may  arrest  all  reflex  activity  and  cause  death 
in  man  as  well  as  in  a  frog,  as  described  by  Goltz.  It  may  be  impaired 
by  an  excess  of  moderate  irritations  coming  simultaneously.  Just  as 
the  conscious  attention  cannot  be  given  at  one  time  to  several  subjects, 
and  elicit  simultaneously  appropriate  responses  to  all,  so  reflex  centres 
may  be  embarrassed  by  numerous  simultaneous  stimuli  and  fail  to  react 
to  any  one  in  proper  degree.  The  condition  subsequent  to  severe 
traumatic  concussion  is  an  illustration. 

When  we  seek  for  illustrations  of  either  increased  or  decreased  reflex 
excitability  from  spinal  disease  it  is  evident  that  we  must  allude  to 
very  serious  diseases.  The  reflex  mechanism  is  too  important  and  too 
stabile  to  be  affected  by  little  irritants,  or  by  slight  causes.  If  it  were 
not,  the  numerous,  inevitable  stimuli  consequent  upon  an  active  life 
would  constantly  put  that  life  in  danger.  How  few  of  the  severe  acci- 
dents which  constantly  occur  really  affect  in  any  marked  degree  the 
reflex  nervous  system  ?  Many  serious  irritations,  traumatic,  painful, 
distressing,  fail  to  cause  a  single  spinal  nervous  symptom  ?  The  sur- 
gical wards  of  a  hospital  offer  little  field  for  the  observation  of  reflex 
nervous  disturbance  —  happily  for  the  inmates.  In  the  lower  animals 
such  affections  rarely  exist. 

Turning  now  to  the  other  cause  of  disturbance  of  reflex  function, 
defective  control  by  higher  centres,  we  at  once  enter  upon  a  broader 
field.  Arrest  of  reflex  activity  is  rare  and  needs  little  consideration. 
It  may  be  produced  voluntarily,  as  one  may  prevent  the  knee-jerk  by 
intention,  or  inhibit  the  act  of  swallowing,  as  in  hysterical  dysphagia. 
It  may  be  produced  involuntarily,  as  when  cerebral  haemorrhage 
inhibits  respiratory  or  cardiac  action  ;  or  extreme  fright  causes  death 
in  the  same  way.  Increase  of  reflex  activity  from  central  causes  is 
common.  The  act  of  control  must  be  constantly  exerted  in  order  to 
preserve  the  balance  of  power  and  the  proper  harmony  of  action  in 
the  nervous  system.  Whether  we  start  from  a  consideration  of  moral 
conduct  or  of  the  plantar  reflex  we  reach  the  same  conclusion.  And 
in  central  control  a  slight  defect  produces  widespread  and  serious 
results.  A  man  who  has  once  abused  a  confidence  will  not  be  im- 
plicitly trusted,  though  he  lives  for  a  hundred  years.  A  small  clot  in 
the  brain  will  set  all  the  motor,  vasomotor,  and  sympathetic  centres  in 
such  a  state  of  instability  as  to  impair  all  confidence  in  their  action. 
A  hemiplegic  who  has  apparently  recovered  still  has  the  increased 
knee-jerk.  This  may  not  in  any  way  impede  the  action  of  the  reflex 
centres.  That  is  carried  on  exactly,  accurately,  satisfactorily,  and  its 
action  does  no  harm.  The  emptying  of  the  bladder  as  soon  as  it  is 
full  is  a  perfectly  normal,  satisfactory  act  on  the  part  of  the  lumbar 


REFLEX    NEUROSES.  911 

spinal  cord/and  active  incontinence  of  urine  is  not  a  symptom  that  it 
is  diseased.  It  is  an  indication  that  impulses  calculated  to  restrain  the 
act  till  an  appropriate  time  and  place  offers  are  prevented  from  reach- 
ing that  normal  reflex  centre.  There  must  be  a  proper  harmony  and 
succession  of  reflex  acts  with  some  definite  order  for  the  nervous 
system  to  fulfil  its  whole  duty.  And  any  defect  in  that  order  may 
cause  effects  which  in  other  conditions  would  not  be  defects. 

If  these  patent  facts  of  physiology  be  accepted,  I  think  we  must  con- 
clude that  in  those  diseases  known  as  reflex  neuroses,  in  which  there 
is  no  real  defect  in  reflex  action,  but  only  an  undue  or  inharmonious 
reflex  activity,  the  actual  origin  of  the  disease  must  be  either  in  an 
excess  of  activity  in  the  lower  centres  or  a  defect  of  control  in  the 
higher  centres.  As  we  have  seen,  an  excess  of  activity  is  commonly 
called  forth  only  by  very  severe  causes ;  while  a  defective  control 
may  come  from  slight  ones.  Phenomena  compai'able  to  tetanus  or 
strychnine  poisoning  are  rarely,  if  ever,  seen  in  reflex  neuroses.  In 
the  various  reflex  neuroses  in  which  peripheral  irritation  is  the  sup- 
posed cause  of  disease,  it  is  my  opinion  that  when  peripheral  irritation. 
is  the  actual  cause,  nature  indicates  it  by  attracting  attention  to  the 
seat  of  irritation  by  discomfort  or  by  pain.^  And  when,  as  in  very 
many  cases,  the  source  of  the  irritation  is  by  no  means  evident,  is  only 
found,  or  supposedly  found,  by  extraordinary  effort,  the  probability  is 
largely  in  favor  of  the  assumption  that  the  reflex  neurosis  is  not  pro- 
duced by  any  direct  peripheral  irritation,  but  is  the  manifestation  of  a 
slight  or  serious  defect  of  control  by  higher  centres  (iue  to  their  im- 
paired nutrition  and  consequent  impaired  activity. 

Theories  are  attractive,  but  misleading.  Experience  is  a  better 
guide.  In  my  own  experience  I  am  sure  that  a  very  large  majority 
of  the  patients  who  present  symptoms  of  reflex  neuroses  have  been 
exposed  to  influences  which  undermine  the  strength  and  nutrition  of 
the  entire  nervous  system,  have  serious  symptoms,  not  only  of  a  so- 
called  reflex  character,  but  of  defective  central  control ;  are  to  be 
regarded  as  suffering  from  functional  or  organic  disease  of  the  highest, 
most  complex,  most  highly  developed  portion  of  the  nervous  system  ; 
and  are  not  merely  the  victims  of  irritation  in  some  peripheral  organ. 

We  know  that  long  since  the  notion  of  a  reflex  paralysis — such,  for 
example,  as  a  general  paraplegia  from  a  slight  wound  of  the  hand  — 
has  been  abandoned,  and  these  cases  are  to-day  explained  more  satis- 
factorily upon  the  theory  of  mental  shock  rather  than  spinal  irritation. 
That  they  are  rare,  must  be  conceded.  Two  surgeons  of  this  city,  of 
the  largest  experience,  have  told  me  that  they  have  never  seen  a  case. 
We  are  coming  rapidly  to  the  notion  that  the  symptoms  in  the  so- 
called  condition  of  spinal  concussion  are  really  due  to  the  same  state 
of  mental  shock.  The  highest,  most  delicate,  most  easily  affected 
centres  of  the  nervous  system,  those  which  arc  the  last  to  arise  in  the 
process  of  evolution  are,  as  Hughlings-Jackson  has  pointed  out,  the 
first  to  fail  in  dissolution,  are  tlu;  (easiest  affected  by  sudden  and  unex- 
'See  Medical  liecord,  January  1,  1890. 


912  REFLEX    NEUROSES. 

pected  mental  irritation.  How  else  can  we  explain  the  fact  that,  in  a 
railway  accident,  passengers  who  are  asleep  rarely  suffer  from  after- 
effects? Passengers  who  are  awake  suffer  the  immediate  alarm  and 
the  subsequent  symptoms. 

The  phenomena  of  hypnotic  suggestion  have  proved  conclusively 
that  many  so-called  spinal  neuroses  are  really  due  to  defective  cerebral 
control.  It  is  not  necessary  to  call  such  defect  hysteria.  Mental 
shock  and  mental  worry  may  produce  such  an  unstable  state  of  the 
highest  centres  that  their  inhibitory  action  is  removed,  allowing  lower 
centres  to  manifest  unusual  activity.  In  such  cases,  then,  the  actual 
manifestation  may  perhaps  be  in  a  lower  centre,  but  the  actual  seat  of 
disease  is  not  necessarily  there.  It  is  in  the  higher  centre,  which 
controls  the  lower. 

Let  us  take,  for  example,  a  case  of  so-called  uterine  reflex  neurosis. 
The  irritation  from  the  organ  reaches  the  lower  spinal  cord.  If  its 
centres  are  really  excited  to  activity,  we  should  expect  such  symptoms 
as  occur  in  a  spinal  meningitis,  viz.  :  hypersesthesia  of  the  legs  and 
cramps  and  spasms  of  the  calves,  vesical  and  rectal  tenesmus,  and  active 
incontinence  of  urine.  But  these  are  not  the  symptoms  found.  You 
do  find  pain  and  a  dragging  sensation  in  the  back.  This  does  not  cause 
any  manifestation  of  true  spinal  reflex  activity.  There  is  no  evidence 
of  such  activity.  It  does  produce  a  wearing  discomfort,  appreciated 
consciously  ;  received,  then,  in  the  highest  cerebral  centres  ;  exhaust- 
ing them,  and  impairing  their  control  over  the  lower,  especially  the 
vaso-motor,  centres.  Hence  the  headaches,  general  nervousness,  flush- 
ings, feelings  of  heat,  etc.,  symptoms  of  general  instability  of  the  lower 
centres,  not  of  the  lumbar  cord  centres  themselves  in  particular.  It  is 
mistake  to  call  this  a  reflex  neurosis.  It  is  a  general  nervous  exhaus- 
tion, due  to  a  peripheral  pain.  The  actual  spinal  reflex  centres  have 
no  part  at  all,  or  very  little  part,  if  any,  in  the  complex  of  symptoms. 

I  think  the  same  is  true  —  mutatis  mutandis  —  of  eye-strain.  Let 
an  eye  be  actually  strained  by  defective  vision,  defective  accommoda- 
tion, or  muscular  asthenopia,  and  the  result  is  increased  winking, 
watering  of  the  eye,  feeling  of  inability  to  use  it,  pain,  heavy  eyelids, 
possibly  conjunctival  hypersemia  ;  and  in  an  animal  under  strychnine, 
nystagmus,  or  twitching  of  the  ocular  muscles  —  a  phenomenon  rarely 
seen  in  man  —  never  seen,  in  my  experience,  from  the  so-called  strain 
of  the  ocular  muscles.  Every  other  muscle  in  the  body,  if  much  over- 
strained, will  become  irritable  ;  the  knee-jerk  can  be  increased  by  exer- 
cise ;  the  biceps  twitches  spontaneously  after  a  weight  is  raised.  Who 
has  not  felt  the  tremor  of  an  over-strained  set  of  muscles?  Why  is  it 
that,  in  eye-strain  of  the  external  ocular  muscles,  nystagmus  is  never 
seen  ?  A  serious  strain  of  the  intra-ocular  tension  —  such  as  occurs 
in  astigmatism  and  hypermetropia  — gives  rise  to  discomfort,  to  volun- 
tary effort  to  strain  the  eyes  in  using,  to  effects,  in  other  words,  upon 
not  the  centres  governing  ocular  motion,  but  upon  the  conscious  volun- 
tary centres  of  the  cortex,  upon  the  centres  of  highest  evolution, 
most  delicate,  most  susceptible  to  slight  effects,     The  result  is  cortical 


REFLEX    NEUROSES.  913 

exhaustion^ similar  exactly  to  that  produced  by  uterine  or  ovarian 
pain,  or  by  pain  anywhere,  or  by  mental  shock  or  mental  worry. 

Take,  lastly,  the  so-called  reflex  convulsions  from  genital  irritation 
in  boys  —  due  to  phimosis,  or  concretions  behind  the  prepuce,  or  to 
masturbation.  Such  convulsions  occur  only  in  neurotic  children. 
They  are  always  preceded  by  mental  irritability,  they  never  occur 
without  some  evidence  of  cerebral  irritation.  It  is  rarely  that  any 
such  evidence  of  spinal  irritation,  as  frequent  micturition,  or  cramps 
in  the  calves  of  the  legs,  attends  them.  The  irritation,  then,  is  acting 
upon  the  entire  nervous  system.  The  weakest  part  yields  first.  That 
part  is  the  part  which  controls  the  other  parts.  The  child  is  not  suf- 
fering from  reflex  irritation.  Irritation  at  the  periphery  is  the  cause 
only  in  part ;  a  weak  nervous  system  is  the  potent  predisposing  cause. 
And,  unless  this  be  strengthened  at  the  same  time  that  the  peripheral 
irritation  is  removed,  there  will  be  a  recurrence  of  the  symptoms  from 
some  other  apparently  trivial  cause,  and  he  who  treats  these  will  never 
cure  the  patient.  The  same  thing  is  true  of  genital  irritation  in  adults. 
Spermatorrhoea,  seminal  emissions,  atrophy  of  the  testicles,  exist  un- 
noticed in  many  persons  until  attention  is  called  to  them,  the  mind 
dwells  on  them,  the  higher  conscious  emotional  nervous  system  is  irri- 
tated by  worry,  and  then  the  symptoms  of  so-called  sexual  or  spinal 
neurasthenia  develop.  The  trouble  is  not  spinal,  is  not  reflex,  it  is 
cortical.  Thus  physiological  investigation  and  careful  clinical  study 
combine  to  prove  that  while  peripheral  irritation  may  produce  nervous 
symptoms,  it  is  not  directly  through  the  lower  centres,  it  is  not  by 
exciting  reflex  action.  It  is  rather  by  producing  exhaustion  of  the 
highest  conscious  centres  which  control  the  entire  nervous  mechanism. 
The  largest  engine  is  governed  by  a  very  small  steam  crank.  A  little 
motion  of  that  will  produce  very  great  motion  elsewhere.  If  the  en- 
gine is  whirling  itself  to  destruction,  do  not  attempt  to  stop  the  fly- 
wheel, do  not  catch  hold  of  the  binding  straps,  but  turn  off  the  steam 
by  the  little  crank. 

In  any  case  of  so-called  reflex  neurosis  do  not  be  satisfied  by  merely 
removing  supposed  peripheral  irritation,  but  increase  the  nutrition  of 
the  entire  system,  give  it  food  and  drink,  strengthen  it  by  every  pos- 
sible means,  baths,  exercise,  rest ;  regular,  not  hurried  work  in  many 
directions,  not  in  one  only ;  and  by  tonics,  to  enable  it  to  exert  all  its 
powers  of  control,  and  the  peripheral  effects  will  disappear  of  their 
own  accord. 

In  the  presence  of  a  case  of  neurasthenia  with  peripheral  symptoms, 
of  chorea,  or  epilepsy,  or  hysteria,  with  sources  of  peripheral  irritation 
present,  go  back  of  the  apparently  important  symptoms  to  those  of 
real  causal  moment,  and  the  therapeutic  results  will  be  more  satisfac- 
tory and  more  enduring. 


58 


PART  IV. 

THE  SYMPATHETIC  NERVOUS  SYSTEM 

AND  ITS  DISEASES. 


CHAPTER  LVI. 

VASO-MOTOR   AND   TROPHIC   NEUROSES. 

Physiology  of  the  Vaso-motor  System.  Erythro-melalgia.  Symmetrical  Gangrene. 
Angio-neurotic  CEdema.  Trophic  Nerves.  Atrophy.  Hypertrophy.  Megalo- 
cephalie.     Neurotic  Ulcers. 

Definition.  —  Id  the  term  vaso-motor  and  trophic  neuroses  it  is 
intended  to  include  a  number  of  forms  of  disturbance  of  circulation 
and  nutrition  which  are  caused  by  disorders  of  the  nervous  system. 
Such  disturbances  may  occur  in  any  part  of  the  body.  They  are  not 
to  be  regarded  as  distinct  diseases,  but  rather  as  symptoms  of  lesions 
in  the  peripheral  or  central  nervous  system.  They  may  present  them- 
selves in  various  forms,  as  hypersemia  or  ansemia  or  instability  of 
vascular  tone,  as  atrophy  or  hypertrophy  or  disintegration  of  normal 
tissue.  Their  consideration  cannot,  however,  be  assigned  to  any  pre- 
vious department  of  this  volume  nor  relegated  to  various  divisions  of 
it ;  partly  because  in  some  cases  they  are  to  be  traced  to  lesions  of  the 
sympathetic  system,  not  elsewhere  considered;  partly  because  of  our 
ignorance  as  to  the  exact  location  in  many  cases  of  the  lesion  of  which 
they  are  manifestations. 

It  is  not  possible  to  distinguish  accurately  in  all  cases  between  the 
vaso-motor  and  the  trophic  neuroses,  for  while  in  many  features  they 
are  distinct,  in  a  large  proportion  of  cases  they  occur  together.  But 
it  is  not  possible  to  ascribe  all  trophic  changes  to  vascular  disturbance, 
nor  all  vaso-motor  changes  to  a  defect  or  excess  of  trophic  action. 
Hence  a  separate  consideration  of  these  allied  subjects  must  be  given. 
It  is  always  to  be  remembered,  however,  that  each  may  give  rise  to  the 
other,  and  that  in  their  pathology  they  are  closely  connected.  Vaso- 
motor disturbances  manifest  themselves  (1)  by  a  dilatation  of  the  ves- 
sels, producing  redness,  heat,  and  rapid  metabolism  in  the  part  affected ; 
or  (2)  by  a  contraction  of  the  vessels,  causing  pallor,  coldness,  and 
malnutrition  ;  or  (3)  by  an  alternation  of  these  conditions  and  conse- 
quent temporary  disturbance  of  function.  Trophic  disturbances  may 
occur  in  conse(juence  of  such  increase  or  decrease  of  blood-supply,  or 
independently  of  any  vascular  change,  causing  (1)  an  abnormal  pro- 

915 


916 


VASO-MOTOR    AND    TltOI'llW    NEUROSES. 


duction  of  tissue  in  an  organ,  or  (2)  a  decrease  in  the  size  and  number 
of  its  constituent  cells,  or  (3)  an  actual  degeneration  of  the  elements 
which  make  it  up,  after  which  their  place  may  be  taken  by  another 
kind  of  tissue.  Under  all  these  circumstances  the  function  of  the  part 
affected  will  be  disturbed,  and  symptoms  will  be  produced  which  will 
vary  with  the  tissue  or  organ  involved.  Hence  a  general  considera- 
tion of  these  symptoms  must  be  given.  Before  proceeding  to  a  detailed 
consideration  of  these  neuroses  it  is  necessary  to  review  the  physiology 
of  the  vaso-raotov  and  trophic  systems,  in  order  to  make  clear  the 
manner  in  which  they  perform  their  functions.  And  inasmuch  as  the 
pathology  of  these  affections  is  best  understood  by  comparison  with 
experimental  lesions  made  by  physiologists  in  investigating  their  func- 
tion, it  will  be  treated  together  with  their  physiology. 

VASO-MOTOR    NEUROSES. 

Physiology.  —  Local  Vascular  Tone.  —  Since  changes  in  the  force 
and  frequency  of  the  heart's  action,  and  variations  in  the  total  amount 

Fig.  318. 


Vaso-motor  Nerves  and  Ganglia  accompanying  the  Arterioles  in  a  Frog  (Glmbert) ;  C,  arterioles ; 
N,  vaso-motor  nerve;  G,  ganglion,  from  wiiich  nerves  issue,  situated  at  the  point  of  anastomosis  of 
several  capillaries ;  R,  fibre  of  Remak. 

of  blood  in  the  body,  affect  the  body  as  a  whole,  the  state  of  circula- 
tion in  any  one  organ  or  part  must  be  dependent  upon  the  degree  of 
contraction  or  dilatation  of  its  own  vessels.     This  is  known  as  the 


PHYSIOLOGY.  917 

local  vascular  tone.  It  is  under  the  control  of  a  system  of  nerve- 
ganglia  with  their  subservient  fibres  which  are  found  in  the  middle 
coat  of  all  arterioles  (Fig.  318).  The  energy  expended  by  these 
ganglia  is  manifested  by  a  constant  moderate  contraction  of  the  circular 
muscular  coat  of  the  artery  —  a  contraction  which  is  as  constantly 
opposed  by  the  dilating  force  of  the  blood-pressure  within  the  vessel. 
An  exact  equipoise  between  these  two  forces  never  occurs,  since  each 
varies  constantly,  but  in  a  state  of  health  one  never  becomes  perma- 
nently excessive.  Considerable  variations,  however,  in  the  local  vas- 
cular tone  are  frequently  observed.  Thus  each  organ  is  influenced  to 
a  certain  degree  by  every  other,  since  an  increase  of  blood  in  one  part 
must  involve  a  decrease  in  all  other  parts,  the  total  amount  of  blood  in 
the  vessels  being  constant.  Alteration  in  the  heart's  action  is  felt 
more  quickly  in  some  organs  than  in  others,  and  thus  the  general 
blood-pressure  by  its  variations  may  cause  secondarily  a  disturbance 
of  local  vascular  tone.  The  variations  now  under  consideration,  how- 
ever, are  not  of  this  kind.  They  are  such  as  are  produced  by  influ- 
ences acting  directly  upon  the  ganglia  in  the  vessel-walls. 

Local  irritation  is  such  an  influence,  and  it  may  excite  the  ganglia 
to  increased  activity,  so  producing  a  contraction  of  the  vessel  and  con- 
sequent pallor ;  or  it  may  suspend  the  action  of  the  ganglia,  so  produc- 
ing a  dilatation  of  the  vessel  and  consequent  flushing. 

Another  influence  is  irritation  acting  from  a  distance  and  conveyed 
to  the  local  ganglia  by  nerve  fibres.  These  nerve  fibres  can  be  distin- 
guished from  all  others  by  their  structure,  being  non-medullated,  and 
by  the  fact  that  they  have  an  indirect  course,  passing  from  the  central 
nervous  system  to  the  sympathetic  ganglia,  and  from  these  to  the  local 
ganglia  in  the  vessels.  The  impulses  sent  along  these  nerves  may 
affect  the  local  ganglia  in  one  of  two  ways,  and  either  cause  contraction 
by  exciting  the  ganglia,  or  dilatation  by  inhibiting  the  action  of  the 
ganglia.  The  result  produced  has  determined  the  names  given  to  the 
impulse,  to  the  fibre  transmitting  it,  and  to  the  centre  whence  the  im- 
pulse proceeds,  and  hence  vaso-constrictors  and  vaso-dilators  are  dis- 
tinguished from  one  another. 

The  history  of  the  development  of  the  sympathetic  nervous  sys- 
tem as  well  as  its  gross  anatomy,  affords  distinct  proof  that  it  is  not  an 
independent  system,  as  Bichat  supposed,  but  is  closely  connected  in  its 
physiological  action  with  the  spinal  cord  and  brain.  Impulses  which 
reach  the  sympathetic  ganglia  from  a  distance  along  the  vaso-constric- 
tor  or  vaso-dilator  fibres  originate  in  the  central  nervous  system.  The 
nervous  mechanism  which  controls  the  local  vascular  tone  is  therefore 
a  complex  one,  consisting  not  only  of  the  set  of  local  ganglia  connected 
with  larger  sympathetic  ganglia,  but  also  of  centres  in  the  spinal  cord 
connected  with  higher  centres  in  the  brain.  The  brain-centres  in  turn 
are  complex,  consisting  of  an  automatic  mechanism  in  the  medulla  regu- 
lating the  action  of  all  the  subordinate  j)arts  below  it,  and  of  a  series  of 
cortical  centres  whose  fun(;tion  it  is  to  stimulate  or  inhibit  the  medul- 
lary mechanism.     It  therefore  becomes  evident  that  local  vascular  tone 


918  VAHO-MOTOR    AND    TROPHIC    NEU ROBES. 

may  be  modified  by  local  causes  acting  on  the  ganglia  in  the  vessels  — 
e.  g.,  cold  or  heat ;  by  changes  in  the  sympathetic  ganglia  —  e.  ^.,  the 
hyperaemia  of  the  face  in  lesions  of  the  cervical  ganglia ;  by  reflex 
action  through  the  spinal  cord  —  e.  </.,  pallor  produced  by  pain;  by 
reflex  action  through  the  medulla  oblongata  —  e.  ^.,  glycosuria  follow- 
ing sciatica  ;  or  by  conscious  or  unconscious  impulses  coming  from  the 
cortex  —  e.  g.,  the  blush  of  shame,  the  vaso-motor  paralysis  of 
hemiplegia. 

Vaso-constrictors.  —  Such  a  mechanism,  however  complex  in  struc- 
ture, would  be  easily  comprehended  if  the  constant  manifestation  of 
energy  in  the  maintenance  of  arterial  tone  had  its  only  source  in  the 
action  of  the  local  ganglia  in  the  vessels,  and  was  affected  only  occa- 
sionally by  impulses  from  a  distance,  as  has  been  thus  far  supposed. 
This,  however,  is  not  the  case,  as  has  been  demonstrated  by  a  series  of 
experiments  beginning  with  the  brilliant  researches  of  Claude  Bernard. 
The  classical  experiments  of  the  French  physiologist  were  made  upon 
the  sympathetic  cord  in  the  neck  of  a  rabbit.  Division  of  this  was 
found  to  produce  a  dilatation  of  the  vessels  of  the  ear.  Irritation  of 
the  peripheral  end  of  the  divided  cord  produced  a  contraction  of  the 
vessels.  Division  of  the  spinal  nerves  connected  with  the  cervical 
sympathetic  and  of  their  anterior  roots,  or  irritation  of  the  cut  ends, 
produced  effects  similar  in  character  to  those  caused  by  division  or 
irritation  of  the  cervical  sympathetic.  Destruction  of  the  spinal  cord 
in  the  lower  cervical  region,  or  division  of  the  cord  at  any  higher  level 
up  to  the  medulla,  was  followed  by  dilatation  of  the  vessels.  If  the 
segment  of  the  divided  cord  just  below  the  section  was  irritated  the 
vessels  contracted.  Destruction  of  the  medulla  at  the  calamus 
scriptorius  and  above  it  for  three  centimeters  produced  a  general  dila- 
tation of  all  the  vessels  in  the  body,  but  division  above  this  level  had 
no  effect.  The  initial  congestion  produced  by  these  various  experi- 
ments was  accompanied  by  a  rise  of  temperature  in  the  part.  It  was 
followed  after  a  time  by  a  partial  recovery  of  vascular  tone,  which  was 
more  complete  the  farther  the  division  from  the  local  ganglia.  These 
facts  v/arranted  the  conclusion  that  the  energy  expended  by  the  local 
ganglia  in  holding  the  vessels  in  a  state  of  constant  moderate  contrac- 
tion is  derived  from  the  central  nervous  system,  primarily  from  the 
automatic  centre  in  the  medulla,  which  in  turn  is  reinforced  by  each 
of  the  secondary  centres  in  the  spinal  cord  and  sympathetic  ganglia  ; 
and  also  that  while  the  medullary  centres  control  the  entire  body,  the 
cord  and  sympathetic  centres  control  only  those  parts  with  which  they 
are  especially  related.  In  order,  therefore,  to  the  maintenance  of 
normal  vascular  tone  the  local  ganglia  must  be  intact,  and  they  must 
be  in  connection  with  the  sympathetic  ganglia  ;  these  must  be  active, 
and  must  be  connected  with  the  spinal  cord  ;  the  cord  must  be  normal, 
and  its  tracts  from  the  medulla  must  be  capable  of  conduction  ;  the 
medullary  centre  must  be  active,  and  not  hindered  or  spurred  by 
cortical  impulses  of  a  conscious  or  unconscious  nature.  Any  injury 
to  one  or  more  of  these  parts  will  produce  a  vascular  dilatation  by 


PHYSIOLOGY.  919 

interfering*-  with  the  transmission  of  vaso-constrietor  impulses  from 
within  outward,  and  any  irritation  of  one  or  more  of  these  parts  may 
cause  a  contraction  of  the  vessels  by  increasing  the  normal  stimulus 
sent  to  the  local  ganglia  by  the  vaso-constrictors. 

Vaso-dilators.  —  The  action  thus  far  considered  has  been  wholly  of 
a  vaso-constrictor  kind,  and  the  dilatation  which  has  been  mentioned 
has  been  due  to  cessation  of  the  constrictor  energy  normally  passing 
outward.  This  may  be  termed  a  passive  dilatation.  It  is  the  kind 
produced  by  division  of  any  one  of  the  sympathetic  ganglia  or  cords. 
But  further  experiments  have  shown  that  another  kind  of  dilatation 
may  be  produced,  traceable  not  to  a  mere  cessation  of  consirictor  im- 
pulses, but  to  an  impulse  of  a  positive  kind  sent  to  the  local  ganglia 
and  resulting  in  a  sudden  suspension  of  their  activity.  Such  an  im- 
pulse is  really  an  inhibitory  impulse  arresting  the  action  of  the  ganglia 
in  spite  of  the  continued  stimulus  sent  to  them  from  the  central  nervous 
system.  Its  result  is  a  dilatation  of  the  arteries,  produced  by  the 
blood-pressure  within  them,  which  may  be  turned  an  active  dilatation. 
That  vaso-dilators  act  by  inhibiting  local  ganglia  which  cause  contrac- 
tion, is  now  accepted,  especially  since  it  is  found  that  such  inhibitory 
activity  is  constantly  displayed  by  other  parts  of  the  nervous  system, 
and  is  competent  to  explain  the  facts.  The  active  dilatation  produced 
by  the  inhibition  of  the  action  of  the  local  ganglia  is  therefore  to  be 
clearly  distinguished  from  the  passive  dilatation  caused  by  the  cessa- 
tion of  normal  tonic  impulses  sent  to  them  from  higher  centres.  The 
former  is  a  positive  active  vaso-dilator  phenomenon.  The  latter  is  a 
negative  paralytic  vaso-constrictor  phenomenon.  The  former  is  more 
effective,  the  dilatation  being  greater  in  degree  and  more  permanent 
than  the  latter,  and  resembles  exactly  the  dilatation  produced  experi- 
mentally by  exhaustion  of  the  activity  of  the  local  ganglia  by  over- 
stimulation of  the  constrictors. 

An  important  point  of  contrast  which  has  been  established  between 
vaso-constrictor  and  vaso-dilator  impulses  is  that  while  the  former  are 
constant  the  latter  are  intermittent.  Hence  they  cannot  be  regarded 
as  opponents  of  one  another.  In  a  normal  quiescent  state  vaso-con- 
strictor energy  is  always  being  supplied  to  counteract  the  continued 
intravascular  pressure  ever  renewed  with  the  cardiac  systole.  The 
vaso-dilators  are  inactive.  In  an  organ  thrown  into  functional  activity 
an  increased  flow  of  blood  at  once  takes  place,  proportionate  to  the 
work  being  done  by  the  organ.  Such  a  functional  hypersemia  might 
be  produced  either  by  a  cessation  of  constrictor  impulses  or  by  an  in- 
hibition of  their  effects.  It  is  by  the  latter  means  and  through  the 
vaso-dilators  that  it  is  produced,  and  it  is  probably  the  chief  function 
of  the  vaso-dilator  nerves  to  regulate  the  blood-supply  in  accordance 
with  the  need  of  a  part.  For  this  reason  these  nerves  have  been  sup- 
posed to  pass  with  the  motor  nerves  to  the  muscles.  As  few  organs 
exist  without  a  possil)lo  use,  it  is  probable  that  vaso-dilators  pass  to  all 
parts  of  the  body,  as  Vul|)ian  ass(!rts,  though  they  have  not  been  demon- 
strated in  every  organ  or  every  part. 


920  VA80-M0T0R    AND    TROPHIC    NEUROSES. 

Like  the  vaso-constrictors,  the  vaso-dilators  can  be  traced  to  the 
spinal  cord,  and  their  centres  tiiere  are  governed  by  a  general  centre 
in  the  medulla,  which  in  turn  may  be  affected  by  impulses  from  the 
cortex.  A  destructive  lesion  in  any  part  of  the  vaso-dilator  system 
does  not  produce  as  marked  effects  as  one  involving  the  vaso-constric- 
tor  system,  since  the  symptoms  of  such  a  lesion  will  only  appear  when 
the  vaso-dilators  are  called  into  play.  When  the  vaso-dilator  nerve 
to  the  submaxillary  gland  is  cut,  no  change  is  observed  until  by  some 
sapid  substance  put  in  the  mouth  its  function  should  be  excited,  when 
the  gland  is  no  longer  found  to  flush  with  blood  as  in  the  normal  state. 
It  is  by  means  of  the  vaso-dilators  that  erectile  organs  become  engorged 
with  blood.  Eckhard  has  shown  the  nervi  erigentes  of  the  penis  to  be 
vaso-dilator  nerves.  If  they  are  destroyed,  the  organs  will  not  respond 
to  the  wonted  stimulus  —  a  symptom  which,  however,  would  only  be 
noticed  at  intervals.  An  irritative  lesion  of  the  vaso-dilator  system 
may  produce  permanent  congestion  of  an  organ  or  part,  but  this  seems 
to  be  rather  more  rare  than  a  congestion  from  paralysis  of  the  con- 
strictors.    It  is  seen  in  injuries  of  the  peripheral  nerves. 

Origin  of  the  Vaso-motor  Nerves.  —  The  exact  course  of  the  vaso- 
constrictor and  vaso-dilator  nerves  has  been  traced  from  various  parts 
into  the  central  nervous  system  by  the  careful  experiments  of  Dastre, 
Morat  and  by  Gaskell.  It  is  now  established  that  they  exist  as  sepa- 
rate nerves,  although  they  often  run  together,  and  that  they  usually 
enter  the  spinal  cord  at  different  levels. 

If  the  various  spinal  nerves  be  cut  singly  from  above  downward, 
and  the  effects  noted,  and  if  the  peripheral  ends  be  irritated  and  the 
effects  noted,  and  if  with  proper  care  the  results  be  analyzed  and  com- 
pared, it  will  be  found  that  the  anatomical  connections  of  the  seg- 
ments of  the  spinal  cord  with  the  sympathetic  ganglia,  which  are  so 
evident  at  each  level,  are  not  the  ones  by  which  physiological  impulses 
pass  out  at  that  level.  The  vaso-constrictors  of  the  head,  which  can 
be  traced  to  the  superior  cervical  ganglion,  do  not  come  from  the  upper 
cervical  region  of  the  cord,  as  might  be  supposed  from  the  connections 
of  that  ganglion,  but  from  the  first  three  dorsal  nerves.  They  reach 
the  ganglion  through  the  sympathetic  cord  in  the  neck,  having  trav- 
ersed the  inferior  cervical  ganglion  on  the  way.  There  are  vaso-con- 
strictors in  the  cranial  cavity  which  accompany  the  third,  fifth,  seventh, 
and  twelfth  nerves  to  the  eye,  face,  and  mouth.  It  is  still  undecided 
whether  they  originate  in  the  cord  and  medulla,  issue  in  the  spinal 
accessory  nerve,  and  with  it  enter  the  cranium  (Gaskell),  or  reach 
those  nerves  by  way  of  the  carotid  and  vertebral  plexus  from  the  infe- 
rior cervical  ganglion  (Dastre  and  Morat).  The  vaso-constrictors  of 
the  arm,  which  can  be  traced  to  the  inferior  cervical  and  upper  thoracic 
ganglia  and  to  the  thoracic  sympathetic  cord,  are  derived  from  the 
seven  upper  dorsal  nerves.  It  is  true  that  division  of  the  roots  of  the 
brachial  plexus  causes  a  slight  dilatation  of  the  vessels  of  the  arm,  but 
this  is  so  much  increased  when  the  dorsal  nerve-roots  are  divided  that 
it  is  evident  that  it  is  through  them  that  the  constrictor  fibres  chiefly  pass. 


PHYSIOLOGY.  921 

The  vaso-c^nstrictors  of  the  leg,  which  can  be  traced  into  the  second 
and  third  lumbar  ganglia  and  lumbar  sympathetic  cord,  are  derived 
from  the  five  lower  dorsal  and  first  lumbar  nerves,  and  only  join  the 
crural  and  sciatic  nerves  after  passing  through  the  abdominal  sym- 
pathetic. The  thoracic  viscera  are  probably  supplied  partly  through 
the  branches  of  the  inferior  cervical  and  thoracic  ganglia,  and  partly 
through  the  pneumogastric,  the  latter  statement  being  disputed  by 
Gaskell.  The  abdominal  viscera  are  supplied  partly  through  the 
splanchnic  nerves,  which  are  made  up  of  fibres  issuing  from  the  cord 
in  the  fifth  to  the  twelfth  dorsal  nerves  inclusive,  and  partly  through 
the  pneumogastric.  Thus  the  dorsal  region  of  the  cord  is  the  origin 
of  the  majority  of  vaso-constrictor  fibres  in  the  body. 

The  origin  of  the  vaso-dilators  is  also  to  be  traced  to  the  dorsal  cord. 
The  vaso-dilators  of  the  bucco-facial  region  come  from  the  second  to 
the  fifth  dorsal  nerves,  whence  they  pass  to  the  first  thoracic  ganglion, 
and  thence  by  the  annulus  of  Vieussens  into  the  cervical  sympathetic 
cord.  Those  of  the  eye,  head,  and  ear  come  from  the  same  ganglion, 
but  have  their  spinal  origin  in  the  eighth  cervical  and  first  dorsal  nerves. 
Those  of  the  arm  are  traced  into  the  upper  thoracic  sympathetic  cord, 
which  they  reach  from  the  five  upper  dorsal  and  last  cervical  nerves. 
Those  of  the  leg  are  traced  to  the  first  and  second  lumbar  ganglia  and 
the  lumbar  sympathetic  cord,  which  they  reach  from  all  the  dorsal 
nerves  from  the  sixth  downward.  Gaskell  holds,  however,  that  the 
vaso-dilators  of  the  extremities  pass  out  of  the  cord  in  the  cervical 
and  lumbar  plexuses  and  accompany  the  cere bro -spinal  nerves.  The 
vaso-dilators  of  the  thorax  and  abdomen  are  supposed  to  pass  in  the 
pulmonary  plexus  and  splanchnic  nerves,  but  this  is  not  yet  fully 
determined. 

Gaskell  believes  that  vaso-motor  nerves  can  be  distinguished  from 
motor  and  sensory  nerves  in  the  spinal  nerve-roots  by  the  smallness  of 
their  calibre.  He  finds  such  fine  fibres  only  in  the  spinal  nerve-roots 
between  the  second  dorsal  and  second  lumbar  segments  of  the  cord  (in 
the  dog),  and  in  the  three  upper  roots  of  the  spinal  accessory  nerve. 
According  to  his  account,  the  vaso-constrictors  issue  from  the  spinal 
cord  in  both  anterior  and  posterior  nerve-roots  as  medullated  fibres, 
and  pass  to  the  sympathetic  ganglia  lying  on  the  vertebrae  (proximal 
or  lateral  ganglia) ;  there  they  lose  their  medullary  sheath,  and  either 
end  in  cells  whence  new  fibres  issue,  or  more  probably  pass  directly 
onward  as  non-meduUated  fibres,  having  a  connection  with  the  uni- 
polar cells  of  the  ganglia  only  for  purposes  of  nutrition.  The  number 
of  fibres  issuing  from  any  one  ganglion  is  much  greater  than  the  num- 
ber entering  it  from  the  cord  ;  hence  it  is  supposed  that  each  medul- 
lated fibre  splits  up  into  a  group  of  non-medullated  fibres;  which  is 
possible,  as  the  researches  of  Ranvier  have  shown  that  each  axis- 
cylinder  is  made  up  of  numerous  fibrils.  Leaving  these  ganglia,  the 
nerves  pass  either  to  the  second  series  of  ganglia  (distal  or  collateral 
ganglia),  whence  they  issue  in  plexuses  to  enter  the  vessels,  or  to  the 
vessels  directly,  where  they  divide  in  plexuses.     It  is  in  the  meshes 


922 


VAHO-MOTOR    AND    TliOl'llW    NEUROSES. 


of  the  plexus  that  the  local  ganglia  of  the  vessel-walls  are  found.  The 
vaso-dilators  are  tliought  to  differ  from  the  vaso-constrictors  in  passing 
directly  to  the  distal  ganglia  as  meduUated  fibres,  not  being  connected 
in  any  way  with  the  proximal  ganglia.  Gaskoll  makes  no  statement 
regarding  their  limits  of  origin  from  the  cord,  except  to  state  that  the 
nervi  erigentes  issue  with  the  sacral  nerves.  He  agrees  with  the  view 
that  the  vaso-dilators  act  as  inhibitory  nerves  upon  the  local  ganglia. 
Vaso-motor  Reflexes.  —  Thus  far,  reference  has  been  made  only  to 
fibres  whose  direction  of  transmission  is  centrifugal,  and  whose  exit 
from  the  spinal  cord  is  by  the  anterior  nerve-roots.  There  are  other 
fibres,  however,  through  which  centripetal  impulses  pass,  and  these 
enter  the  spinal  cord  with  the  posterior  nerve-roots.  The  function  of 
these  fibres  is  to  transmit  sensory  impulses  inward  to  reflex  centres,  and, 
thus  set  in  action  motor  mechanisms  of  a  vaso-constrictor  or  dilator 
kind  whose  effects  are  produced  at  the  periphery.  There  are,  there- 
fore, vaso-motor  reflexes,  as  well  as  skin  and  tendon  reflexes,  whose 
centres  are  in  the  spinal  cord.  These  reflex  acts  may  be  excited  by 
impulses  reaching  the  centre  not  only  through  the  vaso-motor  centri- 
petal nerves,  but  also  through  the  sensory  nerves  of  the  cerebro-spinal 
system.  The  effect  of  changes  of  temperature  on  the  circulation  in 
the  skin  (if  the  right  hand  be  plunged  in  cold  water  there  is  a  fall  of 
temperature  in  the  left  hand),  the  effect  of  pain  upon  the  color  of  the 
face  and  the  size  of  the  pupil,  the  red  cheek  on  the  affected  side  in 
pneumonia,  the  occurrence  of  glycosuria  during  sciatica, — are  all  in- 
stances of  such  reflex  acts.  Many  vaso-motor  affections  are  produced 
by  irritation  causing  reflex  effects  at  a  distance  from  the  seat  of  irrita- 
tion— a  fact  always  to  be  kept  in  mind.  The  utility  of  counter-irri- 
tation to  the  surface  in  diseases  of  the  internal  organs  is  explained  by 
supposing  that  vascular  changes  are  produced  in  those  organs  through 
reflex  mechanisms  set  in  action  by  the  local  irritation.  While  some 
of  these  reflexes  may  have  their  central  mechanism  in  the  local  ganglia, 
it  is  probable  that  the  majority  are  to  be  traced  to  the  spinal  cord. 
It  is  believed  that  the  sympathetic  ganglia  are  not  the  seat  of  reflex 

centres. 

Fig.  319. 


Vaso-dilating  nerves  to  the  cutaneous 

vessels ■  • 

Vessels  of  the  skin  and  surface • 

Blister  applied  to  chest-wall ■ 

Thoracic  wall - 


_ Vaso-motor  centre. 


Afferent  nerves. 


Vaso-contracting  nerves  for  the  ves- 
sels of  the  lung. 
Vessels  of  the  lung. 


Diagram  to  represent  the  Mode  of  Action  of  Counter-irritants  applied  to  the  Chest  (Lauder  Brunton). 
The  Irritation  of  the  afferent  nerves  by  the  blister  on  the  chest  wall  gives  rise  to  a  vaso-constrictor 
reflex  in  ths  vessels  of  the  lung. 


Since  the  vaso-motor  nerves  are  connected  almost  exclusively  with 
the  dorsal  portion  of  the  spinal  cord,  it  is  very  natural  to  conclude  that 


PHYSIOLOGY.  923 

the  vaso-rootor  reflex  centres  are  situated  in  this  region ;  and  the 
hypothesis  has  been  advanced  by  Jacubovitch,  and  strongly  urged  by 
Gaskell,  that  the  cells  of  the  vesicular  columns  of  Clarke,  which  are 
peculiar  to  this  region,  are  the  seat  of  these  reflex  mechanisms.  This 
hypothesis  gains  some  support  from  the  pathology  of  syringo-myelia. 
In  this  disease  the  gray  matter  surrounding  the  central  canal  and  the 
vesicular  columns  is  destroyed.  The  characteristic  symptoms  are 
vaso-motor  and  trophic  disturbances,  consisting  of  changes  in  the  vas- 
cular tone,  changes  of  local  temperature,  and  various  eruptions,  in  some 
cases  going  on  to  ulceration  in  the  skin  and  mucous  membranes.  It 
is,  however,  undecided  whether  the  vaso-motor  centres  of  the  cord  are 
limited  to  the  columns  of  .Clarke,  or  are  situated  in  the  gray  matter 
surrounding  the  central  canal,  since  both  these  parts  are  destroyed  in 
this  disease.  That  they  are  not  located  in  the  anterior  or  posterior 
gray  cornua  is  determined  by  the  fact  that  diseases  limited  exclusively 
to  these  areas  do  not  cause  vaso-motor  disturbances.  The  situation  of 
the  various  reflex  centres  for  the  various  parts  of  the  body  is  at  differ- 
ent levels  of  the  cord,  as  has  been  determined  by  the  experiments 
already  cited  to  establish  the  level  of  origin  of  the  vaso-motor  nerves. 
The  exact  location  of  the  vaso-constrictor  and  vaso-dilator  reflex 
centres  for  definite  parts  is  yet  to  be  ascertained. 

Vaso-motor  Tracts.  —  These  reflex  centres  are  connected  with  the 
medulla  by  tracts  which  lie  in  the  lateral  columns  of  the  spinal  cord, 
although  it  is  not  determined  in  which  part  of  these  columns.  It  is 
not  possible  as  yet  to  separate  the  constrictors  from  the  dilators  in  this 
tract,  nor  to  determine  whether  it  transmits  impulses  in  both  directions 
or  only  from  above  downward.  Nor  is  the  course  of  associating  fibres 
between  reflex  centres  at  different  levels  known.  In  cases  of  trans- 
verse myelitis  the  control  of  the  medulla  is  removed  from  the  vascular 
centres  below  the  lesion,  and  the  lack  of  vascular  tone  seen  in  the 
paralyzed  limbs,  together  with  the  susceptibility  to  local  irritation,  is 
the  result  of  this  division  of  the  vaso-motor  tracts. 

Medullary  Centres.  —  It  has  been  stated  already  that  a  general  vaso- 
motor centre  with  both  constrictor  and  dilator  powers  is  situated  in 
the  medulla.  This  lies  in  two  divisions  on  each  side  of  the  middle  line, 
in  or  just  beneath  the  floor  of  the  fourth  ventricle,  from  the  calamus 
scriptorius  up  to  the  level  of  the  sixth  nerve-nucleus.  Each  division 
governs  the  vascular  tone  of  its  own  side  of  the  body,^  and  lesions  in 
its  region  in  man  prod  ice  unilateral  vaso-motor  symptoms.^  This 
centre  can  be  excited  to  reflex  action  by  strong  irritation  locally  or 
through  the  blood,  in  which  case  a  general  constriction  or  dilatation 
of  the  vessels  of  the  entire  body  will  ensue.  It  seems  probable,  how- 
ever, that  the  general  centre  in  the  medulla  is  made  up  of  a  number 
of  special  centres,  each  of  which  governs  a  definite  set  of  organs.  The 
vascular  tone  of  the  thoracic  and  abdcmiinal  vi'^cera  is  certainly  regu- 

^OwHJanikow,  Ar()eiten  auH  d.  Pliysiol.  Tnstit.  zu  Leipzie;,  1871. 
*M.  A.  Htiirr,  "  Sensory  Tract  in  Central  Nervous  Kystein,"  Journ.  Nerv.  and  Ment. 
Dis.,  July,  1884,  pp.  ;«Ki-398. 


9^4  VASO-MOTOR   AND    TROPHIC    NEUROSES. 

lated  by  a  series  of  such  centres.  Brown-S6quard  and  Schiff  have 
produced  hemorrhages  in  the  lungs,  pleura,  stomach,  intestines,  and 
kidneys  at  different  times  by  destructive  lesions  of  the  medulla,  and 
the  well-known  experiments  of  Bernard  in  which  by  puncture  of  the 
medulla  local  hyperaemia  of  the  liver  or  kidneys  was  caused,  producing 
glycosuria  or  polyuria,  confirm  this  view.  Lesions  of  these  parts  in 
man  produce  similar  effects.  Charcot  has  shown  that  in  cerebral 
hemorrhage  ecchymoses  may  be  found  in  the  stomach,  pleura,  and 
endocardium,  and  that  pneumonia  is  especially  frequent  upon  the  par- 
alyzed side.  De  Jonge '  has  been  able  to  collect  thirteen  cases  of  dia- 
betes mellitus  in  which  a  lesion  of  the  medulla  (hemorrhage  or  tumor) 
was  found  after  death ;  and  Flatten^  has  proven  the  existence  of  sim- 
ilar lesions  in  diabetes  insipidus.  The  connection  of  these  centres  with 
the  liver  and  kidneys  has  also  been  traced.^  The  medulla  con- 
tains a  special  centre  for  the  vaso-motor  nerves  of  the  abdomen,  which 
are  in  the  domain  of  the  splanchnic  nerves.  This  centre  is  excited 
reflexly  by  impulses  reaching  it  through  the  depressor  nerve  of  Cyon 
from  the  heart ;  so  that  when  that  organ  is  overburdened  it  may  be 
relieved  by  a  fall  of  arterial  pressure  produced  by  dilatation  of  the 
abdominal  vessels.  Whether  the  connection  of  the  medulla  with  the 
centres  in  the  semilunar  ganglion  which  preside  directly  over  these 
vessels  is  made  by  way  of  the  spinal  cord  or  by  way  of  the  pneumo- 
gastric  nerve  is  still  undetermined,  though  the  researches  of  Gaskell 
favor  the  former  view.  Gastric  and  intestinal  disturbances  are  certainly 
produced  by  nervous  lesions  in  the  medulla,  but  whether  they  are  due  to 
vascular  changes  is  uncertain.  The  vomiting  of  mucus  and  blood,  and 
the  large  watery  evacuations  which  accompany  mental  shock  or  anxi- 
ety, as  well  as  the  polyuria  associated  with  mental  effort,  have  been 
ascribed  to  irritation  of  local  centres  in  the  medulla  governing  the 
gastro-intestinal  and  urinary  organs  by  impulses  received  from  the 
cortex  above.  The  spleen  is  under  the  control  of  vaso-motor  centres, 
since  section  of  the  splenic  branches  of  the  semi-lunar  ganglia  will 
produce  a  great  enlargement  of  the  organ,  and  irritation  of  the  cut  end 
of  these  branches  will  produce  contraction.*  The  medulla  also  con- 
tains a  vaso-dilator  centre  for  the  erectile  tissues  of  the  genital  organs, 
irritation  of  which  by  mental  action  or  local  disease  causes  impulses 
to  pass  to  the  nervi  erigentes  by  way  of  the  spinal  cord,  resulting  in 
a  flow  of  blood  to  the  parts.  Although  a  centre  has  been  thought  to 
exist  controlling  the  circulation  in  the  lungs,  whose  paralysis  has  been 
supposed  to  explain  the  occurrence  of  sudden  pulmonary  cedema  with- 
out other  known  cause,  no  definite  facts  regarding  it  are  known.  That 
the  action  of  the  heart  is  under  the  control  of  the  medulla  is  a  fact  too 
well  known  to  require  more  than  a  mention.  The  physiology  of  the 
nervous  control  of  the  heart  cannot  be  discussed  here. 

'Arch.  f.  Psych.,  xiii.  ^ Ibid. 

^See  Tyson,  "Diabetes  Mellitus,"  Pepper's  System  of  Medicine,  vol.  i.,  p.  195; 
Edes,  "Diabetes  Insipidus,"  ibid.,  vol.  i.,  p.  30. 

*Tarchanoff,  Pfliiger's  Arch.,  viii.,  p.  97;  Boss,  Diseases  of  the  Nervous  System, 
vol.  i.,  p.  225. 


SYMPTOMS.  925 

While  th^se  medullary  centres  are  certainly  influenced  by  impulses 
reaching  them  from  the  cerebral  hemispheres,  as  is  evident  from  the 
vaso-motor  symptoms  produced  by  mental  action  —  e.  g.  pallor  from 
fright,  blushing,  etc.  —  it  is  impossible  to  state  in  what  portion  of  the 
hemispheres  in  man  the  higher  vaso-motor  centres  lie.  Eulenburg 
and  Landois  locate  them  in  the  motor  area  in  animals.^  They  are 
certainly  beyond  control  of  the  will,  and  are  wholly  reflex  in  their 
action,  a  purely  mental  act  in  this  case  being  the  excitant  of  a  purely 
physical  result.^ 

Pathogenesis.  —  From  this  review  of  the  physiology  of  the  vaso- 
motor system  it  becomes  evident  that  disturbances  of  vascular  tone 
may  be  produced  by  many  different  causes  acting  upon  many  various 
parts.  They  may  be  due  to  local  affections  of  the  part  in  which  the 
symptoms  are  present,  as  in  the  case  of  erythema  after  burns  or  frost- 
bite, or  congestion  of  any  organ  after  injury.  They  may  be  due  to 
affections  of  the  vaso-motor  nerves  passing  to  the  part  affected,  as  in 
the  case  of  vascular  changes  due  to  peripheral  nerve  lesions.  They 
may  be  due  to  affections  of  the  sympathetic  ganglia  connected  with  the 
part  affected,  as  in  the  case  of  migraine,  sudden  flushing  of  one  ear, 
certain  cases  of  polyuria,  and  Basedow's  disease.  They  may  be  due 
to  lesions  in  the  spinal  cord  affecting  the  vaso-motor  centres  or  com- 
pressing the  nerve-roots  on  their  way  to  and  from  the  sympathetic 
ganglia,  as  is  the  case  in  the  various  forms  of  myelitis  and  in  Raynaud's 
disease  or  symmetrical  gangrene,  and  in  meningitis,  tumors  of  the 
cord,  or  Pott's  disease.  They  may  also  be  caused  by  such  conditions 
in  the  cord  as  cut  off  the  vaso-motor  centres  from  the  medullary 
centres,  such  as  transverse  myelitis  from  compression  or  traumatism. 
They  may  be  due  to  lesions  of  the  medulla  oblongata,  as  is  seen  in 
some  cases  of  polyuria  and  glycosuria,  and  in  cases  of  universal 
erythema  following  acute  fevers.  They  may  be  due  to  diseases  of  the 
cerebral  hemispheres,  as  is  evident  from  the  vaso-motor  symptoms 
occurring  in  hemiplegia  and  hysteria.  Finally,  they  may  be  of  a 
reflex  origin,  dependent  upon  some  obscure  source  of  irritation  in  a 
part  quite  distant  from  the  region  in  which  the  symptoms  appear. 

Diagnosis.  —  The  diagnosis  of  the  seat  of  the  lesion  in  many  cases 
of  vaso-motor  neurosis  may  be  made  if  the  organ  or  the  exact  limitation 
of  the  area  affected  be  ascertained,  and  the  history  of  the  case,  together 
with  the  concurrent  symptoms  of  other  kinds,  be  considered.  In  some 
cases  no  organic  cause  can  be  found,  and  in  these  a  reflex  cause  should 
be  diligently  searched  for. 

Symptoms.  —  A  vaso-motor  affection  may  manifest  itself  either  by 
a  spasm  or  a  paralysis  of  the  vessels.  In  angiospasm  the  part  affected 
becomes  pale,  and  irritation  no  longer  causes  a  vaso-motor  reflex.     It 

'Arch.  f.  Path.  Anat.,  VA.  Ixviii.,  p.  245. 

*  In  afMition  to  th<!  articles  already  cited  the  reader  is  referred  to  Landois's  Physiol- 
of(y,  to  Duval's  article,  "  Vaso-nioteurs,"  in  the  Dictionnaire  de  M(!decine  et  de  Chir- 
iiririe,  vol.  xxxviii.,  for  a  .summary  of  vaso-motor  physiolofjjy,  and  to  Gerhardt's 
"  J'(;l)er  An^io-neiirosen,"  Volksmann's  Sammlnnj^  klin.  Vortrilf^e,  No.  209,  and  to 
Ga-skoll's  researches,  puhlished  in  the  Journal  of  Physiology,  1880. 


926  VA80-M0T0R    AND    TROPHIC    NEUROSES, 

looks  shrunken,  and  if  the  skin  over  it  is  loose  it  may  be  thrown  into 
folds  or  shrivelled,  presenting  the  appearance  seen  in  the  hands  after 
long  immersion  in  hot  water.  The  lack  of  blood  in  the  part  arrests 
the  processes  of  metabolism  which  are  normally  constant,  and  if  the 
condition  continues  this  may  result  in  such  a  disturbance  of  nutrition 
tliat  ulceration,  or  even  gangrene,  may  ensue  The  local  anaemia, 
combined  with  the  cessation  of  metabolism,  produces  a  fall  of  tem- 
perature in  the  affected  part,  which  is  then  more  easily  affected  by  the 
temperature  of  the  air  than  in  a  normal  state,  so  that  exposure  to  cold 
is  very  liable  to  cause  freezing.  These  conditions  necessarily  produce 
an  impairment  of  function,  so  that  if  the  affection  is  located  in  the 
extremities,  as  the  fingers,  they  are  soon  rendered  useless.  The  term 
digiti  mortal  has  been  applied  to  this  state.  In  the  surface  of  the  body 
angiospasm  causes  cutis  anseriua,  pallor,  numbness,  tingling,  slight 
anaesthesia,  and  analgesia.  If  it  occurs  in  a  limb,  the  finer  motions 
are  imperfectly  performed,  and  in  time  the  nutrition  of  the  muscles 
may  be  so  impaired  as  to  produce  atrophy  and  paresis.  It  may  even 
lead  to  gangrene.  Nothnagel  has  recorded  '  five  cases  of  sciatica  in 
which  the  pain  produced  a  reflex  spasm  of  the  vessels  of  the  leg, 
which,  persisting,  resulted  in  partial  paralysis,  atrophy,  lowering  of 
temperature,  pallor,  and  sensory  disturbances.  Ross  mentions  the 
sudden  appearance  of  circumscribed  patches  on  the  hands  and  fore- 
arms of  washerwomen,  in  which  there  is  a  pallor,  coldness,  and  partial 
anaesthesia.  These  may  be  limited  to  the  distribution  of  a  single 
nerve,  and  may  be  accompanied  by  trophic  affections. 

Spasm  of  the  veins  may  occur  as  well  as  of  the  arteries,^  or  inde- 
pendently of  them.  In  the  latter  case  the  blood  will  not  pass  out  of 
the  capillaries.  The  part  will  then  be  blue,  swollen,  oedematous,  and 
painful ;  the  temperature  will  be  lowered  by  increased  radiation  of 
heat,  and  all  the  sensations  and  functions  be  impaired  in  greater  or  less 
degree.  If  this  continues,  nutrition  may  suffer,  and  in  the  end  gan- 
grene develop,  which  will  take  its  course  and  lead  to  the  throwing  off 
of  the  part.  Grainger  Stewart  has  described  such  a  condition  occurring 
in  both  hands  and  feet.^  It  may  be  likened  to  a  severe  form  of  Ray- 
naud's disease. 

Angio-paralysis  is  more  frequent  than  angiospasm,  and  may  be  due 
either  to  paralysis  of  the  vaso-constrictors  or  to  excitement  of  the  vaso- 
dilators. It  shows  itself  by  a  bright- red  or  mottled  appearance  of  the 
skin,  and  increase  of  local  temperature,  and  more  rapid  processes  of 
nutrition,  together  with  an  increase  of  secretion  if  the  part  is  a  gland  or 
a  mucous  membrane,  and  an  increase  of  sweat  if  it  is  the  skin.  In  the 
latter  case  an  increased  sensitiveness  to  changes  of  temperature,  a  sub- 
jective sensation  of  heat,  and  hypersesthesia  and  hyperalgesia  may  occur. 
The  hypersesthesia  on  the  paralyzed  side  which  is  present  in  hemipara- 
plegia  spinalis  is   ascribed   to  the  vaso-motor  paralysis.     But  these 

*Arch.  f.  Psych.,  v. 

^  Weiss,  "  Symmetrische  Gangran,  "  Wiener  Klinik,  1882. 

■5  .Grainger  Stewart,  An  Introduction  to  the  Study  of  Nervous  Diseases,  p.  138.. 


SYMPTOMS.  927 

symptoms  soon  give  place  to  others.  The  dilatation  of  the  vessels, 
which  at  first  caused  an  increased  flow  of  blood  to  the  part,  produces  a 
slowing  of  the  blood-current  in  the  part,  just  as  a  river  runs  less  rapidly 
where  it  becomes  wider.  The  slowing  of  the  current  in  the  skin 
allows  of  a  more  complete  cooling  of  the  part  as  the  radiation  of  heat 
and  the  evaporation  of  moisture  are  increased,  and  the  slowness  of  the 
renewal  of  blood  impairs  the  processes  of  nutrition,  so  that  to  the  first 
stage  of  redness,  heat,  and  increased  metabolism  there  ensues  a  stage 
of  blueness,  cold,  and  defective  nutrition,  and  the  function  of  the  part 
may  be  impaired.  In  this  stage  it  usually  presents  a  mottled  appear- 
ance, and  may  be  slightly  swollen  and  cedematous,  and  the  continued 
increase  of  perspiration  gives  it  a  clammy  coldness  to  the  touch.  In 
all  of  these  conditions  severe  pain,  sometimes  of  a  burning  character, 
is  a  very  distressing  symptom  (causalgia).  These  conditions  are  seen 
in  peripheral  nerve-lesions,  and  give  rise  to  the  appearances  which 
have  been  so  admirably  described  by  Weir  Mitchell.^ 

A  peculiar  combination  of  symptoms  may  be  mentioned  here,  to 
which  Weir  Mitchell  has  given  the  name  of  erythromelalgia.^  This 
symptom  begins  with  tenderness  and  pain  in  the  soles  of  the  feet, 
which  are  soon  followed  by  a  marked  distension  of  the  capillary  vessels. 
The  congestion  is  attended  by  a  sensation  of  burning  pain  similar  to 
that  produced  by  a  blister.  The  surface  is  at  first  of  a  dull  dusky-red 
color  ;  later  it  appears  purple.  The  redness  is  not  uniformly  distrib- 
uted over  the  sole,  but  occurs  in  patches  of  irregular  shape,  being 
especially  frequent  over  prominent  parts  exposed  to  pressure  and  fric- 
tion, and  the  attacks  seem  to  be  brought  on  by  long  standing  or  walk- 
ing. At  first  there  is  a  rise  of  temperature  in  the  affected  surface,  the 
arteries  pulsate  visibly,  the  veins  are  swollen,  and  there  may  be  some 
oedema.  Later,  the  foot  is  cold  and  pale.  Sensations  of  touch  and 
temperature  are  normal,  but  the  part  is  so  extremely  tender  that  walk- 
ing is  impossible.  There  is  no  paralysis.  One  or  both  feet  may  be 
affected,  but  the  patches  of  redness  are  rarely  symmetrical.  The  hands 
are  occasionally  affected.  The  condition  may  occur  in  paroxysms  or 
may  remain  for  some  time.  It  resists  all  known  methods  of  treat- 
ment, although  appUcations  of  cold  relieve  the  burning  pain  to  some 
extent  and  the  tenderness  enforces  rest. 

With  angio-paralysis  may  be  classed  the  taches  c^rebrales  of  Trous- 
seau, no  longer  considered  diagnostic  of  meningitis,  but  denoting  a 
weakened  condition  of  vaso-constrictor  action  in  the  local  ganglia  of 
the  vessel-wall,  which  may  occur  upon  local  irritation  of  the  skin  in 
any  severe  disease  affecting  the  nutrition  of  the  general  nervous 
system. 

Actual  rupture  of  the  capillaries  in  the  course  of  vaso-motor  diseases 
is  rarely  obs(;rved,  although  the  stigmata  appearing  in  hysterical  and 
cataleptic  patients  may  be  ascribed  to  this  cause.  In  this  connection 
tabetic  ecchymoses  may  be  mentioned,  which  appear  suddenly  with- 

^  Injuries  of  Nerves. 

^Ar'ner.  Jour,  of  the  Med.  Sci.,  July,  1878, 


928  VASO-MOTOIi    AND    TROPHIC    NEUROSES. 

out  local  injury,  and  resemble  an  ordinary  bruise,  running  a  similar 
course.     They  occur  only  in  the  course  of  locomotor  ataxia.* 

In  addition  to  these  forms  of  vaso-motor  affections  there  is  a  condi- 
tion of  instability  of  vascular  tone  which  manifests  itself  by  sudden 
transient  changes  in  the  circulation  of  various  organs.  This  is  a  func- 
tional affection,  usually  due  to  malnutrition.  It  is  seen  in  many  cases 
of  neurasthenia  and  hysteria,  and  manifests  itself  by  sudden  flushes  or 
pallor,  alternations  of  heat  and  cold,  local  sweating,  attacks  of  mental 
confusion,  and  inability  to  use  any  organ  continuously  from  disturb- 
ance of  the  power  of  the  vaso-dilators  to  maintain  a  condition  of  func- 
tional hypersemia.^  Little  is  actually  known  about  the  causes  of  this 
state  of  the  vascular  system,  although  much  has  been  written  about  it. 

Intermittent  claudication  is  a  symptom  of  sudden  giving  out  of  one 
leg,  causing  the  patient  to  limp  or  to  fall,  due  to  a  spasm  of  the  large 
vessels.  This  is  usually  due  to  arterio-sclerosis,  but  may  be  a  func- 
tional neurosis. 

Many  functional  derangements  of  the  internal  viscera  have  been 
ascribed  to  such  vaso-motor  instability  with  more  or  less  probability,^ 
but  hypothesis  of  this  kind,  however  plausible,  is  evidently  beyond 
confirmation.  It  is  especially  in  affections  of  this  kind  that  causes  of 
reflex  irritation  are  to  be  carefully  sought.  Cutaneous  angio-neuroses, 
such  as  have  just  been  described,  may  affect  any  part  of  the  body. 
They  usually  appear  suddenly,,  producing  much  discomfort  and  an 
impairment  of  function  in  the  part  if  it  is  an  extremity.  They  dis- 
appear as  rapidly  as  they  come.  The  duration  of  such  attacks  varies 
from  a  few  minutes  to  several  days.  They  are  very  liable  to  recur. 
If  it  is  the  vessels  under  the  control  of  the  cervical  sympathetic  which 
are  affected,  the  symptoms  will  be  those  of  migraine  or  of  lesion  of  the 
ganglia.  If  it  is  the  vessels  in  the  extremities  which  are  involved, 
the  condition  of  digiti  mortui  or  erythromelalgia  or  symmetrical  gang- 
rene may  be  produced. 

A  singular  epidemic  occurred  in  France  in  1828  and  1830  which 
was  termed  acrodynia.  Many  persons  were  suddenly  seized  with 
vomiting  and  purging,  and  soon  after  the  onset  the  extremities  became 
red  or  mottled  in  blotches,  swollen  and  cedematous,  and  hot,  painful, 
and  tender.  The  attacks  lasted  from  a  few  days  to  two  months,  and 
during  this  time  the  skin  became  thick  and  hard,  the  muscles  weak 
and  subject  to  spasms,  and  the  general  health  was  impaired.  Relapses 
occurred  in  many  cases,  but  all  finally  recovered,  and  hence  the  exact 
nature  of  the  disease  was  not  ascertained. 

Course.  —  In  any  case  of  vaso-motor  neurosis  the  course  of  the 
disease  and  its  termination  will  depend  chiefly  upon  its  cause.  If 
the  cause  is  some  permanent  lesion  of  the  nervous  system,  the  condi- 
tion will  remain,  and  in  this  case  the  termination  will  depend  upon 
the  severity  of  the  symptoms.     Angiospasm  may  be  so  severe  as  to 

'  Straus,  Arch,  de  Neurologie,  tome  i. ,  p.  536. 

''Angel,  Arch,  fiir  Psychiatrie,  xv.,  618. 

^  Fox,  The  Influence  of  the  Sympathetic  System  in  Disease,  London,  1885. 


PLATE  XXX 


1    .;. 


Nasvus  in  the  Distribution  of  the  Roots  of  the  Fifth,  Seventh,  and  Eighth 
Cervical  Nerves  and  First,  Second,  and  Third  Dorsal  Nerves.  A  similar  distri- 
bution of  herpes  has  been  observed. 

Icon,  de  Salp.,  xxii    486. 


TREATMENT.  929 

lead  to  gangrene  and  the  separation  of  the  pan  affected,  or  may  be  so 
slight  as  to  cause  only  subjective  discomfort  and  a  little  pallor. 
Angio-paralysis  may  lead  to  an  extreme  degree  of  congestion,  which 
is  attended  by  heat  and  pain  at  first,  later  by  parsesthesia  and  coolness, 
with  increased  liability  of  the  part  to  be  affected  by  changes  in  the 
surrounding  air.  This  stage  is  succeeded  by  one  of  less  marked  dila- 
tation of  the  vessels  and  a  spontaneous  partial  recovery,  although  the 
more  moderate  symptoms  may  continue  indefinitely  and  seriously  im- 
pair the  function  of  the  part.  If  the  cause  is  a  temporary  derange- 
ment of  function  in  the  vascular  mechanism,  is  reflex  irritation  which 
can  be  removed,  or  is  a  curable  organic  disease,  the  symptoms  will 
subside  rapidly  or  gradually  and  perfect  recovery  may  follow.  If  the 
condition  is  one  of  irritability  in  the  vaso-motor  centres,  producing 
alternations  of  flushing  or  pallor,  such  as  is  observed  in  nervous 
exhaustion,  it  may  recur  irregularly  for  a  considerable  length  of  time 
until  the  causative  condition  can  be  removed. 

Prognosis.  —  The  prognosis  must  be  determined  in  each  case  by  a 
consideration  of  the  cause  of  the  affection,  of  the  nature  of  the  symp- 
toms, of  the  severity  of  the  disease,  and  of  the  possibility  of  success 
in  both  symptomatic  and  causative  treatment.  In  the  angio-paralytic 
cases  an  eventual  spontaneous  relief  from  much  of  the  discomfort  may 
be  promised,  although  the  duration  of  the  symptoms  cannot  be 
predicted. 

Treatment. — Treatment  must  be  directed  primarily  to  removing 
or  diminishing  the  severity  of  the  cause.  A  review  of  the  section  on 
Pathogenesis  will  indicate  how  wide  a  field  this  may  include,  and  the 
reader  must  be  referred  to  the  special  articles  which  are  alluded  to  in 
that  place  for  therapeutic  measures.  Special  diligence  is  to  be  shown 
in  searching  for  a  source  of  reflex  irritation.  When  the  cause  cannot 
be  reached,  and  when  the  symptoms  are  of  such  severity  as  to  demand 
immediate  attention,  treatment  may  be  directed  to  them. 

In  all  conditions  of  vaso-motor  disease  it  is  important  to  shield  the 
part  from  external  injury  ;  for  if  the  vessels  are  dilated  they  are  liable 
to  rupture,  and  any  abrasion  of  the  surface  may  produce  serious  inflam- 
mation and  ulceration ;  and  if  the  vessels  are  contracted  any  injury 
will  be  repaired  slowly  and  imperfectly  on  account  of  the  anaemia,  and 
may  even  hasten  the  approach  of  gangrene. 

Perfect  rest,  bandaging  with  cotton,  and  even  the  application  of  a 
light  splint  to  the  extremities  will  be  advisable  in  cases  of  angiospasm. 
It  is  desirable  to  retain  the  animal  heat,  inasmuch  as  its  supply  is 
deficient.  In  angio-paralysis  rest  in  a  somewhat  elevated  position  and 
applications  of  mild  evaporating  lotions  are  indicated  in  the  early 
stage;  later,  the  limb  may  be  bandaged.  It  is  not  advisable  to  attempt 
by  tight  bandaging  to  counteract  the  effect  of  the  vascular  paralysis, 
for  the  nutrition  of  the  limb  is  liable  to  suffer  and  gangrene  may  be 
induced. 

Massage  of  a  part  affected  with  vaso-motor  symptoms  is  of  great 
service,  since  the  circulation  can  be  increased  in  the  veins,  and  thus 
69 


930  VASO-MOTOR    AXD    TROPHIC    NEUROSES. 

indirectly  in  the  capillaries,  and  the  nutrition  of  the  part  can  thus  be 
favored.  It  is  more  efficacious  in  angio-paralysis  than  in  angiospasm. 
Too  rough  rubbing  is  of  course  to  be  avoided,  lest  the  skin  be  injured. 
All  counter-irritation  is  to  be  strictly  forbidden. 

Electricity  has  been  used  with  varying  results.  According  to  Erb, 
moderate  faradic  applications  contract  the  vessels ;  strong  faradic  ap- 
plications, especially  with  the  brush,  dilate  the  vessels.  The  galvanic 
current  at  first  contracts  the  vessels,  but  this  is  followed  by  a  secondary 
dilatation,  which  will  be  greater  and  occur  more  rapidly  the  stronger 
the  current  used.  Cathodal  closures  contract  the  vessels ;  the  anodal 
continuous  current  dilates  them  widely.  Stabile  continuous  currents 
through  a  nerve  dilate  the  vessels  which  the  nerve  supplies.  Inasmuch 
as  vaso-constrictors  and  vaso-dilators  pass  together  in  many  nerves, 
and  are  found  together  in  all  parts,  it  is  impossible  to  apply  electricity 
to  either  alone.  In  those  cases,  therefore,  in  which  it  has  been  ascer- 
tained which  set  of  vaso-motors  is  aft'ected,  it  is  not  always  possible  to 
produce  a  direct  effect  upon  that  set  by  electrical  treatment.  Erb 
recommends  in  conditions  of  vaso-motor  spasm  a  trial  of  the  galvanic 
current,  the  cathode  on  an  indifferent  point,  the  anode  being  applied 
over  the  vaso-motor  centres  governing  the  part,  and  also  over  the  area 
of  the  body,  which  is  affected,  and  held  there  while  a  moderate  con- 
tinuous current  is  passing,  interruptions  being  avoided  ;  or,  the  cathode 
being  placed  on  the  neck,  the  anode  may  be  applied  to  the  nerves 
passing  to  the  affected  part ;  or  a  strong  continuous  current  may  be 
sent  through  the  nerve,  its  direction  being  changed  several  times  dur- 
ing a  moderately  long  application.  Finally,  the  faradic  brush  applied 
to  the  part  or  a  strong  faradic  current  sent  through  its  nerve  may  relax 
the  spasm.  In  any  case,  all  these  methods  should  be  tried  before 
electrical  treatment  is  abandoned. 

In  vaso-motor  paralysis  other  methods  are  used.  The  cathode  is 
placed  on  the  part  congested,  and  a  weak  galvanic  current  is  employed 
with  frequent  interruptions  or  even  with  changes  of  the  pole ;  or  the 
cathode  may  be  moved  about  upon  the  reddened  skin  while  a  mild 
continuous  current  is  passing.  A  very  weak  faradic  current  with  wet 
electrodes,  or  even  a  weak  faradic  current  applied  with  a  brush,  may 
be  of  service.     Here,  again,  various  methods  may  be  tried. 

If  the  extremities  are  affected,  it  may  be  well  to  immerse  them  in  a 
basin  of  water  which  is  connected  with  one  pole  of  the  battery,  and  the 
current  directed  in  the  manner  just  described,  according  to  the  case. 
It  must  be  confessed  that  no  definite  results  can  be  predicted  from  the 
use  of  electricity  in  these  cases,  and  much  more  experience  is  needed 
before  definite  rules  can  be  laid  down.  The  records  show  that  in 
apparently  similar  cases  opposite  methods  of  application  have  produced 
favorable  effects,  while  in  other  cases  all  methods  have  failed.  Too 
much  reliance  should  not  be  placed  in  electrical  treatment.  Erythro- 
melalgia  is  an  obstinate  affection,  and  symptomatic  treatment,  directed 
chiefly  to  quieting  the  pain  by  opium  and  allaying  the  sensation  of 
burning  by  cool  baths,  must  be  resorted  to. 


TREATMENT.  931 

Internal  remedies  may  be  tried  appropriate  to  the  condition  present. 
In  angiospasm  nitrite  of  amyl  inhaled,  or  nitro-glycerin  yi  ^  gr  t.  i.  d., 
may  give  considerable  relief,  although  both  of  these  drugs  are  to  be 
used  with  caution.  Chloral  hydrate  is  also  of  some  service,  and  where 
the  patient  is  in  pain  and  suffers  from  insomnia  this  may  fulfil  several 
indications.  In  angio-paralysis  ergot  has  been  used  with  advantage. 
Oxygen  inhalations  are  of  service.  Chloride  of  potassium  may  also 
be  tried.  It  is  evident,  however,  that  such  remedies,  acting  as  they 
do  upon  the  general  arterial  system,  are  not  to  be  depended  upon  in 
the  treatment  of  local  conditions,  since  they  have  no  selective  action 
upon  the  affected  part.  The  majority  of  the  drugs  known  as  sedatives 
and  antispasmodics  have  been  used  in  these  conditions,  but  the  records 
of  individual  cases  show  that  they  are  not  of  much  avail. 


CHAPTER  LVII. 

SYMMETRICAL  GANGRENE. 

Synonyms.  —  Local  asphyxia,  Asphyxie  locale,  Raynaud's  disease  ; 
Syrametrische  Gangran. 

Definition.  —  Symmetrical  gangrene  is  an  affection  of  the  nervous 
system  characterized  by  arterial  or  venous  spasm  appearing  in  sym- 
metrical parts  of  the  body,  especially  in  the  phalanges  of  all  the  extrem- 
ities, which  may  result  in  trophic  changes  or  in  gangrene.  There  are 
various  stages  in  the  disease,  which  have  given  rise  to  the  various  names 
by  which  it  is  known.  The  stage  of  local  syncope,  in  which  there  occurs 
a  moderate  contraction  of  the  arterioles  and  consequent  pallor  of  the 
part,  may  be  followed  by  a  stage  of  local  asphyxia,  in  which  the  com- 
plete contraction  of  the  arterioles  cuts  off  entirely  the  supply  of  arterial 
blood,  and  the  regurgitation  of  venous  blood  produces  cyanosis  of  the 
part ;  and  this,  if  continued,  may  result  in  the  gangrene  of  the  part, 
which  is  then  thrown  oif.  Instead  of  a  condition  of  local  asphyxia, 
there  may  be  a  spasm  of  the  smaller  veins,  resulting  in  a  local  erythema, 
which  may  go  on  to  capillary  stasis  and  then  to  gangrene.  The  spasm 
of  the  vessels  may  cease  at  any  stage  as  suddenly  as  it  began ;  and  if 
this  occurs  in  the  first  or  second  stage,  no  gangrene  results. 

Symptoms.  —  The  disease  begins  suddenly  in  all  cases,  and  the  con- 
stitutional symptoms  are  less  prominent  than  the  local  ones.  In  some 
cases  there  are  noticed  a  certain  degree  of  mental  disturbance,  a  condi- 
tion of  depression  with  a  tendency  to  sigh  and  cry  without  cause,  dis- 
turbed sleep  with  unpleasant  dreams,  irritability,  and  headache.  A  loss 
of  appetite  and  disorders  of  digestion  may  follow,  and  then  the  local 
symptoms  appear.  In  other  cases,  which  seem  to  be  the  majority,  the 
local  condition  develops  without  any  such  premonitory  disturbances 
of  the  nervous  and  digestive  systems,  although  these  may  ensue.  The 
local  symptoms  first  noticed  may  be  parsesthesise  or  pain  in  all  the 
extremities,  usually  limited  to  the  tips  of  the  fingers  and  the  toes. 
These  are  continuous  and  severe,  and  are  immediately  followed  (and 
occasionally  preceded)  by  an  appearance  of  ischsemia  or  of  cyanosis  or 
of  erythema,  in  the  order  of  frequency  named. 

(1)  The  fingers  may  look  pale  and  dead,  presenting  the  appearance 
of  the  so-called  digiti  mortui,  and  may  be  cold,  painful,  and  anaesthetic. 
If  this  condition  is  moderate  in  degree,  a  certain  amount  of  blood  will 
continue  to  flow  through  the  contracted  arterioles,  and  then  it  corre- 
sponds to  the  description  given  by  Raynaud  of  syncope  locale.  If  it 
is  extreme,  the  part  may  be  wholly  deprived  of  arterial  blood,  and 
then  a  true  local  asphyxia  is  present.     In  this  stage  the  oatients 

932 


SYMPTOMS.  933 

usually  suffer  considerably,  although  some  do  not  complain  of  pain 
until  the  next  stage.  The  ischseraia  is  attended  with  an  impairment 
of  sensation  to  touch,  temperature,  and  pain,  and  finer  motions  become 
clumsy  on  account  of  the  subjective  numbness  and  actual  anaesthesia. 
At  the  same  time,  the  fingers  look  shrunken,  the  skin  being  thrown 
into  folds,  as  if  the  hand  had  been  soaked  in  hot  water,  or  they  may 
appear  as  if  frozen,  the  skin  being  hard  and  immovable.  The  secretion 
of  perspiration  may  be  increased,  and  the  fingers  feel  damp  as  well  as 
cold,  or  it  may  be  suspended.  The  local  temperature  is  lowered.  If 
the  part  is  cut,  little  or  no  blood  will  flow.  At  this  stage  the  arterial 
spasm  may  suddenly  relax  and  the  part  return  gradually  to  its  normal 
condition,  the  cessation  of  the  constriction  of  the  arteries  and  the  return 
of  blood  being  usually  accompanied  by  burning  pain,  which  may  last 
for  some  hours.  The  duration  of  such  an  attack  may  vary  from  a  few 
moments  to  several  days.  If  it  continues  longer,  this  stage  is  usually 
succeeded  by  the  second  stage,  of  cyanosis. 

(2)  The  stage  of  cyanosis  results  from  one  of  two  conditions  :  either 
the  arterial  spasm  is  so  complete  that  no  blood  passes  into  the  part,  in 
which  case  venous  blood  from  lack  of  vis  a  tergo  or  in  response  to  gravi- 
tation regurgitates  into  the  capillaries,  distending  them  and  producing 
a  state  of  blueness ;  or  a  venous  spasm  occurs,  preventing  the  exit  of 
blood  from  the  part,  which  then  becomes  actively  congested,  and  the 
blood  in  the  capillaries,  from  want  of  renewal,  soon  becomes  venous 
and  produces  the  cyanotic  appearance.  The  stage  of  ischsemia  may  be 
so  short  that  it  is  hardly  noticed,  so  that  the  patient's  attention  is  first 
attracted  by  the  swollen,  blue,  and  extremely  painful  condition.  The 
skin  may  be  stretched,  the  tissue  infiltrated  with  products  of  exuda- 
tion, which  can  be  passed  out,  as  can  also  the  venous  blood,  and  the 
surface  may  itch  as  well  as  be  painful.  Anaesthesia  is  rarely  present 
in  this  stage,  and  there  may  even  be  hyperaesthesia.  The  part  is  cool 
from  the  increased  radiation  of  heat  and  cessation  of  the  processes  of 
metabolism,  the  local  temperature  being  lowered.  The  small  vessels 
on  the  surface  will  be  visibly  injected,  and  capillary  ecchymoses  may 
rarely  be  seen.  There  is  less  liability  to  difficulty  in  movement  in  this 
stage  than  in  the  former  one,  as  the  sensations  of  the  part  are  not 
benumbed,  but  if  present  it  is  due  to  the  swelling.  This  condition, 
like  that  in  the  former  stage,  may  cease  suddenly,  the  recovery  of  the 
normal  appearance  being  as  a  rule,  slower  than  after  a  simple  ischae- 
raia.  The  duration  of  this  stage  has  varied  from  a  few  seconds  to 
several  days.     It  is  usually  followed  by  gangrene. 

(3)  The  condition  of  local  erythema  is  described  here  because  it  may 
lead  to  gangrene,  and  has  therefore  been  considered  by  Weiss  as  one 
of  the  early  stages  of  the  disease.  As  a  rule,  however,  it  is  not  fol- 
lowed by  the  death  of  the  part,  and  the  affection  in  these  cases  is  prob- 
ably one  of  erythromelalgia  rather  than  of  symmetrical  gangrene. 
Like  the  stage  of  ischaemia,  the  stage  of  erythema  may  appear  sud- 
denly. The  part  presents  a  bright-red  or  a  mottled  appearance  —  is 
hot  and  swollen,  and  painful.     The  vessels  are  visibly  injected,  the 


934  SYMMETRICAL    GANGRENE. 

local  temperature  is  raised,  the  secretion  of  sweat  may  or  may  not  be 
increased,  and  the  patient  feels  a  burning  sensation  rather  than  pain. 
Hypersesthesia  to  touch  and  temperature  and  pain  is  usually  present, 
or  the  sensations  are  normal.  The  blood  can  be  pressed  out,  but 
returns  immediately.  In  this  condition  of  hypersemia  slight  injuries 
lead  frequently  to  an  inflammatory  process,  ulcers  may  form  in  the 
pulps  of  the  fingers  or  around  the  nails,  and  the  eschars  may  appear 
dark  and  even  gangrenous  ;  or  an  actual  condition  of  gangrene  may 
appear  in  the  tips  of  the  fingers,  the  exact  method  of  its  occurrence 
being  a  matter  of  dispute.  The  erythematous  condition  is  much  more 
likely  to  be  permanent  than  are  the  other  stages  of  the  disease  —  another 
fact  which  has  led  to  some  hesitation  in  considering  it  a  true  stage. 
This  condition  of  erythema  may  be  due  to  paralysis  of  the  vaso-con- 
strictors,  the  converse  of  the  spasm  occurring  in  ischsemia.  It  has 
also  been  ascribed  to  an  irritation  of  the  vaso-dilators ;  and  this 
appears  to  be  the  more  probable  hypothesis. 

(4)  The  stage  of  gangrene  is  always  preceded  by  that  of  cyanosis, 
and  the  death  of  the  tissue  is  due  to  the  arrest  of  nutrition  consequent 
upon  a  stasis  of  the  blood.  It  is  not  necessary  to  invoke  the  injury  of 
trophic  nerves  to  explain  its  appearance.  In  the  tips  of  the  cyanotic 
fingers,  on  their  palmar  surface,  beneath  the  epidermis,  a  small  blister 
appears,  filled  with  a  dark  serous  fluid  or  with  pus  or  blood.  This 
soon  ruptures,  and  a  dark  dry  scab  forms,  beneath  which  an  ulceration 
may  go  on  destroying  the  corium,  but  not  penetrating  deeper.  In  the 
majority  of  cases  the  gangrene  is  limited  to  a  small  area  of  the  pulps 
of  the  fingers,  and  only  involves  the  superficial  layers  of  the  corium. 
The  gangrenous  spot  is  surrounded  by  a  purple  margin.  When  the 
sphacelus  has  separated  a  scar  remains  which  is  frequently  insensitive. 
In  other  cases  when  the  sphacelus  is  thrown  off"  it  leaves  a  deep  ulcer, 
which  may  look  as  if  the  lost  tissue  had  been  cut  out  with  a  punch,  and 
this  gradually  granulates  and  heals.  In  still  other  cases  the  entire 
skin  of  the  terminal  phalanx  may  become  black  and  dry,  presenting 
a  true  gangrenous  appearance.  Then  a  line  of  demarcation  is  formed, 
usually  at  the  junction  of  the  terminal  with  the  middle  phalanx  ; 
separation  of  the  gangrenous  part  occurs,  and  a  stump  is  left  covered 
with  thin,  glossy  skin.  This  extensive  gangriene,  involving  an  entire 
phalanx,  is  the  exception  rather  than  the  rule. 

While  the  gangrenous  process  is  in  progress  in  the  tip  of  the  finger 
the  nails  cease  to  grow,  and  may  become  bulbous  and  rigid ;  the  epi- 
dermis elsewhere  may  become  dry  and  desquamate,  and  ulceration 
around  the  root  of  the  nail  may  take  place.  W^ith  the  completion  of 
the  stage  of  gangrene,  which  may  last  from  one  to  five  weeks  accord- 
ing to  its  extent,  the  local  symptoms  terminate. 

It  is  the  symmetrical  distribution  of  the  local  symptoms  just  enumer- 
ated which  is  the  peculiar  characteristic  of  the  disease.  The  fingers  of 
both  hands,  the  toes  of  both  feet,  symmetrically  situated  spots  upon  the 
back,  trunk,  thighs,  legs,  forearms,  and  arms  (in  the  order  of  frequency 
named),  are  afi'ected  either  singly  or  in  combination.     In  the  majority 


NAWRB.  935 

of  cases  finders  and  toes  are  aiFected  together,  and  a  few  spots  are  seen 
on  the  truifk.  In  many  eases  the  toes  escape.  In  a  large  number  of 
cases  the  face  has  been  affected,  spots  of  cyanosis  appearhig  on  the  nose 
or  ears  or  lips.  As  a  rule,  the  stage  of  gangrene  only  ensues  in  the  tips 
of  the  extremities,  but  a  few  cases  are  recorded  in  which  little  areas  of 
skin  elsewhere  have  passed  through  all  the  stages  of  the  disease.  Pig- 
mentation occurs  in  spots  upon  the  body  when  the  process  does  not  go 
on  to  gangrene. 

Among  the  rare  symptoms  which  have  occurred  in  some  cases  are 
great  impairment  of  temperature,  pain,  and  electric  sensations  in  the 
affected  extremities ;  swelling,  pain,  redness  about,  and  effusion  into, 
the  joints  ;  considerable  loss  of  motion  in  the  muscles  of  the  hands  and 
feet,  with  diminution  of  electric  excitability,  but  no  qualitative  change  ; 
and  oculo-pupillary  changes  ascribed  to  an  irritation  of  the  cervical 
sympathetic  fibres  at  their  origin  in  the  spinal  cord. 

In  addition  to  the  constitutional  symptoms  mentioned,  which  may 
usher  in  the  disease  and  may  continue  during  its  course,  there  have 
been  observed  temporary  albuminuria,  glycosuria,  and  hsematuria. 
Fever  never  occurs  as  a  symptom  of  the  disease,  and  if  present  must 
be  ascribed  to  some  other  condition.  The  special  senses  have  been 
affected  in  a  few  cases.  In  one  case  a  spastic  contraction  of  the  retinal 
arteries  alternated  with  attacks  of  ischsemia  in  the  extremities.^  The 
intellect  is  usually  unaffected,  but  Weiss  observed  a  case  in  which 
transient  aphasia  occurred,  which  he  attributes  to  local  spasm  in  the 
arteries  of  the  cortex.  The  patient  could  find  words  only  after  long 
thought,  and  spoke  slowly  and  with  difficulty. 

Course  and  Duration.  —  The  onset  of  the  disease  is  sudden.  The 
symptoms  in  the  first  two  stages  may  last  only  for  a  few  minutes  and 
pass  off,  or  the  disease  may  pass  through  all  the  stages  and  terminate 
in  gangrene.  It  is  usual  for  the  first  stage  of  ischsemia  to  last  several 
days,  varying  in  severity ;  for  the  second  stage  to  last  several  days ; 
and  for  the  stage  of  gangrene  to  occupy  about  three  weeks.  The  short- 
est duration  of  a  single  attack  has  been  ten  days,  the  longest  five 
months.  If  the  gangrene  begins  simultaneously  in  all  the  fingers,  the 
duration  will  be  shorter  than  if  it  proceeds  to  one  after  another.  In 
one-third  of  the  cases  a  recurrence  of  the  disease  within  a  year  of  the 
first  attack  has  been  observed,  and  it  is  probable  that  the  proportion 
would  have  been  larger  had  all  the  patients  been  kept  under  observa- 
tion. In  some  cases  three  and  four  attacks  have  succeeded  each  other 
with  some  rapidity,  some  of  the  attacks  being  much  less  severe  and 
shorter  than  others.  In  some  cases  the  condition  of  gangrene  has 
developed  only  in  one  out  of  three  attacks.  When  the  condition  is  one 
of  local  erythema  the  duration  may  be  indefinite,  the  state  becoming 
chronic  and  lasting  for  several  years. 

Nature.  —  The  nature  of  the  disease  is  a  matter  of  deduction  from 
the  study  of  the  symptoms,  no  autopsies  having  as  yet  been  made.  As 
already  stated,  the  symptoms  are  explained  on  the  theory  of  a  vaso- 
'  Raynaud,  Arch.  gdn.  de  med.,  1874,  p.  11  ;  Galezowski  examined  the  discs. 


936  SYMMETRICAL    GANGRENE. 

constrictor  irritation  in  the  stages  of  ischaemia  and  cyanosis  —  of  a  vaso- 
dilator irritation  in  the  stage  of  erythema.  Whether  this  irritation  is 
the  direct  result  of  abnormal  processes  going  on  in  the  vaso-motor 
centres  in  the  spinal  cord,  or  is  the  reflex  result  of  irritation  arising 
elsewhere,  is  undetermined.  Raynaud  held  that  it  must  be  of  central 
origin,  since  in  his  cases  galvanization  of  the  spinal  cord  modified  the 
arterial  spasm.  The  latter  observation  has  not  been  confirmed  by  other 
observers.  Weiss  believes  that  the  condition  may  occur  in  response 
to  irritation  arising  in  the  skin,  in  the  viscera,  or  in  the  brain,  and  thus 
prefers  the  theory  of  reflex  origin.  This  theory  is  adopted  by  several 
observers,  who  find  a  source  for  such  irritation  in  the  female  genital 
organs  in  their  cases.  Other  writers  affirm  that  there  is  always  a  local 
arterial  disease  —  sometimes  of  syphilitic  origin,  which  affects  the  vaso- 
motor nerves  in  the  blood-vessel  walls ;  or  may  go  on  to  obliterating 
endarteritis. 

Etiology.  —  The  disease  occurs  in  adult  life,  only  two  cases  having 
been  observed  in  persons  fifty  years  old.  It  is  most  frequent  between 
the  ages  of  fifteen  and  thirty,  although  children  and  adults  beyond  the 
age  of  thirty  are  about  equally  liable.  Females  are  more  liable  to  it 
than  males,  four-fifths  of  the  recorded  cases  having  been  in  women. 
It  occurs  more  frequently  in  the  winter  months,  exposure  to  cold  being 
a  common  exciting  cause.  Other  exciting  causes  are  nervous  exhaus- 
tion, especially  occurring  in  those  who  are  predisposed  to  nervous  dis- 
eases by  heredity  ;  general  weakness  from  anaemia,  malnutrition,  or 
the  occurrence  of  acute  fever  or  exhausting  disease ;  and  mental  agita- 
tion, a  fright  having  preceded  the  attack  in  several  cases.  In  women 
menstrual  disorders  and  uterine  disease  have  been  considered  as 
etiological  factors.  Occupation  has  something  to  do  with  its  occurrence, 
since  washerwomen,  waitresses,  and  chambermaids  are  the  class  most 
often  affected.  In  many  cases,  however,  no  cause  of  local  irritation 
can  be  found. 

Diagnosis.  —  The  diagnosis  rests  upon  the  development  of  vaso-mo- 
tor symptoms  in  the  extremities,  situated  symmetrically,  going  on  to 
gangrene,  in  a  person  not  afflicted  with  cardiac  disease  or  with  endar- 
teritis of  any  kind,  and  not  having  been  exposed  to  frost-bite  or  ergot- 
poisoning.  The  age  of  the  patient,  the  symmetrical  position  of  the 
symptoms,  the  persistence  of  the  pulse  in  the  main  arteries,  and  the 
limitation  of  the  gangrene  to  the  tips  of  the  extremities  distinguish  it 
readily  from  senile  gangrene.  The  history  of  tiie  case,  the  absence  of 
itching,  and  the  presence  of  pain  during  the  arterial  spasm  which  passes 
ofP  when  the  spasm  ceases,  serve  to  separate  it  clearly  from  chilblains. 
Congenital  cyanosis  is  produced  by  cardiac  anomalies,  and  the  entire 
body  is  affected.     Ergot-poisoning  can   be  ascertained  by  the  history. 

Prognosis. — Life  i§  not  endangered  by  this  disease,  no  fatal  cases 
having  been  recorded.  Recovery  from  an  attack  is  certain,  but  the 
duration  Qannot  be  stated,  as  it  will  depend  in  any  case  on  the  charac- 
ter, the  extent,  and  the  severity  of  the  symptoms.  The  possibility  of 
a  recurrence  of  the  attack  should  be  stated  to  the  patient. 


TREATMENT.  937 

Treatment. — The  methods  of  treatment  have  varied,  and  none  are 
wholly  satisfactory.  If  the  causes  can  be  met — e.  g.  aneemia,  nervous 
exhaustion — they  should  be  treated.  If  not,  the  disease  itself  may 
be  attacked  by  means  of  electricity.  Or  the  symptoms  may  be  treated 
as  they  demand  it.  Electricity  has  been  used  by  almost  all  observers. 
The  faradic  current  produces  an  aggravation  of  all  the  symptoms  ex- 
cept in  the  stage  of  erythema,  and  has  been  discarded.  The  galvanic 
current  may  be  employed  in  several  ways.  Two  methods  are  in  use. 
In  the  first  the  positive  pole  is  applied  over  the  cervical  region,  and 
the  negative  pole  over  the  lumbar  region,  a  descending  current  being 
thus  sent  through  the  spinal  cord.  The  current  should  be  of  moderate 
strength,  not  above  twenty-five  milliamperes,  few  patients  being  able 
to  endure  the  strength  implied  in  Raynaud's  statement  that  he  used 
sixty-four  cells  of  a  Daniel  battery.  The  duration  of  the  application 
should  be  ten  minutes,  and  the  electricity  may  be  applied  once  daily. 
In  the  second  method  the  anode  is  applied  over  the  brachial  or  lumbar 
plexus,  as  the  case  may  be,  and  the  cathode  passed  over  the  affected 
extremity,  the  current  being  constant  and  care  being  taken  not  to 
break  it  suddenly.  The  strength,  duration,  and  frequency  should  be 
the  same  as  in  the  first  method.  From  these  two  methods,  separately 
or  combined,  Raynaud  claimed  to  have  seen  favorable  results.  His 
assertions  have  not  been  confirmed  by  other  observers  who  have  fol- 
lowed his  directions  closely,  and  hence  considerable  doubt  at  present 
prevails  as  to  the  efficacy  of  the  electric  current.  The  so-called  elec- 
trical application  to  the  cervical  sympathetic  is  certainly  useless.  In 
the  stage  of  erythema  a  very  weak  faradic  current  applied  to  the  hands 
in  a  bath  may  be  of  service. 

Many  observers  have  found  that  the  progress  of  the  case  to  recovery 
was  quite  rapid  if  the  limb  were  put  at  rest  in  an  elevated  position, 
were  kept  warm  by  cotton  batting  or  similar  bandaging,  and  were 
kept  clean  with  antiseptic  lotions  when  the  stage  of  gangrene  set  in. 
Massage  is  to  be  used  in  all  cases,  the  limbs  or  affected  parts  being 
gently  rubbed  with  the  dry  hand  or  with  aromatic  liniments  or  oils. 
All  local  injury,  however,  and  especially  counter-irritation,  are  to  be 
carefully  avoided.  General  tonic  treatment,  especially  iron  and  cod- 
liver  oil,  is  to  be  used  in  all  cases. 

The  pain  occurring  in  the  early  stages  is  often  so  severe  as  to  re- 
quire the  use  of  opium  or  other  narcotics.  And  when  the  nervous 
symptoms  are  especially  aggravated,  and  irritability  and  insomnia  give 
the  patient  discomfort,  bromide  and  chloral  may  be  employed. 


CHAPTER   LVIII. 

ANGIO-NEUROTIC   (EDEMA. 

A  CONDITION  of  cederaatous  swelling  of  limited  portions  of  the  sur- 
face of  the  body,  transient  in  duration  and  attended  by  marked  vaso- 
motor changes,  has  been  recognized  for  some  years.  It  has  been 
termed  acute  oedema,  or  angio-neurotic  oedema.  The  latter  term  im- 
plies a  knowledge  of  its  pathology,  which  is  not  as  yet  established. 

The  essential  features  of  the  disease  are  as  follows  :  A  swelling  of 
the  tissues  affected  of  an  oedematous  kind,  with  a  change  in  their  color, 
temperature,  and  consistence,  usually  without,  but  occasionally  with,  a 
disturbance  in  the  sensations  of  the  part  and  an  interference  with  the 
functions  of  the  parts,  due  to  the  stiffness  and  swelling.  The  swelling 
is  usually  transient,  comes  and  goes  rapidly  often  after  exposure  to 
cold,  but  may  remain  permanently  for  some  days.  It  appears  to  be 
of  the  nature  of  oedema  of  the  corium  itself,  rather  than  of  the  cutaneous 
connective  tissue,  as  it  is  hard,  does  not  pit  on  pressure,  and  cannot  be 
entirely  removed  by  pressure  or  manipulation.  , 

The  color  of  the  part  affected  is  usually  red  or  reddish  blue,  but  is 
sometimes  whitish-yellow  or  pale. 

The  temperature  is  usually  lower  than  in  the  unaffected  parts. 

The  sensations  felt  by  the  patient  are  those  of  stiffness,  burning, 
numbness,  or  pain,  and  the  discomfort  is  increased  by  the  application 
of  cold.     Objectively,  anaesthesia  is  rare. 

Any  part  of  the  body  may  be  affected  by  the  oedema,  but  hands  and 
feet  and  face  are  the  parts  most  often  affected. 

The  duration  of  the  attack  varies  from  a  few  hours  to  a  few  days. 
In  the  majority  of  cases  there  is  a  complete  cessation  of  symptoms 
between  the  attacks.  In  a  few  there  is  only  a  remission.  The  con- 
dition differs  from  chronic  hereditary  trophoedema  and  from  perma- 
nent oedema  of  trophic  origin  (see  page  945)  in  being  temporary  in 
duration  and  occurring  in  attacks,  with  free  intervals.  The  appear- 
ance presented  during  the  attack  resembles  that  shown  in  Figs.  320 
and  321,  page  945. 

The  disease  has  been  well  described  by  Simon  in  the  Johns  Hopkins 
Hospital  Reports,  by  Osier,  Quincke,  Strubing,  and  others.  As  to  its 
nature  or  pathology  nothing  is  known.  Osier  has  shown  that  it  may 
be  hereditary. 

Various  remedies  have  been  tried  in  its  treatment,  but  none  have 
seemed  of  much  avail.  In  one  of  my  cases  massage  appeared  to  be 
of  benefit.     In  none  of  them  did  nitroglycerin,  or  drugs  of  an  allied 

938 


ANGIO-NEUMOTIC   (EDEMA.  939 

action,  or  ejgot  have  any  effect.  Strychnine  is  however  of  decided 
use. 

The  following  cases  are  recorded  as  a  contribution  to  the  clinical 
features  of  the  disease  : 

Case  I.  Local  Transient  (Edema,  limited  to  the  Might  Face.  — 
A  woman,  aged  twenty-eight  years,  was  married,  of  good  family  history, 
and  had  always  been  in  good  health.  She  could  assign  no  cause  for  her 
peculiar  disease,  from  which  she  had  suffered  at  intervals  for  two 
years.  It  had  not  come  on  after  any  emotional  excitement  or  physical 
strain.  At  first  it  had  given  her  very  little  discomfort,  but  during  the 
past  year  she  has  suffered  a  good  deal.  When  she  is  in  any  way  ex- 
posed to  cold  upon  her  face  she  notices  that  the  right  half  of  the  face 
begins  to  feel  stiff  and  to  burn.  When  she  looks  at  herself,  she  finds 
the  entire  right  side  of  the  face  swollen  and  whitish  in  color,  and  to 
the  touch  the  swollen  part  is  perceptibly  hard.  This  condition  may 
pass  off  in  a  few  hours,  but  often  lasts  for  three  or  four  days,  gradu- 
ally subsiding.  She  has  noticed  that  warm  applications  will  relieve 
it  in  some  degree.  It  often  comes  on  after  she  has  been  driving  in 
the  summer,  but  is  more  troublesome  in  the  winter.  It  sometimes 
comes  spontaneously  without  exposure  or  known  cause.  It  has  no 
relation  to  or  connection  with  her  general  health,  indigestion,  or  men- 
struation. Fear  of  it  or  expectation  will  not  produce  it,  though  she 
is  in  constant  dread  of  it,  as  it  often  interferes  with  her  going  into 
society  or  keeping  social  engagements. 

The  right  half  of  the  face  was  swollen  at  the  time  of  her  visit  to 
the  office.  The  surface  of  the  forehead  was  elevated.  The  tissues 
about  the  eye,  the  eyelids,  and  soft  parts  beneath  the  eye  were  swollen 
and  stiff,  and  so  tense  that  the  eye  could  not  be  opened  as  widely  as 
the  other.  The  cheek  was  perceptibly  rounded  and  fuller  than  the 
other,  and  the  chin  shared  in  the  swelling.  The  neck  was  not  at  all 
affected,  its  tissues  being  loose.  The  swollen  surface  was  colder  to 
the  touch  than  the  opposite  side,  and  had  a  whitish-yellow  tint  with- 
out the  natural  color,  which  was  good  on  the  left  side.  An  applica- 
tion of  heat  for  a  few  minutes  had  no  perceptible  effect.  The  condi- 
tion seen  had  been  present  for  two  days.  The  face  had  an  appearance 
of  oedema  but  did  not  pit  on  pressure  and  was  much  harder  than 
oedematous  tissue.  It  resembled  almost  exactly  the  condition  seen  in 
myxoedema,  but  the  swelling  did  not  produce  that  thick  fold  running 
up  on  the  nose  from  the  cheek  seen  so  frequently  in  myxoedematous 
patients. 

Under  massage  the  condition  subsided  within  a  few  hours,  and  this 
treatment  was  kept  up  daily  for  two  months.  Since  that  time  there 
has  been  but  one  return  of  the  swelling,  so  that  in  this  case  massage 
has  certainly  afforded  great  relief  for  at  least  six  months. 

Case  II.  Local  Transient  CEdema.  —  A  woman,  aged  thirty -seven 
years,  had  been  subject  to  emotional  strain  for  a  few  months  before 
her  present  illness,  iiaving  lost  three  children  at  once  and  having 
grieved  very  mucii.     She  first  noticed  that  her  hands  swelled  when- 


940  ANGIO-NEUBOTIC   (EDEMA. 

ever  she  put  them  into  cold  water.  When  winter  came  on  this  swell- 
ing on  exposure  to  cold  was  noticed  in  all  other  parts  of  her  body  — 
e.  g.,  the  face  and  legs  when  out  of  doors,  and  the  buttocks  when  she 
went  to  stool  in  a  cold  closet.  It  has  been  most  severe  in  her  hands. 
During  the  swelling  the  skin  feels  as  if  it  would  burst,  the  hands  are 
hot,  and  she  cannot  use  them.  No  other  symptoms  are  complained  of. 
Examination.  —  She  is  a  well-nourished  woman  with  no  physical 
signs  of  heart,  lung,  kidney,  or  uterine  disease.  Her  face  is  swollen 
on  the  lower  part  of  both  cheeks,  feels  hard  to  the  touch,  but  does  not 
pit  on  pressure,  and  has  a  yellowish,  waxy  appearance.  This  she  says 
is  due  to  the  cold  wind  and  is  temporary.  It  subsided  somewhat 
during  her  stay  in  my  warm  office.  The  left  hand  was  subjected  to 
experiment.  It  was  first  tested  and  found  to  be  the  same  as  the  right 
in  point  of  all  sensations.  It  measured  195  mm.  around  the  knuckles, 
and  54  mm.  around  the  middle  joint  of  the  middle  finger.  The  dyna- 
mometer grasp  was  75°,  right  85°.  It  was  then  held  in  cold  water 
for  a  minute.  After  half  a  minute  in  water  it  looked  reddish  pink; 
when  taken  out  of  the  water  it  was  of  a  blotchy  blue ;  in  a  minute  it 
had  become  uniformly  bluish  red;  in  a  minute  and  a  half  paler;  in 
three  minutes,  yellow ;  and  at  the  end  of  five  minutes  and  a  half  it 
was  white  and  waxy  in  appearance  and  the  skin  had  become  quite 
glossy.  The  veins  on  the  back  of  the  hand  were  distended  and  she 
complained  of  throbbing  in  all  the  fingers,  and  this  was  perceptible  to 
the  touch.  The  entire  hand  had  become  swollen  so  that  at  the  end  of 
ten  minutes  it  measured  204  mm.  around  the  knuckles  and  62  mm. 
around  the  middle  finger.  At  the  same  time  the  fingers  became  so 
stiff  that  they  were  moved  with  difficulty,  and  the  dynamometer  grasp 
was  reduced  to  35°,  the  effort  of  squeezing  producing  pain,  distension 
of  the  veins,  and  a  feeling  as  if  the  skin  would  burst.  The  tempera- 
ture rose  rapidly  during  the  swelling  of  the  hand.  Two  surface  ther- 
mometers were  laid  on  the  hands,  each  marking  70°  F.  After  one 
minute  the  thermometer  on  the  right  hand  was  78°,  on  the  left  hand 
87.5°;  after  three  minutes,  81.75°  and  91.75°;  after  nine  minutes, 
85.5°  and  93°,  at  which  point  they  remained  for  three  minutes  and 
were  then  removed.  These  thermometers  had  previously  been  tested 
and  were  duplicates  in  sensitiveness.  The  sensibility  to  touch  and 
pain  was  not  altered  during  the  swelling.  A  rubber  band  applied  to 
the  little  finger  forced  the  blood  out  and  reduced  the  cedema,  but,  on 
taking  it  off,  dark-blue  lines  were  left  and  the  swelling  returned  at 
once.  The  swelling  appeared  to  be  of  an  cedematous  nature,  but  the 
parts  were  very  hard  and  did  not  pit  on  pressure.  The  waxy  appear- 
ance and  glossy  skin  began  to  fade  in  fifteen  minutes  after  the  hand 
had  been  put  into  water  and  the  hand  became  whiter  and  the  pain  and 
throbbing  less.  At  the  end  of  twenty-five  minutes  motion  was  freer, 
and  the  measurements  were  195  mm.  and  61  mm.  During  the  entire 
time  the  pulse  was  88  at  the  wrist  and  its  tension  did  not  vary  per- 
ceptibly to  the  touch.  The  fingers  were  still  swollen  when  she  left 
at  the  end  of  forty  minutes.     She  states  that  the  nails  grow  more 


ANGIO-NEUEOTIC    (EDEMA.  941 

rapidly  tha^  formerly.  There  is  no  change  in  the  sweat.  Various 
kinds  of  treatment  had  been  ineffectual,  and  the  patient  still  suffers 
from  these  symptoms  in  cold  weather. 

Case  III.  Local  Transient  CEdema^  limited  to  the  Left  Hand.  — 
Female,  aged  fifty-four  years,  is  a  healthy  woman  and  has  a  good 
family  history.  Has  never  suffered  from  any  severe  diseases.  The 
present  condition  began  at  Christmas  without  known  cause,  certainly 
not  after  special  exposure  to  cold.  It  is  limited  entirely  to  the  fingers 
and  thumb  of  the  left  hand,  the  hand  itself  escaping  The  fingers  are 
swollen,  dark  blue  in  color,  hard  to  the  touch,  stiff  in  movement,  and 
after  exposure  to  cold  become  very  painful.  The  size  of  the  phalanges 
of  the  index  compared  with  that  of  the  right  hand,  was  as  follows  . 
First  phalanx,  ||  mm.;  second  phalanx,  ||  mm.;  third  phalanx, 
1^  mm.  There  was  no  anaesthesia  to  touch,  temperature,  or  pain,  but 
cold  was  felt  more  acutely  in  the  affected  fingers,  and  after  the  hand 
had  been  exposed  to  cold,  cotton  was  not  always  felt.  The  oedema 
could  be  slightly  reduced  by  manipulation,  but  not  permanently,  and 
the  color  returned  at  once  after  pressure.  The  stiffness  and  pain  were 
much  increased  by  immersion  in  cold  water,  and  then  relieved  by  heat. 
She  cannot  carry  the  hand  hanging  down  without  much  pain.  Power 
was  impaired  by  the  stiffness.  There  was  no  atrophy  of  the  hand 
muscles.  The  electric  resistance  was  carefully  measured,  but  was 
equal  on  both  sides.  There  was  no  atheroma  of  the  radial  or  other 
arteries,  and  tension  was  not  high.  Treatment  had  been  of  no  avail. 
Massage  was  recommended.  The  condition  here  was  stationary,  with 
remissions,  in  which  respect  it  differed  from  the  other  cases,  in  which 
it  had  been  intermittent. 

Case  IV.  (Edema  of  the  Face.  —  Female  aged  forty  after  a  nervous 
shock  noticed  a  swelling  about  both  eyes  which  made  it  difficult  to 
open  them.  This  gradually  extended  half  way  up  the  forehead  and 
down  over  both  cheeks  and  on  the  sides  of  the  nose  and  about  the 
mouth  and  chin,  so  that  all  facial  movement  was  hampered  and  there 
was  a  lack  of  play  in  facial  expression,  the  face  being  puffy  and  mask- 
like. It  did  not  pit  on  pressure.  It  was  dusky  and  slightly  cyanotic 
and  cool  to  the  touch.  The  swelling  became  greater  when  the  face 
was  exposed  to  cold  and  then  tingling  and  pain  were  felt.  The  tongue 
was  not  affected.  There  was  no  anaesthesia.  There  was  no  swelling 
elsewhere  in  the  body  and  no  nephritis.  The  only  other  symptom  was 
insomnia.  The  condition  failed  to  yield  to  anti-gouty  remedies  which 
had  been  tried.  It  had  persisted  for  six  weeks  continously  when  I 
saw  her.  She  was  put  on  small  doses  of  strychnine  frequently  repeated 
and  was  sent  away  from  home  for  a  mental  change.  The  condition 
subsided  within  a  week  and  has  not  recurred. 


CHAPTER   LIX. 

TROPHIC  NEUROSES. 

Trophic  Nerves  and  Nervous  Centres,  —  The  nutrition  of  the 
body  depends  upon  the  nutrition  of  the  individual  cells  of  which  it  is 
made  up.  Each  cell  has  the  power  of  appropriating  from  the  blood 
such  substances  as  will  preserve  its  existence,  enable  it  to  perform  its 
functions,  and  produce  a  successor.  Whether  this  power  is  inherent 
in  the  cell  or  is  controlled  by  the  nervous  system  is  a  question  upon 
which  authorities  are  divided.  Those  who  hold  the  first  position  deny 
the  existence  of  trophic  nervous  centres  and  of  trophic  nerves  from 
those  centres  of  the  organs  and  elements  of  the  body,  claiming  that 
this  hypothetical  trophic  system  has  not  been  demonstrated  anatomic- 
ally, and  that  the  facts  urged  in  its  support  are  capable  of  another 
interpretation.  Those  who  believe  in  the  existence  of  a  trophic  system 
have  been  able  to  demonstrate  the  existence  of  fine  peripheral  nerve- 
fibres  passing  to  and  ending  in  individual  cells  of  the  skin,  glands,  and 
other  organs,  and  have  brought  forward  a  large  collection  of  facts 
which  merit  a  careful  examination.     They  are  as  follows  : 

Atrophy.  —  When  a  nerve  is  cut  certain  changes  occur  in  it  which 
are  known  as  Wallerian  degeneration.  These  have  been  already  de- 
scribed in  connection  with  injuries  of  the  nerves  (page  159). 

Degeneration  in  the  tracts  of  the  spinal  cord  occurs  after  various 
forms  of  lesion,  and  is  similar  in  its  processes  to  degeneration  in  the 
peripheral  nerves.  The  increase  in  the  connective-tissue  elements  is 
more  noticeable  in  contrast  with  the  parts  unaffected,  and  from  the 
density  of  the  tract  involved  the  result  has  been  called  sclerosis.  The 
researches  of  Homen  have  shown  that  the  process  of  degeneration 
begins  in  the  entire  length  of  the  affected  tract,  and  does  not  proceed 
from  the  point  of  lesion  onward,  as  was  formerly  supposed.  This  is 
described  on  page  403  in  lesions  of  the  spinal  cord. 

When  a  muscle  is  separated  from  its  connection  with  the  central 
nervous  system,  either  by  a  division  of  the  nerve  passing  to  it  or  by  a 
destruction  of  the  cells  in  the  anterior  cornu  of  the  spinal  cord  from 
which  that  nerve  arises,  it  undergoes  an  atrophy  which  is  peculiar  in 
being  immediate  and  rapidly  progressive,  thus  contrasting  strongly 
with  the  gradual  and  slighter  atrophy  from  disuse  in  cases  of  cerebral 
paralysis  where  the  cells  mentioned  and  the  nerve-fibres  are  intact. 
There  is  at  first  a  simple  diminution  in  the  number  of  the  fibrillse  of 
which  the  muscular  fibre  is  made  up,  together  with  an  increase  in  the 
interstitial  connective  tissue  nuclei.  Then  an  albuminoid  and  fatty 
degeneration  of  the  muscular  elements  occurs,  with  a  proliferation  of 

942 


ATROPHY.  •  943 

muscle-corpuscles  or  nuclei,  and  a  gradual  absorption  of  the  debris. 
The  intersfltial  connective  tissue  then  increases  rapidly,  forming  fibrous 
bands  through  the  degenerated  muscle  which  compress  the  few  mus- 
cular fibres  remaining,  until  as  a  result  the  muscle  is  transformed  into 
a  mere  ribbon  of  connective  tissue  without  any  power  of  contractility. 
As  these  changes  go  on  the  electrical  reactions  change,  the  three  de- 
grees of  reaction  of  degeneration  corresponding  to  the  three  stages  of 
atrophy  described.  These  phenomena  of  nerve-  and  muscle-degenera- 
tion are  observed  in  traumatic  or  idiopathic  neuritis,  in  acute  and 
chronic  poliomyelitis  anterior,  in  general  myelitis  involving  the  an- 
terior cornua,  and  in  bulbar  paralysis. 

The  influence  of  the  nervous  system  on  the  nutrition  of  the  bones 
has  also  been  ascertained.  When  a  bone  is  developing,  a  lesion  of 
the  nerve  to  it,  or  of  the  deeper  portion  of  the  anterior  cornua  of  the 
spinal  cord  from  which  these  nerves  arise,  will  modify  and  partly 
arrest  its  growth.  This  is  often  seen  in  anterior  poliomyelitis  and  in 
hemiatrophy  of  the  face  occurring  in  children.  In  the  adult  a  no  less 
marked  effect  is  produced,  although  the  results  are  less  noticeable.  A 
condition  known  as  osteoporosis  is  caused,  consisting  of  an  enlargement 
of  the  Haversian  canals  and  an  infiltration  of  fatty  matter  into  them 
and  an  actual  decrease  in  all  the  inorganic  constituents  of  the  bone, 
which  loses  in  weight,  becomes  thinner  and  more  fragile,  so  that 
spontaneous  fractures  may  occur.  This  condition  has  been  noticed 
more  frequently  in  diseases  of  the  spinal  cord  than  in  neuritis  ;.  it  is 
said  to  occur  in  locomotor  ataxia.  It  has  been  found  in  a  few  cases 
of  long-standing  hemiplegia  and  also  in  dementia  paralytica,  no  expla- 
nation of  its  pathogeny  in  these  instances,  however,  being  offered.  In 
a  case  of  ataxia  with  a  lesion  in  the  medulla  which  involved  the  nuclei 
of  the  fifth,  ninth,  tenth,  and  eleventh  nerves  on  one  side,  all  the 
teeth  of  the  upper  jaw  on  that  side  fell  out  within  a  few  weeks,  those 
in  the  lower  jaw  remaining.  Changes  in  the  nutrition  of  the  bones 
have  also  been  recorded  in  cases  of  progressive  muscular  atrophy  in  the 
paralyzed  limbs. 

The  condition  of  the  skin  and  its  appendages  is  influenced  decidedly 
by  changes  in  the  nervous  system,  either  in  the  nerves,  in  the  spinal 
ganglia,  or  in  the  central  gray  matter.  Here  it  is  the  sensory  nerves 
which  convey  the  trophic  influence,  not  the  motor  nerves,  as  in  the 
cases  hitherto  considered ;  and  when  the  lesion  producing  trophic 
changes  in  the  skin  is  central,  it  is  situated  in  the  posterior  cornua  of 
the  spinal  cord  or  in  the  gray  matter  near  the  central  canal. 

It  has  been  recently  shown  that  the  distribution  of  nsevus  —  the 
dilatation  of  the  capillaries  in  the  skin  —  is  determined  by  the  nervous 
supply  of  the  skin,  just  as  the  distribution  of  herpes  is  determined. 
The  [)late  shows  a  case  of  nsevus  of  the  trunk  and  arm  in  which  this 
limitation  to  the  distribution  of  certain  cervical  and  dorsal  nerve  roots 
is  manifest.  This  figure  might  easily  be  duplicated  from  figures  of 
herpes  zoster. 

The  glossy  skin  seen  on  the  fingers  after  injuries  to  the  nerves  is  a 


944  TEOFHIC   NEUBOSES. 

type  of  such  atrophy  from  disturbance  of  trophic  impulses.  Glossy 
fingers  present  a  smooth,  shining  appearance,  are  dry  from  the  diminu- 
tion in  the  secretion  of  sweat,  feel  soft  and  satin-like  to  the  touch  from 
the  marked  thinning  of  the  skin,  and  frequently  show  a  defective  or 
irregular  growth  of  the  nails,  which  may  be  ridged,  curved,  or  deformed. 
(See  Fig.  104,  page  203.)  They  are  red  and  mottled  from  accompanying 
vaso-motor  paralysis,  and  are  usually  hot  and  painful.  Changes  in  the 
pigmentation  of  the  skin  and  hair  are  recorded  as  a  not  infrequent 
accompaniment  of  severe  neuralgia  and  as  a  result  of  great  mental  anx- 
iety. Thus  in  several  cases  of  supraorbital  neuralgia  the  eyebrow  on 
the  affected  side  has  turned  white ;  in  infraorbital  neuralgia  the  beard 
has  become  gray  ;  and  in  both  the  hair  has  been  observed  to  fall  out. 
The  sudden  turning  white  of  the  hair  is  ascribed  to  a  swelling  of  the 
hair  by  air  within  it.  In  one  case,  frequently  cited,  the  hair  and  nails 
fell  out  after  a  stroke  of  lightning. 

When  a  gland  is  cut  off  from  its  nervous  connection  with  the  cord 
or  cerebral  axis  by  section  of  its  nerves,  its  function  is  impaired  and 
its  nutrition  suffers,  so  that  after  a  time  it  loses  weight  and  undergoes 
a  progressive  total  atrophy.  This  has  been  proven  experimentally  in 
animals  in  the  submaxillary  gland.  It  has  been  observed  in  the  testi- 
cle in  man  after  division  of  the  spermatic  nerve  (N^laton)  and  after 
destruction  of  the  spinal  cord  by  traumatic  and  idiopathic  myelitis 
(Klebs,  Foster).  The  sweat-glands  are  known  to  be  under  the  control 
of  a  central  nervous  mechanism,  as  cases  of  hyperidrosis,  anidrosis, 
and  chromiodrosis  prove  ;  and  an  atrophy  of  them  and  of  the  sebaceous 
glands  has  been  observed  after  nervous  lesions. 

Progressive  hemiatrophy  of  the  face  is  described  elsewhere  (page 
654).  The  following  case  of  progressive  hemiatrophy  of  the  entire 
body  may  be  mentioned  here:  A  boy,  aged  fourteen,  dislocated  his 
ankle,  which  in  a  few  days  became  swollen,  red,  hot,  and  painful. 
The  inflammation  extended  up  the  leg,  but  did  not  involve  the  knee, 
and  soon  subsided.  After  a  short  time  the  foot  began  to  atrophy. 
The  atrophy  extended  up  the  leg,  and  involved  the  thigh ;  it  then 
progressed  to  the  trunk  and  the  arm,  and  lastly  to  the  face  on  the 
affected  side,  until  in  the  course  of  two  years  there  had  developed  a 
unilateral  atrophy  of  the  entire  body.  Muscles,  fat,  and  bones  were 
all  affected,  but  no  difference  in  the  skin  or  hair  of  the  two  sides  was 
noticed.  Fibrillary  tremors  were  present  in  the  muscles.  The  elec- 
tric reactions  were  not  altered,  but  were  gradually  lost.  There  was  a 
hypersensitiveness  to  touch  and  to  cold,  but  no  other  sensory  disturb- 
ance. The  boy  was  alive  and  fairly  well  when  the  case  was  reported. 
It  is  unique. 

I  have  recently  seen  a  little  girl  who  has  a  distinct  atrophy  of  the 
skin,  subcutaneous  tissue,  and  muscles  of  the  anterior  inner  surface  of 
the  left  thigh  exactly  limited  to  the  distribution  of  the  anterior  crural 
nerve  ;  without  anaesthesia,  and  without  paralysis. 

These  various  instances  of  atrophy  cannot  be  ascribed  to  simple  dis- 
use, since  they  differ  markedly  in  their  pathological  changes  and  in 


EYPEBTROPEY. 


945 


the  rapidity  of  their  progress  from  such  atrophy.  Nor  are  they  to  be 
referred  t#  vaso-motor  disturbances,  since  in  many  cases  no  vascular 
changes  are  evident.  Their  distribution  in  the  body  often  corresponds 
exactly  with  that  of  peripheral  nerves,  and  they  accompany  nerve 
lesions  too  frequently  to  be  explained  on  any  theory  of  coincidence. 

Hypertrophy.  —  Trophic  changes  are  not  limited  to  the  process  of 
atrophy.  There  are  conditions  of  hypertrophy  of  supposed  nervous 
origin.  Samuel  considers  the  hypertrophy  of  one  testicle  which  attends 
atrophy  of  the  other  from  section  of.  its  nerve  as  an  example  of  this. 
It  has  been  ascertained  that  one  kidney  hypertrophies  when  the  other 
is  atrophied  or  extirpated.  When  the  spleen  is  removed  the  lymphatic 
glands  increase  in  size.  But  these  facts  are  capable  of  another  explana- 
tion —  viz.,  that  increased  demand  upon  the  organ  leads  to  its  increased 
growth.  Hypertrophy  of  the  skin  and  of  the  tongue  is  seen  in  cretins 
in  contrast  with  the  deformity  of  the  body  and  atrophy  of  the  limbs. 
A  hemihypertrophy  of  the  face  has  been  noticed  in  several  cases,  the 
counterpart  of  hemiatrophy  ;  and  in  one  case  a  unilateral  hypertrophy 
of  the  entire  body  was  observed. 

Fig.  320. 


Trophoedema  of  the  hands.     Icon,  de  la  Salpetrigre,  July,  1910. 


Fig.  321  shows  a  condition  of  hypertrophy  of  one  leg  due  to  con- 
genital oedema  and  Fig.  320  shows  the  condition  of  the  hands  in  a 
case  of  hypertrophy  of  similar  origin.  In  both  cases  the  condition 
was  permanent  and  not  variable,  and  in  the  former  it  was  present 
through  tliree  generations  in  different  members  of  the  family,  and 
hence  was  considered  hereditary.  The  local  thickening  of  the  skin 
known  as  ichthyosis  hystrix,  and  other  hypertrophies  of  the  skin,  cer- 
60 


946 


TEOPEIC   NEUROSES. 


tain  deposits  of  pigment,  and  vitiligo,  have  been  ascribed  to  nervous 
causes.  Mitchell  has  recorded  cases  of  abnormal  growth  of  the  nails 
and  hair  after  injuries  to  the  nerves,  and  similar  phenomena  have  fol- 
lowed central  lesions  and  are  common  during  attacks  of  insanity,  dis- 
appearing when  the  patient  recovers.  He  has  also  described  a  thicken- 
ing of  the  skin  of  the  first  three  fingers  and  of  the  back  of  the  hand 
following  a  wound  of  the  brachial  plexus.     These  conditions  of  the 

skin  and  its  appendages  indicate  an 
abnormal  activity  in  the  cells  of  the 
affected  part,  a  rapid  metabolism  and 
reproduction,  resulting  in  an  undue 
production  of  tissue,  apparently  de- 
pendent on  nervous  impulses  reaching 
the  cells  from  a  distance.  The  insane 
ear  may  be  mentioned  in  this  connec- 
tion as  a  trophic  disturbance  due  to 
central  lesion.  A  peculiar  form  of 
hypertrophy  limited  to  the  head  and 
neck,  and  which  I  named  megalo- 
cephalie,  was  described  by  me  some 
years  ago. 

The  patient  was  a  woman,  aged  fifty- 
two  years,  American  by  birth,  and  of 
healthy  parentage,  her  mother  being 
still  alive  at  the  age  of  seventy-eight. 
She  has  one  brother  and  two  sisters  liv- 
ing and  healthy ;  the  family  history  is 
free  from  all  evidence  of  cachexia  or 
neurotic  habit ;  she  bad  been  married 
since  the  age  of  twenty-one,  but  has 
had  no  children  ;  she  has  been  in  ex- 
cellent health  until  the  beginning  of 
this  disease  —  six  years  ago.  The  first 
symptoms  noticed  were  formication  felt 
in  the  tips  of  the  fingers,  particularly  of 
the  right  hand,  which  gradually  exten- 
ded upward  to  the  shoulder;  this  numb- 
ness was  associated  with  a  slight  clum- 
siness of  movement  and  uncertainty 
in  tactile  perception,  although  there 
has  never  been  and  is  not  now  any 
actual  anaesthesia,  either  of  touch,  temperature,  or  of  pain,  and  the 
muscular  sense  is  normal.  She  has  never  felt  any  of  this  numbness 
in  the  legs  or  body,  but  she  has  noticed  for  the  past  five  years  a  dis- 
turbance in  the  gait,  consisting  of  an  uncertainty  in  movement  and  a 
subjective  feeling  of  weakness.  During  all  this  time  there  has  been 
a  slowly  progressive  enlargement  of  the  head  and  neck,  and  at  the 
same  time  a  gradual  diminution  in  stature.     Five  jears  ago  she  was 


Chronic  hereditary  trophoedema  of  one 
leg.    (H.  Meige,  La  pratique  neurologique). 


EYPEBTBOPHT. 


947 


five  feet,  sevgn  inches  in  height,  now  she  is  five  feet  two  ;  this  short- 
ening is  partly  due  to  a  stooping  posture  resembling  that  assumed  in 
paralysis  agitans,  partly  due  to  an  apparent  sinking  of  the  head  and 
neck  between  the  shoulders,  which  causes  a  very  peculiar  appearance. 


Fig.  322. 


Ji-iS* 


Condition,  In  1889,  before  eulargemeut  of  head  and  neck  began. 
Fig.  323. 


Condition,  in  1894,  showing  uniform  enlargement  of  tlie  liead. 

The  striking  feature  of  this  disease  and  its  chief  characteristic  is  a 
slowly  progressing  increase  in  the  size  of  the  head,  face,  and  neck, 
both  t)je  hard  and  soft  tissues  being  apparently  aflfected.     The  gkin  is 


948  TBOPHIC   NEUBOSES. 

cliifusely  thickened  to  a  slight  extent,  is  thrown  into  folds  and  not 
merely  stretched  over  the  enlarged  bones,  as  it  would  be  were  it  not 
involved  in  the  process  ;  and  while  it  is  not  rough  or  scaly,  is  slightly 
pigmented  and  has  something  the  appearance  of  the  skin  in  myxoe- 
deraa.  The  subcutaneous  tissue  is  also  diifusely  thickened,  so  that  it 
throws  the  skin  out  and  forms  a  loose  body  beneath  it.  The  bone 
appears  to  be  the  portion  primarily  and  chiefly  affected,  just  as  in 
acromegaly.  There  is  apparently  a  general  hypertrophy  of  the  bone 
over  the  entire  calvariura,  more  marked  just  behind  the  temples. 
This  is  not  limited  to  definite  small  areas,  but  is  quite  uniform  every- 
where, so  that  the  skull  is  enlarged  uniformly.  The  head  measures 
twenty-four  inches  in  circumference  about  the  forehead,  fifteen  inches 
from  ear  to  ear  over  the  vertex,  and  sixteen  inches  from  the  root  of 
the  nose  to  the  occipital  protuberance.  Even  the  vertebrae  of  the 
cervical  region  appear  to  take  part  in  the  enlargement,  and  as  a  result 
the  head  is  less  movable  upon  the  spine  than  it  should  be,  and  the 
spines  of  the  vertebrae  from  the  fourth  to  the  seventh  are  decidedly 
prominent  in  the  neck.  A  feeling  of  weight  in  the  head  is  quite 
intense  and  had  led  to  the  hair  being  cut  short,  and  it  was  noticed 
that  the  hair  had  become  quite  thin,  especially  at  the  sides,  when 
formerly  it  had  been  thick  and  heavy.  This  diflFuse  growth  of  the 
head  and  neck  is  quite  well  shown  in  the  photographs,  which  are  all 
the  more  instructive,  as  the  contrast  between  the  woman  in  her  ordi- 
nary state  of  previous  health  five  years  ago  and  in  her  present  condi- 
tion is  shown.  The  thickening  of  the  facial  tissues  tends  to  decrease 
the  natural  mobility  of  the  face,  so  that  the  facial  expression  is  more 
fixed  than  in  a  state  of  health.  The  thickening  seems  to  have 
encroached  upon  the  cavities  of  the  nose  and  mouth,  there  being  some 
obstruction  to  breathing  and  swallowing  without  thickening  of  the 
tongue.  There  is  a  hard  respiratory  sound  in  the  trachea  constantly 
in  breathing,  and  this  has  been  present  for  a  year  past. 

The  general  symptoms  which  this  patient  suffers  from  are  gradually 
increasing  mental  irritability  and  general  nervousness,  without  any 
mental  or  emotional  defect ;  an  increasing  disability  to  walk  and  to  use 
her  hands,  attended  by  much  numbness,  especially  in  the  hands,  an 
occasional  pain  in  the  neck,  arms,  and  hands ;  some  awkwardness  of 
movement  in  the  fingers,  but  no  marked  ataxia,  for  she  can  knit,  can 
thread  a  needle  and  do  fancy  work.  There  is  no  anaesthesia  present, 
and  there  is  no  loss  of  pain  or  thermal  sense.  The  act  of  walking  is 
performed  with  difficulty  and  is  now  only  possible  with  the  aid  of  hold- 
ing a  chair,  though  there  is  no  ataxia  of  movement  and  no  spastic 
rigidity  of  the  legs ;  the  knee-jerks  are  slightly  increased,  but  there  is 
no  clonus  ;  the  control  of  the  sphincters  is  perfect.  In  walking  she  has 
a  general  sense  of  insecurity  and  feebleness,  but  there  is  no  evidence  of 
paralysis.  She  stoops  forward  much  like  a  patient  suffering  from  paral- 
ysis agitans,  but  has  no  tendency  to  fall  or  to  festinatiou.  She  is  able 
to  walk  up  and  down  stairs  with  help.  Her  neck  is  sixteen  inches  in 
circumference  —  at  least  four  inches  larger  than  formerly.     There  are 


EYPEBTBOPEY.  949 

two  soft  nonrfluctuating  swellings  below  the  ears  having  the  feel  of  dif- 
fuse, fatty  infiltrations,  and  the  neck  is  uniformly  increased  in  size. 
There  is  no  apparent  swelling  of  the  thyroid  gland.  The  cervical  ver- 
tebrae feel  thick  and  are  unduly  prominent,  but  are  not  tender.  She  has 
had  considerable  pain  along  the  course  of  the  occipital  nerves  ;  in  other 
respects  her  general  health  is  very  good,  the  digestive  and  urinary  func- 
tions being  perfect.  The  heart  is  regular,  is  not  enlarged,  and  the  pulse 
tension  is  normal.  There  are  no  cardiac  murmurs,  but  a  blowing  mur- 
mur is  heard  over  the  arch  of  the  aorta  synchronous  with  the  first 
sound.  There  is  no  area  of  dulness,  and  there  are  no  other  symp- 
toms to  suggest  aneurism.  No  cause  is  to  be  found  for  this  condition, 
which  has  advanced  so  gradually  that  its  actual  date  of  onset  is  diffi- 
cult to  determine,  though  she  is  certain  that  it  began  more  than  five 
years  ago. 

The  very  extraordinary  appearance  of  the  head  in  this  patient  sug- 
gested to  me  that  she  might  be  suffering  from  a  condition  known  as 
leontiasis ;  but  a  careful  study  of  the  literature  regarding  leontiasis 
convinced  me  that  under  this  name  a  number  of  entirely  different  con- 
ditions have  been  grouped. 

The  term  leontiasis  has  been  applied  to  a  form  of  leprosy  affecting 
the  face,  and  very  clearly  described  by  Leloir  in  his  monograph  upon 
lepra,  where  several  plates  illustrating  the  appearance  of  the  face  in 
this  disease  are  to  be  found.  In  this  condition  there  occurs  a  deposit 
of  new  material  here  and  there  upon  the  face  beneath  the  skin,  and 
these  deposits  gradually  enlarge  and  coalesce  until  the  entire  face  is 
changed  into  a  hard  mass  with  deep  seams  and  roughened  surface,  the 
features  being  almost  obliterated  and  the  eyes  sometimes  closed.  The 
bones  of  the  face  are  not  usually  involved,  and  the  disease  does  not 
extend  upward  beyond  the  line  of  the  hair,  and  does  not  produce  any 
enlargement  of  the  cranial  bones.  The  term  leontiasis  has  been  ap- 
plied to  this  because  the  rough,  seamed,  and  thickened  appearance 
about  the  mouth  suggests  the  idea  of  the  jaw  of  a  lion.  It  is  said  that 
the  term  has  been  in  use  since  the  time  of  Galen. 

There  is  a  second  condition  to  which  the  term  leontiasis  has  been 
applied  —  namely,  a  condition  of  elephantiasis  affecting  the  tissues  of 
the  head  and  neck.  This  is  mentioned  by  Virchow  in  his  work  upon 
tumors.  He  describes  it  as  a  diffuse  thickening  of  the  soft  parts  of  the 
head  and  face,  consisting  of  an  increase  in  fibrous  tissue,  and  considers 
it  a  form  of  fibroma  molluscum.  This  form  corresponds  in  its  history 
to  elephantiasis  ;  it  begins  quite  suddenly  with  an  acute  condition, 
which  may  be  either  erysipelas  or  dermatitis  or  phlebitis ;  the  skin 
becomes  swollen  and  red  simultaneously,  and  there  is  often  an  erup- 
tion of  vesicles  after  the  acute  symptoms  subside,  but  the  swelling 
does  not  disappear  entirely,  and  the  skin  pits  upon  pressure.  Later, 
there  is  a  recurrence  of  the  acute  condition  and  an  increased  swelling  ; 
and  so  the  disease  goes  on  with  acute  exacerbations  and  remissions  ; 
ulceration  of  the  surface  and  very  deep  pigmentation  are  commonly 
observed  in  the  parts  affected.     After  describing  this  condition  and 


950  TROPHIC   NEUBOSES. 

recording  a  clinical  case  described  by  Gruber,  Virchow  says  :  "I  do 
not  wish  to  affirm  that  there  is  not  a  form  of  leprosy  which  calls  to 
mind  the  appearance  of  leontiasis ;  on  the  contrary,  I  have  often  seen 
such  a  form  of  leprosy  in  Norway,  but  the  greatest  deformity  occur- 
ring ordinarily  without  ulceration  is  the  peculiar  characteristic  of 
fibroma  moJluscum,  or  elephantiasis  molluscum.'" 

I  think  it  is  quite  evident  that  the  disease  from  which  this  patient  is 
suffering  does  not  in  any  way  correspond  to  either  of  these  two  dis- 
eases to  which  the  term  leontiasis  has  been  applied. 

There  is  a  third  disease  to  which  Virchow  gives  the  name  bony 
leontiasis  (leontiasis  ossea),  which,  however,  resembles  megalo-cephalie 
more  closely.  In  his  description  of  tumors  of  a  bony  nature,  after 
describing  exostosis  and  periosteal  thickenings,  Virchow  devotes  a 
section  to  hyperostosis  of  the  face  and  head.  This  may  take  two  forms  : 
first,  a  difiFuse  hyperostosis  of  the  cranium  ;  secondly,  localized  hyper- 
ostosis upon  the  maxillary  bones.  This  latter  condition  we  may  dis- 
miss without  further  consideration,  inasmuch  as  it  presents  no  points  of 
a  resemblance  to  our  own  case.  It  has  been  studied  carefully  by  Le 
Dentu  in  La  Revue  mensuelle  de  Medecine  et  de  Chirurgie,  1879.  The 
diffuse  hyperostosis  of  the  cranium  is,  according  to  Virchow,  sym- 
metrical and  uniform,  so  that  it  does  not  present  any  bony  excrescences 
and  suggests  the  idea  of  tumor.  Virchow's  description  is  based 
upon  the  examination  of  certain  skulls,  which  are  preserved  in  various 
museums,  the  first  one  having  been  described  by  Malpighi,  1 G97.  There 
are  about  six  of  these  skulls  known  in  Europe,  all  of  them  presenting 
tremendous  enlargement  of  the  bones  of  the  head  of  a  diffuse  charac- 
ter. Virchow  says  :  "  This  condition  is  for  the  skeleton  what  leon- 
tiasis is  for  the  soft  parts ;  the  appearance  of  these  pathological 
specimens,  seen  a  certain  distance,  produces  the  effect  of  leontiasis 
modelled  in  plaster.  Hence,  I  have  the  less  hesitation  in  giving  the 
name  of  bony  leontiasis,  since,  as  I  believe,  the  disease  corresponds 
exactly  with  that  which  we  call  elephantiasis  in  the  soft  parts.  Ordi- 
narily, the  hyperostosis  is  associated  with  a  fibrous  change  in  the  soft 
parts  ;  in  the  rare  cases  which  we  have  seen,  it  is  the  periosteum  which 
is  the  principal  seat  of  the  disease.  Unfortunately,  we  have  no 
records  of  clinical  observations,  and  no  knowledge  of  the  exact  condi- 
tions present  by  recent  autopsy."  ^ 

In  a  monograph  upon  leontiasis  ossea,^  Baumgarten  has  made  a 
careful  stiidy  of  this  condition  described  by  Virchow,  and  has  collected 
the  descriptions  of  all  these  skulls  in  the  various  museums  of  Europe. 
He  has  shown  that  some  of  these  skulls  present  a  uniform  thickening 
of  all  the  bones,  while  others  have  shown  an  enlargement  limited  to 
the  bones  of  the  face,  the  cranium  escaping.  He  points  out  the  fact 
that  the  latter  class  of  skulls  probably  belonged  to  patients  suffering 
from  the  disease  aci'omegalia,  in  which,  as  is  well  known,  the  bones 

^VircKow:  Pathol,  des  Tumeurs,  vol.  i.,  p.  324." 

2  Virchow  :  loc.  cit.,  vol.  ii.,  p.  22. 

^  La  Leontiasis  Ossea,  par  Dr.  F.  Baumgarten,  Paris.     Steinheil,  1892. 


ulcSbations  of  neubotic  obigin.  951 

of  the  face^  are  very  markedly  enlarged.  It  is  evident  from  Baum- 
garten's  study  that  the  term  leontiasis  ossea  must  be  limited  to  the 
cases  in  which  the  hyperostosis  of  bone  has  affected  the  bones  of  the 
cranium  only,  or  these,  together  with  the  bones  of  the  face.  He  con- 
siders that  the  pathological  changes  present  are  either  a  thickening 
of  the  surface  of  the  bone  alone,  or  a  change  both  in  the  surface  and 
the  diploe  ;  the  latter  being  in  some  cases  transformed  into  a  spongy 
tissue  ;  in  others,  into  a  hard  tissue  like  ivory.  He  admits  that  the 
literature  does  not  contain  any  description  of  this  disease  observed 
during  life,  and  he  does  not  think  that  cases  have  been  observed. 

It  is  my  opinion  that  the  case  here  described  is  this  disease,  and  that 
we  can  affirm  that  in  these  cases  there  is  not  only  a  hypertrophy  of  the 
bony  tissue,  but  also  of  the  soft  parts  about  it.  It  is  probable  that  the 
diffuse  thickening  of  the  bones  of  the  head  and  neck  causes  some  pres- 
sure upon  the  contents  of  the  skull  and  upon  the  nerves  at  their  exits 
between  the  vertebrae.  This  might  explain  the  symptoms  which  are 
present  in  our  patient  —  especially  the  complaints  of  numbness  and  the 
difficulty  in  her  gait. 

Various  forms  of  treatment  have  been  pursued  in  the  case  under 
consideration.  A  thorough  trial  of  thyroid  extract  failed  to  affect  the 
condition  in  any  way,  and  did  not  cause  any  variation  in  temperature 
from  normal.  The  use  of  arsenic  and  strychnine,  given  in  moderate 
doses,  appears  to  have  had  some  tonic  effect,  and  these,  together  with 
the  moderate  use  of  corrosive  sublimate  internally,  are  the  remedies 
relied  upon. 

I  have  ventured  to  give  the  condition  the  name  megalo-cephalie, 
because,  as  already  shown,  the  term  leontiasis  has  been  applied  to  three 
entirely  distinct  conditions,  and  therefore  leads  to  confusion. 

Since  this  case  was  described  by  me  in  1894  several  similar  cases 
have  been  put  on  record.  They  all  illustrate  the  condition  of  a 
localized  hypertrophy  of  the  bones  of  the  head. 

Ulcerations  of  Neurotic  Origin. — The  most  important  trophic 
disturbances  are  those  which  consist  of  an  actual  disintegration  of 
tissue.  These  are  very  numerous.  Herpes  zoster,  certain  forms 
of  eczema  and  pemphigus,  lepra  ansesthetica,  scleroderma,  acute 
ulceration  of  the  fingers,  perforating  ulcer  of  the  foot,  acute  bed-sores, 
keratitis  with  anaesthesia  of  the  cornea,  sympathetic  ophthalmia,  and 
various  forms  of  joint  disease,  are  examples  of  such  trophic  diseases. 
For  the  discussion  of  the  skin  and  eye  diseases  mentioned,  the  reader 
is  referred  to  special  articles  on  those  subjects.  Herpes  zoster  has 
been  discussed  in  the  chapter  upon  Neuralgia,  page  731.  In  regard  to 
lepra  anaesthetica,  reference  may  be  made  to  page  243. 

I  have  reported  a  case  of  acute  ulceration  of  the  ends  of  the  fingers 
and  toes  occurring  in  an  anaemic  girl  aged  twelve,  and  persisting  for 
more  than  a  year,  associated  with  cyanosis  of  the  hands  and  feet,  and 
with  a  tendency  to  ra))id  ulceration  of  any  part  of  the  body  which 
happened  to  be  exposed  to  pressure.  The  hair  of  the  eyebrows,  eye- 
lids, and  to  a  less  extent  that  of  the  seal]),  fell  out  during  the  disease. 


962  TROfSlC  NEUMOBES. 

There  was  no  local  asphyxia  and  no  appearance  of  gangrene ;  hence 
the  case  was  not  one  of  Raynaud's  disease.  Its  symmetrical  distrsbu- 
tion,  the  lack  of  any  constitutional  organic  disease  or  of  any  local 
cause  for  the  production  of  the  ulceration,  and  the  anaemic  and  nerv- 
ous condition  present,  all  pointed  to  a  disturbance  of  the  central 
nervous  system.  It  did  not  yield  to  treatment,  either  local  or  general 
or  electrical.     Mills  has  seen  a  similar  case. 

Perforating  ulcer  of  the  foot  begins  on  the  sole,  beneath  any  of  the 
metatarso-phalangeal  articulations,  preferably  the  first  or  the  fifth,  or 
under  the  heel,  as  a  small  pustule  under  the  epidermis.  This  rup- 
tures, and  the  ulcer  which  results  begins  to  extend  in  a  direction  verti- 
cal to  the  surface,  involving  the  deeper  tissues  or  even  opening  into 
the  joint  and  destroying  the  bone.  It  appears  rather  like  a  sinus  than 
an  ulcer,  and  is  remarkable  from  the  fact  that  it  is  not  painful  and  is 
insensitive  to  touch,  although  it  may  prevent  the  patient  from  walking 
on  account  of  extreme  tenderness.  The  epidermis  around  the  sinus  is 
thickened  and  inseusitive,  and  there  may  be  anaesthesia  of  the  entire 
sole  of  the  foot,  and  even  of  the  leg,  although  this  is  due  to  the  neu- 
ritis present,  which  is  also  the  cause  of  the  ulcer,  rather  than  to  the 
ulceration.  The  circulation  is  sluggish  in  the  affected  extremity ;  it 
becomes  cyanotic  on  exposure  to  cold,  and  seems  peculiarly  liable  to 
become  oedematous.  The  oedema  may  go  on  to  suppuration,  and  in- 
volve the  articulations,  and  ankylosis  of  the  smaller  joints  may  follow. 
This,  too,  is  to  be  traced  to  the  neuritis.  The  skin  of  the  foot  becomes 
pigmented,  and  may  be  dried  or  covered  with  offensive  sweat.  As 
the  patient  cannot  walk  while  the  ulcer  remains,  the  condition  demands 
treatment.  Rest,  moist  warm  applications,  antiseptic  lotions,  scraping 
out  the  sinus,  and  other  surgical  means  appropriate  to  the  treatment 
of  ulcers  and  sinuses  do  not  often  prove  of  benefit,  and  in  obstinate 
cases  recourse  has  been  had  to  amputation  of  the  foot.  Electrical 
treatment  has  been  tried  in  vain. 

Such  perforating  ulcers  may  occur  symmetrically  on  both  feet,  and 
may  be  numerous.  They  also  occur  rarely  on  the  hands.  They  have 
been  observed  not  infrequently  in  locomotor  ataxia,  occurring  either 
early  or  late  in  the  disease,  see  page  363,  and  have  appeared  in  patients 
suffering  from  dementia  paralytica.  The  only  constant  lesion  found 
is  a  degeneration  of  the  peripheral  nerve  supplying  the  affected  part. 

Acute  bed-sores  (decubitus)  develop  in  many  cases  of  spinal  and 
cerebral  disease,  but  not  in  all.  It  is  argued  that  anaesthesia  of  a  part 
or  simple  pressure  upon  a  part  or  irritation  of  an  anaesthetic  part  will 
not  explain  their  occurrence,  and  that  they  must  be  traced  to  a  destruc- 
tion of  trophic  centres  in  the  cord  or  brain.  No  amount  of  attention 
to  the  position  and  cleanliness  of  the  patient  is  thought  to  be  sufficient 
to  prevent  them  in  some  cases,  and  their  progress  is  often  so  rapid  as 
to  remove  them  from  the  category  of  simple  ulceration.  Their  most 
frequent  seat  is  upon  the  buttocks,  over  the  sacrum,  on  the  heels,  and 
over  the  scapulae ;  but  it  is  believed  that  in  the  conditions  in  which 
they  appear  in  these  places  pressure  on  any  part  may  cause  one.     They 


ULCERATIONS  OF  NEUROTIC   OBlGIN.  953 

begin  within  a  few  hours  after  the  occurrence  of  the  lesion  as  irregular 
mottled  patches  of  redness,  in  which  there  soon  appear  small  or  large 
vesicles  filled  with  dark  serum.  These  rupture,  leaving  little  areas  of 
ulcerated  surface,  which  soon  unite  to  form  a  large  ulcer  with  softened, 
infiltrated,  and  bloody  base  and  ragged  edges.  The  tissues  around  and 
within  the  ulcer  have  a  tendency  to  slough  and  to  become  gangrenous, 
and  the  process  goes  on  rapidly  until  a  considerable  area  of  the  surface 
is  completely  destroyed,  together  with  the  deeper  structures,  fat, 
muscles,  and  fasciae,  down  to  the  bone,  which  may  become  necrosed. 
Acute  cystitis  is  so  frequently  associated  with  acute  bed-sore  that  it  is 
traced  to  the  same  nervous  cause,  though  here,  again,  another  expla- 
nation is  possible.  The  constitutional  disturbance  produced  by  these 
two  affections  may  be  increased  by  a  true  septicaemia,  to  which  the 
patient  succumbs  ;  or  metastatic  abscesses  may  be  formed,  and  pyaemia 
cause  death  ;  or,  lastly,  the  ulcer  on  the  back  may  lay  bare  the  vertebrae 
and  erode  their  ligaments,  thus  opening  a  way  for  the  extension  of; 
suppuration  to  the  meninges  of  the  spinal  cord.  The  duration  may  be 
from  a  few  days  to  two  weeks,  and  the  prognosis  is  very  grave.  ; 

Chronic  bed-sores  develop  in  a  similar  manner,  although  all  the 
processes  described  occur  more  slowly  and  are  much  less  severe.  They 
do  not  show  the  tendency  to  extend  so  widely  or  so  deeply,  nor  to 
become  gangrenous.  They  remain  stationary  after  attaining  a  certain 
size,  and  the  tissues  seem  to  lack  all  tendency  to  reproduction  and 
repair.  When  the  central  lesion  which  they  follow  begins  to  pass 
away  they  heal  ;  sometimes  local  treatment  is  successful  ;  but  in  many 
cases  they  persist  for  years,  always  threatening  the  life  of  the  patient 
from  the  possibility  of  their  sudden  aggravation  or  from  the  occur- 
rence of  the  complications  already  mentioned  in  connection  with  the 
acute  process. 

Since  neither  form  of  decubitus  occurs  after  lesion  of  the  anterior 
cornua  of  the  spinal  cord,  nor  in  sclerosis  of  the  posterior  or  lateral 
columns,  nor  in  those  cases  of  locomotor  ataxia  in  which  extensive 
invasion  of  the  posterior  cornua  is  present,  while  they  are  particularly 
frequent  as  a  complication  of  general  myelitis,  hemorrhage  in  the  cord, 
and  syringo-myelitis,  it  is  argued  that  the  trophic  centres  whose  destruc- 
tion is  the  cause  of  tliese  bed-sores  lie  in  the  central  gray  matter  of  the 
cord,  near  to  the  central  canal.  In  unilateral  lesion  of  the  cord  rhe 
bed-sore  occurs  on  the  anaesthetic  side  of  the  body  —  i.  e.,  on  the  side 
opposite  to  the  seat  of  the  lesion  —  a  fact  which  may  indicate  a  decus- 
sation of  the  trophic  nerve-fibres  in  the  cord,^  but  which  has  also  been 
urged  in  proof  of  the  theory  that  the  sore  is  always  due  to  irritation 
of  an  insensitive  surface. 

The  treatment  of  bed-sores  belongs  to  the  domain  of  surgery,  the 
same  methods  being  pursued  as  in  the  case  of  any  large  ulcer.  The 
application  of  electricity  to  the  sore  has  been  tried,  but  the  results  are 
not  sufficiently  encouraging  to  warrant  its  recommendation. 

IRecklinpTiausenhelievesthat  these  cases  of  disturbance  of  nutrition  can  be  explained 
by  injury  of  :in;eslheiic  parts  in  which  vaso-niotor  disiurbances  of  central  origin  exist 
(Ilandbuch  d.  Path,  d.  Ernahrung,  pp.  230,  2.'57), 


954  TBOFHIC  NEVEOSES. 

Joint  affections  of  neurotic  origin  are  discussed  in  the  chapters  on 
Locomotor  Ataxia,  Hemiplegia,  and  Hysteria. 

It  is  evident  from  this  review  that  the  nervous  centres  have  some 
influence  upon  the  activity  of  the  cells  of  which  the  body  is  made  up, 
and  that  they  control  the  processes  of  growth,  function,  repair,  and 
reproduction.  The  facts  are  too  numerous,  too  varied,  and  too  positive 
to  admit  of  any  other  explanation.  Trophic  disturbances  must  there- 
fore be  considered  as  a  set  of  symptoms  referable  to  various  lesions  of 
the  nervous  system.  It  is  evident  from  the  preceding  discussion  that 
they  may  be  produced  by  disease  of  the  peripheral  nerves ;  by  disease 
of  the  gauglionic  cells,  which  nourish  those  nerves  ;  by  disease  of  the 
spinal  cord,  especially  in  the  region  of  the  central  gray  matter;  and, 
finally,  by  disease  of  the  brain.  Whether  in  the  last  condition  the 
effect  is  a  direct  one,  or  is  produced  secondarily  by  an  irritation  of  the 
spinal  centres,  cannot  yet  be  determined.  There  are  no  trophic  centres 
as  yet  localized  in  the  cerebrum,  but  the  pathological  facts  already 
mentioned  warrant  the  conclusion  that  such  centres  will  not  long  elude 
search.  Certain  facts  observed  in  cases  of  infantile  hemiplegia  point 
to  the  motor  area  of  the  cortex  as  the  seat  of  trophic  centres  for  the 
motor  mechanisms ;  since  it  is  found  that  when  the  motor  cortex  is 
destroyed  in  early  life  the  bones  and  muscles  which  it  controls  fail  to 
develop  properly.  Trophic  centres  for  sensory  mechanisms  are  not 
yet  discovered.  Certain  investigations  of  Luciani  point  to  the  cerebel- 
lum as  the  part  of  the  brain  which  governs  the  general  nutrition  of 
the  body,  but  these  need  confirmation. 

Conclusion.  —  While  an  attempt  has  been  made  here  to  consider 
vaso-motor  and  trophic  neuroses  separately,  it  must  be  admitted  that  in 
very  many  conditions  the  two  are  coincident.  This  follows  inevitably 
from  what  has  been  stated  regarding  the  localization  of  the  vaso-motor 
and  trophic  centres  in  the  spinal  cord,  and  regarding  the  course  of  the 
vaso-motor  and  trophic  nerves  from  the  spinal  centres  to  the  periphery. 
These  two  classes  of  centres  and  nerves  lie  side  by  side  in  the  central 
and  peripheral  organs,  and  it  is  less  surprising  that  they  should  be 
jointly  affected  than  that  one  should  ever  be  involved  alone.  In  any 
case  of  lesion  of  the  peripheral  nerves  or  of  the  central  nervous  sys- 
tem they  may  be  expected.  In  all  cases  they  are  to  be  regarded  as 
symptoms  of  such  lesions  rather  than  as  distinct  diseases. 


INDEX. 


ABDOMINAL  reflex,  62 
Abducens   nerve,   642 
paralysis,  642 
Abnormal     positions     assumed,     signifi- 
cance of,  596,  600 
Abscess  of  brain,  549.     See  Brain,  ab- 
scess of 
Acoustic  nerve,  662 

diseases  of,  663 
Acrodynia,  928 
Acroparsesthesia,  39 
Agraphia,   123,   139 
Alcoholic  dementia,  218 

epilepsy,    824 

neurasthenia,  8'50' 

neuritis,  212 
Alexia,  123,  137 
Alternating  ana?sthesia,  117 
Amaurotic  family  idiocy,  634 
Ambulatory  automatism,  817 
Amnesia,  137 
Amyotrophic  lateral  sclerosis,  294,     See 

Sclerosis,  amyotrophic  lateral 
Anaemia,  cerebral,  468 

of   optic   nerve,   628 

pernicious,  392 

of  spinal  cord,  392 
Anaemic  vertigo,  901 
Anaesthesia,  alternating,  117 

in  brain  disease,   106 

in  Cauda  equina  lesions,  76 

dolorosa,  42 

in  hysteria,  77,  882 

in  locomotor  ataxia,  364 

in  neuritis,  41 

in  pons  lesions,  117 

in  spinal  cord   disease,  73 

subsidence  of,  42 

in  syringomyelia,  75 
Analgesia,   73 
Anarthria,  101,  141 
Aneurism   of   aorta   compressing   spinal 

cord,  449 
Angina,   hysterical,   885 
Angio-neurotic    rndoma,    938 


Anglo-paralysis,    926 
Angiospasm,  925 
Ankle  clonus,  63 
Ankylosis,  spinal,  452 
Anosmia,  624 
Aphasia,  176 

due   to   tumors,   593 

examination  in,   141 

varieties  of,  136,  138 
Aphonia,   hysterical,   883 
Aphthongia,    674 
Apoplectic  neuritis,  176 
Apoplexy,  472 

athetosis  in,  498' 

attack  of,  485 

cerebellar,  491 

diagnosis  of^  500 

etiology   of,   480 

gait  in,  488 

gradual  onset  in,  495 

irregular  types  of,  493 

without  loss  of  consciousness,  495 

pathology  of,  473 

premonitions  of,  482,  468 

prognosis   of,   504 

recurrences   of,   496 

symptoms   of,   482 

mental  of,  484,  490 

tendon  reflexes  in,  497 

terminal  state  of,  496 

treatment  of,  506 
Apraxia^   136 

Aran-Duchenne's  paralysis,  285,  288 
Argyll-Eobertson  pupil,  641,  645 
Arm,   spasms   of,   93 
Arrangement    of    ocular    nerve    nuclei, 

638 
Arsenical   neuritis,   224 
Arterial   sclerosis,  468,  474.     See   Scle- 
rosis, arterial 
Arteries,  spasm  of,  926 
Arthronathy  in  locomotor  ataxia,  366 

in   syringomyelia,  331 
Asphyxia,   local,    932 


955 


956 


invei. 


Association  fibres,  134 

of  ideas,  135,  144 
Astasia-abasia,  883 
Astereognosis,   109 
Ataxia,   77,   361 

cerebellar,   599,  600 

Friedreich's,  388 
Ataxic  paraplegia,  383 
Atrophic   paralysis,   284 
Atrophy,  942 

cerebral,  514 

of   glands,   944 

muscular,  316 
peroneal,  316 
Hofifman's,   316 

of  optic  nerve,  634 

of  skin,  944 
Alternating  paralysis,   105 
Auditory  nerve,  662 

tract,  128-131 

vertigo,  808 
Aura  in  epilepsy,  814 
Axis  cylinder,  25 

histology  of,  36 
Axone,  collaterals  of,  25^  30 

diameter  of,   36 

ending  of,  25,  26,  30 

Golgi's  types  of,  26 

histology  of,  36 

types  of,  26 

BABINSKI'S  reflex,  62 
view  of  hysteria,  887 
Balance  of  body,  666 
Barany's  test,  667 
Basal  ganglia,  145 

tumors  of,  595 
Bechterew's  nucleus,  665 
Bedsores,  80,  952 
Bends.     See  Caisson  disease. 
Beri-beri,  245 
Birth  palsy,  179 
Bladder  mechanism,  64 
Blepharospasm,    791 
Blindness,  122,  126 

hysterical,   882 

psychical,  122 
Bloodvessels   of  brain,  463 

of  spinal  cord,  259 
Brachial  neuralgia,   71 

neuritis,  176 
Brain,  abscess  of,  549 


Brain,  abscess  of,  diagnosis  of,  562 
etiology  of,  549 
pathology   of,  552 
stages  in,  559 
symptoms    of,    559 
treatment  of,  563 
anaemia  of,  000 
bloodvessels  of,  463 
cortex,  convolutions  of,  83 
fissures   of,   83 
structure  of,  85 
cysts  in,  517 

diseases  of,  pargesthesia  in,  95,  ICG 
paralysis  in,  99,  103 
symptoms    of,    anaesthesia    in, 
106 
mental,  86 
pulse  in,  90 
respiration  in,"  90 
temperature   in,   90 
ear  and,  relations  of,  564 
hyperaemia  of,   000 
maldevelopment  of,  514 

clinical  groups  of,  514 
epilepsy  due  to,  527 
etiology  of,  514 
frequency  of,  514 
mental  defects  in,  528 
pathology   of,  515 
prognosis  of,  530 
sensory   defects  in,   529 
symptoms   of,  522 
treatment  of,  530 
sclerosis  of,  516 
sensory  tracts  in,  112 
tumors   of,    571 
course  of,  606 
diagnosis  of,  602 

from  abscess,  603 
from  anaemia,  605 
from   hemorrhage,   605 
from   hydrocephalus,   604 
from  lead  poisoning,  605 
from  meningitis,  604,  605 
from  nephritis,  606 
duration  of,  606 
etiology  of,  571 
pathology   of,   573 
prognosis  of,  607,  611 
statistics  of,  572 
sudden  death  in,  607 
symptoms  of,  582 


INDEX. 


957 


Brain,  tumors  of,  symptoms  of,  general, 
**     584 
local,  589 
treatment  of,  608 
medical^  608 
surgical,  608 

results  of,  609 
ventricular    puncture    in,    609 
x-ray  examination  in,  605 
venous   sinuses    of,    thrombosis    of, 
567 
Breast,   neuralgia   of,   743 
Bulbar  paralysis,  614 

acute  apoplectiform,  615 
chronic,  616 

diagnosis  of,  619 
etiology  of,  616 
pathology  of,   616 
symptoms    of,    616 
prognosis  of,  620 
Bulbs,  olfactory,  624 
Burdack,  column  of,  68 

CAISSON  disease,  701 
diagnosis  of^  704 
etiology  of,  701 
pathology   of,  702 
symptoms  of,  702 
treatment  of,  704 
Calcarine  cortex,  120 
Cancer   and   neuritis,   256 
Carbon-monoxide   as   cause    of   neuritis, 

222 
Carcinoma  of  vertebree,  437 
Cardiac  plexus,  34 
Caries  of  spine,  433 
Catalepsy,  886 
Cauda  equina,  lesions  of,  428 
anaesthesia  in,  76 
paralysis  in,  76 
Cells,  motor,  of  spinal  cord,  53 

size  of,  22 
Cerebellar  apoplexy,  491 
ataxia,  599,   600 
attacks,  600 
encephalitis,  535 
pontine  angle,  tumors  of,  601 
tract,   118 
vertigo,  900 
Cerebellum,  anatomy  of,  150 
connections  of,  153 
lesions  of,  symptoms  of,  151,  153 


Cerebellum,  middle  peduncle,  lesion  of, 
153 
tumors  of,  598 
Cerebral  anaemia,  468 
atrophy,  514 

axis,  sensory  tracts  in,  114 
circulation,    463 
embolism,   476 

situation  of,  480 
hemorrhage,   473 

situation  of,  480 
vs.   embolism,   501 
vs.   thrombosis,  502 
hypersemia,  467 
meningitis,    713,      See    Meningitis, 

cerebral 
puncture,  605 
syphilis,  690 
thrombosis,  476 

situation  of,  480 
Cerebrospinal  fluid,  155 

in  meningitis,  716 
meningitis,  717 

syphilis,  treatment  of,  salvarsan  in, 
682 
Cervical  sympathetic  nerve,  175 

symptoms    of    injury    of, 
175 
Cervico-brachial  neuritis,   174 
Cervico-occipital  neuralgia,  741 
Characteristics  of  voluntary  acts,   99 
Charcot-Marie-Tooth  disease,  316 
Charcot's  joints,  366 
Chemical  changes  in   degeneration,  33 
Cheyne-Stokes  respiration,  670 
Choked  disc,  630 
Chorea,  778,  785 

diagnosis  of,  783 
duration  of,  782 
electrical,  801 
etiology  of,  778 
hereditary,  786 
Huntington 's,   785 
pathology  of,  783 
prognosis  of^  783 
Sydenham's,   778 
symptoms  of,   780 
treatment  of,  783 
Chromophile  bodies,   22 
Circumflex   nerve,   182 
Climatic  neurasthenia,  851 
Clonus.  63 


958 


INDEX. 


Coal  tar  products  as  cause  of  neuritis, 

224 
Coccygodynia,  750 
Coccyx,  neuralgia  of,  750 
Cochlear  nerve,  662 
Collaterals  of  axons,  25,  30 
Coma,  88 
Combined      sclerosis.        See      Sclerosis, 

combined 
Commissural  fibres,   142 
Compression  of  spinal  cord,  443 
Consciousness,  870 
Conjugate  motion   of  eyes,   643 
Convulsions,  89 

of  epilepsy,  815 

localized,  91 
Copper  poisoning,  234 
Corpora  quadrigemina,  147 

tumors  of,  597 
Coprolalia,  801 
Corpus  callosum,  143 

tumors  of,  594 
Corti,  organ  of,  662 
Cortical  acts  vs.  spinal  acts,  100 

areas,  evolution  of,  143 

epilepsy,  91 
Counterirritants,  action  of,  922 
Cranial  nerves,  622 

puncture,   605 
Cranio-cerebral  topography,  156 
Crural   neuralgia,    744 
Crus  cerebri,  tumors  of,  597 
Cuneus,   120,  121 
Cystitis,   80 
Cysts  in  brain,  517 

DEAFMUTISM,  514,  529 
Deafness,  663 
cerebral,  126 
hysterical,  882 
psychical,  126 
word,   127 
Decubitus,  952 

Degeneration,   chemical   changes   in,   33 
of  nerves,  159 
reaction  of,  44 
of  spinal  cord,  402 
Deiters'  nucleus,  665 
Delirium,  hysterical,   881 
Delusions  in  paresis,  543 
Dementia,  alcoholic,  218 
organic,  491 


Dementia  paralytica.     See  Paresis 
in  paresis,  543 
senile,   470 
Dendritis,  22 

Depression  in  paresis,  543 
Diabetic  neuritis,  253 
Diadokokinesis,  600 
Diphtheritic  neuritis,  235 

paralysis,  235 
Diplegia,  514,  525 
Disseminated  sclerosis,  675 
Disturbances    of   taste    due   to    tumors, 

593 
Dizziness,  86 
Double  consciousness,  868 
Duchenne  's  ascending  paralysis,  285,  288 

paralysis,  179 
Dysbasia  lordotiea,  799 
Dystonia  musculorum  deformans^  799 
Dystrophies,  muscular,  303 
etiology  of,  304 
pathology   of,  305 
prognosis  of,  315 
symptoms  of,  307 
treatment  of,  316 
varieties  of,   303,   307 
Dystrophy,  Erb's  juvenile,  312 
Laundouzy-Dejerine,   313 

EAE,   brain   and,   relations   of,   564 
Ecchymoses,  368 
Echolalia,  790 
Eighth  nerve,  662 
Electrical  charges  in   neuritis,  44 

reactions,  44 
Eleventh  nerve,  671 
Emaciation,  91 
Embolism,  cerebral,  476 

of  retinal  artery,  628 
Emotional  control,   disturbance  of,   144 

origin  of  hysteria,  891 
Encephalitis,  535 

cerebellar,  535 

etiology  of,  535 

hemorrhagic,  535 

pathology  of,  536 

prognosis  of,  538 

symptoms  of,  537 

treatment  of,  539 

varieties  of,  535 
Endarteritis,  468,  474 

syphilitic,   688 


INDEX. 


969 


Endoneurium,  39 

Epicritic  sen^tions,  42 

Epidemic  cerebrospinal  meningitis,  717 

symptoms  of,  717 
Epilepsy,  813 

alcoholic,  824 

aura  in,  814 

course  of,  819 

convulsions  of,  815 

cortical,  91 

degeneration   marks,   820 

diagnosis  of,  830 

etiology  of,  821 

hereditary,  821 

Jacksonian,   91,   816 

mental  equivalent,  817 

petit  mal,  813 

procursive,  813 

prognosis  of,  831 

psychical  attacks,  817 

reflex,   825 

spinalj   59 

status  epilepticus,  817 

theories   of,   826 

organic   origin,   826 
toxa?mic  origin,  829 

traumatic,  823 

treatment,  8'32 
medical,  832 
surgical,  836 

varieties  of,  813,  818 
Equilibrium,  666 
Erb's  juvenile  dystrophy,  312 

paralysis,  179 

paraplegia,   693 

spinal  syphilis,  693 
Eruptions  after  neuritis,  42 
Erythromelalgia,  44,  927 
Examination   of  patient,   17,  20 
Excitement  in  paresis,  543 
External   cutaneous  nerve,   190 

popliteal  nerve,   192 

"nACE,  hemiatrophy  of,  654,  944 
-L     herpes  zoster  of,  653 

hypertrophy   of,  945 
Facial  nerve,  655 

paralysis,  656 

spasm,  93,  791 
Family  periodic  paralysis,  774 
Feeble-mindedness,  514,  528 
Fibrillary  twitching  of  muscles,  60 


Fifth  nerve,  649 

Foot,  perforating  ulcers  of,  952 

Fourth  nerve,  642 

Fracture    of    vertebrae,    effects    of,    421 

Fragilitas  ossium,  943 

Freud's  theories  criticised,  890 

view   of   hysteria,   888 
Friedreich's  ataxia,  388 
Functional  vs.  organic   disease,   18 

GAIT  in  apoplexy,  488' 
in  disease,  65 

in  spinal  disease,  65 
Galton's  tests,  664 
Ganglion,  Gasserian,  650 

of  Scarpa,  665 
Gangrene  after  neuritis,  43     - 

symmetrical,  932 
Gas  poisoning,  222 
Gaskell's  researches,  920 
Gasserian   ganglion,   650 
General    vs.    local    symptoms    in    brain 

disease,  82 
Genital  reflex,  62 
Gerlier's  disease,  900 
Gilles  de  la  Tourette's  disease,  783 
Gliosis     spinalis.       See     Syringomyelia, 

320 
Glosso-labio-laryngeal  paralysis,  616 
Glosso-pharyngeal  nerve,   667 
Glossy   skin,  943 

in  neuritis,  43 
Golgi's  types  of  axones,  26 
Goll,  column  of,  70 
Gordon 's  reflex,  62 
Gouty  neuritis,  251 
Gowers'  disease,  388 
Grand  mal,  813 
Grinding  of  teeth,  653 
Grippe  as  cause  of  neuritis,  239 

HABIT  spasm,  783 
Haemato-myelia,  416 
Hair,  condition  of,  in  neuritis,  43 
Hallucinations,  visual,  124 
Head,  hypertrophy  of,  947 
Headache,   84,   755 
Hearing,  sense  of,  662 
Hemianppsthesia,   108,  110,  118,  499 
Hemianopsia,  120,  125,  627 

from  tumor,  593 
Hemiasynergia,  600 


960 


INDEX. 


Hemiataxia,  110,  115,  118,  499 
Hemiatrophy   of   body,   944 

of  face,  654,  944 

of  tongue,  673 
Hemichromatopsia,   122 
Hemihypertrophy  of  body,  945 
Hemiopic  pupillary  reflex,  628 
Hemiplegia,  104,  495,  497 

infantile,  514 
Hemitremor^   499 
Hemorrhage,  cerebral,  473 

in  meninges,  708' 

spinal,  261,  416 
Hemorrhagic  encephalitis,  535 
Hereditary  chorea,  785 

diseases,  17 

syphilis,  695 
Herpes  zoster,   731 

of   face,   653 
treatment  of,   733 
Heteromere   neurones,    72 
Hoffman 's  muscular  atrophy,  316 
Huntington 's  chorea,   785 
Hydrocephalus,  518,  715,  719 
Hypersemia,  cerebral,  467 

of  optic  nerve,  628 

of  spinal  cord,  392 
Hyperplasia,   syphilitic,   694 
Hypertonia  in  locomotor  ataxia,  364 
Hypertrophy,  945 

of  face,  945 

of  head,  947 

of  skin,  940 
Hypnosis,    874 

Hypnotic  treatment,  887,  895 
Hypnotism,  874 

method  of  production,  874 
Hypoglossal  nerve,  672 
Hypotonia,  364 
Hysteria,  878 

anaesthesia  in,  77 

diagnosis   of,   892 

epidemics  of,  892 

etiology  of,  891 

hypnosis  in,   890 

major,  881 

minor,  880 

prognosis  of,  892 

rest  cure  in,  893 

symptoms,   878^886 

theories  of,  887,  896 

treatment   of.   892 


Hysteria,  varieties  of,  878 
Hysterical  anaesthesia,  882 

angina,   885 

aphonia,  883 

astasia-abasia,  883 

attacks,   881 

blepharospasm,  884 

blindness,  882 

borborigmi,   886 

contracture,   883-884 

cough,  884 

deafness,   882 

delirium,  881 

joints,  885 

pain,   884 

paralysis,  882 

phantom  tumors,  886 

somnolence,  886 

spasms,  881 

suppression  of  menses,  886 
of  urine,  886 

temperament,  878 

tremor,  883 

vomiting,  886 

IDIOCY,  514,  528 
Incisures    of    Schmitt-Lantermann, 
38 
Infantile  hemiplegia,  514 
Infectious  neuritis,  240 
Injuries   of   nerves,    159 
Insomnia,  88,  903 
Instability  of  conduct,  867 
Insular  sclerosis,  675 
Intercortical  aphasia,  138,  139,  140 
Intercostal   nerves,   189 

neuralgia,  742 
Intermittent  claudication,  469,  928 

paralysis,  774 

symptoms,  significance  of,  18 
Internal   popliteal  nerve,  192 
Interstitial  neuritis,  168 
Intracranial  pressure,  466 
Ischgemic  paralysis,  195 
Island  of  Eeil,  tumors  of,  594 

JACKSONIAN  epilepsy,  91,  816 
Janet 's  view   of  hysteria,  887 
Juvenile  dystrophy,  Erb's,  312 

"ITAKKI,  245 

-iV     Klumpke's  paralysis,  175 

Korsakoff's  psychosis,  218 


INDEX. 


961 


Krause,  respirsftory  bundle  of,  668 
Kubisagari,  900 

LANDOUZY  Dejeiine  dystrophy,  313 
Landry's  paralysis^  454 
Language,  disturbance  of,  134 
Laryngeal  vertigo,  902 
Lateral    sclerosis,    337.      See    Sclerosis, 

lateral 
Lead  neuritis,  230 
Leg,  spasms  of,  95 
Lemniscus,  115 

Lenticular   nucleus,    symptoms    of,    145 
Leontiasis  ossea,  949,  950 
Leprous  neuritis,  242 
Lingual  lobule,  594 
Lissauer^  column   of,   70 
Little's  disease,  337,  525 
Local   vs.    general    symptoms   in    brain 

disease,  82 
Localization     of     motor     functions     in 
spinal  cordj  55 
of  reflex  functions  in  spinal  cord, 
61,  4 
Lockjaw,   652 
Locomotor  ataxia,  342 

anEesthesia  in,  364 
arthropathy  in,  366 
ataxia  in,  361 
course  of,  371 
crisis   in,    358 
diagnosis    of,    373 
etiology  of,  350 
gait  in,  362 
hypertonia  in,  364 
joint  affections  in,  365 
mental  complications  in,   369 
onset  of,  356 
paralysis  in,  371 
pathology  of,  342 
stages  of,  351 
symptoms    of,    351 

unusual,  370 
treatment  of,  375 

salvarsan  in,  378 
trophic  disturbances  in,  365 
Lumbar  nerves,  189 
plexus,  189 
puncture,  716 
Lumbo-abdominal  neuralgia,  743 
61 


MALADIE  des  tics  convulsives,  783 
Maldevelopment    of    brain,    514. 
See  Brain,  maldevelopment  of 
Marie's  disease,   685 

view  of  aphasia,  141 
Marshall's   view    of   consciousness,    870 
Mastodynia,  743 
Mauthner,  sheath  of,  38 
Mechanism  of  ordinary  acts,  31 
Median  nerve,  187 
Medulla   oblongata,   148 

lesions  of,  symptoms  of,  150 
tumors  of,  598 
Medullary  centers  of  vasomotor  nerves, 

923 
Megalo-cephalie,  950 
Mendel's  reflex,  62 
Meniere's  disease,  898 
Meningeal  hemorrhage,  708 
Meningitis,  cerebral,  713 

cerebrospinal  fluid  in,   716 
epidemic  type,  717 
in  infants,  719 
pathology  of,   714 
prognosis  of,  727 
secondary  form  of,  721 
septic  type,  721 
symptoms  of,  717 
syphilitic,  716,  726 
treatment  of,  727 
tuberculous,  715,  723 
varieties  of,  713 
spinal,  457,  460.     See  Spinal  Men- 
ingitis 
Meningo-encephalitis.     See  Paresis 
Menses,   suppression   of,  hysterical,   886 
Mental  shock,  as  cause  of  hysteria,  891 
state  in  alcoholic  neuritis,  218 
symptoms  in  brain  diseases,  81 
Mercurial  neuritis,  234 

treatment  of  syphilis,  697 
Method  of  examination,  17,  20 
Migraine,  760 

ophthalmic,   645 
Migratory  neuritis,  168 
Mills'  disease,  496 
Mind  blindness,  122 

due  to  tumors,  593 
deafness,  127 
Mode  of  onset  of  symptoms,  18 
Monoplegia  vs.  hemiplegia,  104 
Morbid   fears,   842,   864 


962 


INDEX. 


Morbid  ideas,  866 
impulses,  865 
Morton's  toe,  193 
Morvan's  disease,  329 
Motor  aphasia,  138 

cells  of  spinal  cord,  53 
centers  of  speech,  100 
nerves  of  eyeballs,  631 
tract,   103 
Mucous  colitis,  848 
Multiple  neuritis,  197 

sclerosis,      675.         See      Sclerosis, 
multiple 
Muscular  atrophy,  316 

dystrophies,  303.     See  Dystrophies, 

muscular 
reflexes,  61 
sense  area  in  cortex,  107 

loss     of,     from    pons     lesions, 

115 
test  of,  109 
tract  of,  113,  114,  115 
tone,  influence  of  cerebellum  on,  600 
Musculo-spiral  nerve,  184 
Myasthenia  gravis,  768 
course  of,  771 
diagnosis  of,  772 
etiology  of,  768 
history  of,  768 
pathology  of,  769 
prognosis   of,   773 
symptoms  of,  769 
treatment  of,   774 
Myasthenic  reaction^  771 
Myatonia   congenita,   766 
Myelin  sheath,  36 
Myelitis,  acute,  394 

cases  of,  405 
course  of,  404 
etiology  of,  395 
pathology  of,  397 
prognosis   of,  408 
symptoms  of,  403 
treatment  of,  409 
chronic,  410 

diagnosis  of,  414 
etiology  of,  410 
pathology  of,  412 
symptoms  of,  413 
treatment  of,  415 
Myelomalacia,  394,  400 
Myoclonia,    778 


Myoclonus  epilepsy,   802 
Myokimia,   778 
Myotonia  congenita,  802 
Myriaehit,  801 

Vr^VUS,  443 
ll      Narcolepsy,  886 
Nasal  vertigo,  902 
Nausea,  88 
Nerves,  39 

abducens,   642 

paralysis  of,  642 
acoustic,    662 
auditory,  662 
circumflex,  182 
cochlear,   662 
cranial,    622 
cutaneous,  external,  190 
degeneration  of,  159 
diseases  of,  39 

diagnosis  of,  46 
symptoms   of,  39 
anaesthesia,   41 
electrical  changes,  44 
pain,  40 
parsesthesia,  39 
.     paralysis,  44 
spasm,  46 

trophic    disturbances,    42 
vaso-motor    symptoms,    43 
vs.  spinal  cord  disease,  46 
eighth,  662 
eleventh,  671 
facial,  655 
fifth,   649 
fourth,   642 
glossopharyngeal,  667 
hypoglossal,  672 
injuries  of,  159 
intercostal,   189 
lumbar,  189 
median,  187 
musculo-spiral,  184 
ninth,    667 

ocular,  paralysis  of,  636,  644 
oculo-motor,   636 
olfactory,   623 
optic,  625 
plantar,  192 
pneumogastric,  668 
popliteal,   external,   192 
internal,  192 


INDEX. 


963 


Nerves,  regeneration  of,  163 

sciatic,  190      ^ 

seventh,  655 

sixth,   642 

spinal  accessory,  671 

suprascapular,  183 

tenth,  668 

third,  636 

paralysis  of,  636 

thoracic,  posterior,  184 

of  tongue,  672 

trigeminal,  649 

paralysis  of,  652 

trophic,  942 

tumors   of,   193 

twelfth,  672 

ulnar,  187 

vagus,  668 

vestibular,  665 

of  Wrisberg,  656 
Nervous  diseases,  causes  of,  general,  18 

ulcers,  951 
Neuralgia,  729 

brachial,   741 

of  breast,  743 

cervico-occipital,  741 

of  coccyx,  750 

course  of,  731 

crural,  744 

etiology  of,  729 

intercostal,  742 

lumbo-abdominal,  743 

pathology  of,  731 

sciatic,  744 

special  forms  of,  733 

symptoms  of,  730 

of  testicles,  749 

treatment  of,  732 

trigeminal,  733 
Neurasthenia,   837 

alcoholic,  850 

m  childhood,  852 

climatic,  851 

course  of,  853 

diagnosis  of,  853 

from  errors  in  swollen  glands,  848 

etiology  of,  838 

nicotine,  851 

prognosis   of,   854 

symptoms  of,  840 
cerebral,  840 
emotional,  842 


Neurasthenia,  symptoms  of,  gastric,  847 

intestinal,  847 

mental,  840 

sexual,  850 

spinal,   843 

vasomotor,  844 
syphilitic,   851 
traumatic,  844 
treatment,  854 

climatic,  858 

hydrotherapeutic,  857 

mental,  855 

physical,  856 

thyroid,  849 
Neuritis,  166 

alcoholic,  212 

mental  state  in,  218 
anaesthesia  in,  41 
apoplectic,  176 
arsenical,  224 
brachial,   176 
cancer  and,  256 
carbon-monoxide,  222 
eervico-brachial,  174 
diabetic,  253 
diagnosis  of,  46 
diphtheritic,  235 
electrical  changes  in,  44 
eruptions  after,  42 
gangrene  after,  43 
glossy  skin  in,  43 
gouty,  251 
after  grippe,   239 
hair  in,  43 
infectious,  240 
interstitial,  168' 
leprous,  242 
mercurial,  234 
migratory,  168 
multiple,  197 

course  of,  204 
diagnosis  of,   205 
duration  of,  204 
etiology  of,  197,  234,  256 
prognosis  of,  207 
symptoms  of,  198 
treatment  of,  207 
varieties  of,  197 
optic,  29 
pain  in,  40 
paralysis  in,  44 
parenchymatous^  168 


964 


INDEX. 


Neuritis,  paraesthesia  in,  39 

pathology  of,  167 

periaxillary,  169 

phosphorus,  234 

prognosis  of,  170 

rheumatic,  250 

senile,  258 

of  shoulder  joint,  183 

spasms  in,  46 

special  forms  of,  174 

symptoms  of,  170 

syphilitic,   255 

talipes  in,  202 

treatment  of,  170 

tuberculosis  and,  254 

varieties  of,   166 

vasomotor  symptoms  in,  43 
Neuro-fibrils,  25 
Neuroma,   193 
Neurones,  22,  53 

classes  of,  22,  28,  31 
central,  22 
peripheral,  28 

effect  of  work  on,  32 

function   of,  31 

heteromere,   72 

nutrition  of,  32 

pathology  of,  32,  33 

tautomere,  72 
Neuronophages,  399 
Neuroses,  trophic,  942 
Ninth  nerve,  667 
Nissl  bodies,  22 
Nodding  spasm,  786 
Nodes  of  Eanvier,  37 
Noguchi  test,  696 
Nuclei  of  ocular  nerves,  637 
Nucleus  ambiguus,  116 

amygdalus,  133 

cuneatus,  115 

gracilis,  115 
Numbness.     See  Parajsthesia,  39 
Nutrition   of  bones,   943 
Nystagmus  in   infants,    786 

OBSTETEICAL  paralysis,  179 
Occipital  cortex,  120 

lobe,   tumors    of,    593 
Occupation  neuroses,  859 
Ocular   palsy,   recurrent,   645 
rheumatic,  644 
vertigo,   900 


Oculo-motor  nerves,  636 

palsy,  636,  690 
CEdema,  angio-neuritic,  938 

of  optic  nerve,  630 
OEsophagus,  spasm  of,  671 
Olfactory  bulb,  624 

nerve,  623 
Operation  in  fractured  spine,  431 
Ophthalmic  migraine,  645 
Ophthalmoplegia  externa,  645 

interna,  645 
Oppenheim's  disease,  766 

reflex,  62 
Optic  decussation,  627 
nerve,  625 

anaemia  of,  628 
atrophy  of,  634 
hyperaemia  of,  628 
oedema  of,  630 
neuritis,   89,   631 
thalamus,  anatomy  of,  146 
symptoms  of,  146 
tumors  of,  596 
Optical  aphasia,  138,  560 
Organ  of  Corti,  662 
Organic  dementia,  491 

vs.  functional  disease,  18 
Osteoporosis,  943 

PACHYMENINGITIS  cerviealis,  450 
in  lumbar  region,  451 
externa,  705 
interna,  765 

etiology  of,  705 
pathology  of,  705 
symptoms  of,  706 
treatment  of,   707 
Pain  in  neuritis,  40 
referred,  751 
sense,  tract  of,  115 
in  spinal  cord  disease,  78 
Painful  knee,  744 
shoulder,  153 
Palmar  reflex,  62 
Palsy,  birth,  179 

ocular,  recurrent,  645 

rheumatic,  644 
oculo-motor,  636,  691 
shaking,  806 
Paraesthesia,    39 

in  brain  disease,  95,  106 
m  neuritis,  39 


INDEX. 


965 


Paraesthesia  Jn  spinal  cord  disease,  67 
Paralysis  agitans,  806 
course  of,  810 
diagnosis  of,  810 
etiology  of,  806 
gait  in,  808 
posture  in,  809 
symptoms  of,  807 
theories  of,  811 
treatment   of,  811 
alternating,  105 
Aran-Duchenne 's,  285,  288 
in  brain  disease,  99,  103 
bulbar,  614.    See  also  Cauda  equina 

lesions,   76 
of  cervical  sympathetic  nerve,  438 
chronic  atrophic,  284 

diagnosis  of,  291 
pathology  of,  285 
prognosis  of,  291 
symptoms  of,  287 
treatment  of,  294 
varieties  of,  284 
diphtheritic,  235 
Duchenne's,  179  , 

ascending,  285,  28'8 
Erb's,  179 
facial,  656 

glosso-labio-laryngeal,  616 
hysterical,  882 
ischsemic,  195 
intermittent,  774 
Landry's,  454 
in  locomotor  ataxia,  371 
in  neuritis,  44 
obstetrical,  179 
of  ocular  nerves,   636,  644 
periodic,   774 
in  spinal  cord  disease,  48 
of  sterno-mastoid  muscle,  671 
of  tongue,  672 
of  third  nerve,  638 
of  trapezius  muscle,  671 
varieties  of,  48,  58,  59 
Paraphasia,  139 
Paraplegia,  Erb's,  693 
senile,  415 
syphilitic,  401 
Paramyoclonus  multifilex,  799 
diagnosis  of,  801 
symptoms  of,  799 
treatment  of,  801 


Parasyphilitic  diseases,  695 
Parenchymatous  neuritis,  168 
Paresis,  540 

course  of,  546 

delusions  in,  543 

dementia  in,  543 

depression   in,   543 

diagnosis   of,  546 

epilepsy  in,  545 

etiology  of,  540 

excitement  in,   543 

mental  states  in,  543 

pathology  of,  541 

prognosis  of,  547 

symptoms  of,  542 
in  motor,  545 

treatment  of,  548 

salvarsan  in,  548 

types  of,  543 
Parietal  lobe,  tumors  of,  593 
Parkinson's  disease,  806 
Patella   reflex,   62 
Pathetic  nerve,  642 
Pellagra,   698 

course  of,  700 

etiology  of,  698 

history  of,  698 

prognosis   of,   700 

symptoms  of    698 

treatment  of,  700 
Perforating  ulcers,  367 

of  foot,  952 
Periaxillary  neuritis,  169 
Perineurium,  39 
Periodic  paralysis,  774 
Pernicious  anaemia,  392 
Peroneal  muscular  atrophy,  316 
Petit  mal,  813 
Phobias,  842 
Phosphorus  neuritis,  234 
Pigmentation  of  skin,  944 
Plantar  nerves,  192 

reflex,  62 
Plumbism,  230 
Pneumogastrie  nerve,  668 
Polio-encephalitis  of  Wernicke,  538 
Poliomyelitis,  262 

chronic  form  of,  285 

course  of,  274 

deformities  in,  274 

diagnosis  of,  276 

epidemics,  63,  276 


966 


INDEX. 


Poliomyelitis,  etiology  of,  262 

pathology  of,  264 

prognosis  of,  278 

spinal  fluid  in,  268 

symptoms  of,  268 

treatment  of,  279 

types  of  paralysis,  272 
Polyneuritic  psychosis,  218 
Polyuria,  91 
Pons  Varolii,  148 

lesions  of,  anaesthesia  in,  117 

symptoms  of,  150 
tumors  of,  598 
Porencephalitis,  517 
Posterior  sclerosis,  342 

thoracic  nerve,   184 
Procursive  epilepsy,  813 
Professional  cramps,  859 
Protopathic  sensations,  42 
Pseudo-bulbar  paralysis,  620 
Pseudo-muscular  hypertrophy,  307 
Pseudo-sclerosis,  684 
Psychasthenia,  863 

course  of,  876 

etiology  of,  863 

hypnosis,  874 

persuasive,  875 

suggestion  in,  874 

symptoms  of,  864 

double  consciousness,  868 
instability  of  conduct,  867 
morbid  fears,  864 
ideas,  866 
impulses,  865 
psychic  pain,   867 

treatment  of,  876 
Psychical  blindness,  122 

deafness,  126 

epilepsy,  817 

pain,  867 

vertigo,  902 
Psychosis,  Korsakoff's,  218 

polyneuritic,  218 
Pulse  in  brain  disease,  90 
Pupillary  mechanism,  640 

reflex,  61,  627 


Q 


UINCKE'S  puncture,  155 


RANVIEE,  nodes  of,  37 
Raynaud's  disease,  932 
Reaction  of  degeneration,  44 


Rectal  mechanism,  64 
Recurrent  ocular  paralysis    645 
Referred   pains,   751 
Reflex  action,  60 

of  bladder  and  rectum,  67 

epilepsy,  825 

neuroses,   909 
Reflexes,  abdominal,  62 

Babinski's,  62 

exaggeration  of,  63 

genital,  62 

Gordon's,   62 

loss  of,  62 

Mendel's,  62 

muscular,  61 

Oppenheim's,  62 

palmar^  62 

patellar,  62 

plantar,  62 

pupillary,  61,  627 

scapular,  61 

skin,   64 

spinal,  61 

tendon,  61 

loss   of,    62 

triceps,  61 
Regeneration  of  nerves,  163 
Relation  of  subconscious  ideas,  371 

of  vertebrjB  to  spinal  segments,  420 
Respiration  in  brain  disease,  90 

Cheyne-Stokes,    670 
Respiratory  bundle  of  Krause,  668 
Rest  cure,  856,  893 
Retinal  artery,  diseases  of,  629 
embolism   of,   628 
thrombosis   of,   629 
Rheumatic  neuritis,  250 

ocular  palsy,  644 

SACRAL  plexus,  190 
Salvarsan,  696 

in  cerebrospinal  syphilis,  682 
in  locomotor  ataxia,  378 
in  paresis,  348 

Sarcoma   of  vertebrae,  437 

Scapular  reflex,  61 

Scarpa,  ganglion  of,  665 

Scheme   of  examination,   20 

Schmitt-Lantermann,   incision   of,    38 

Schultze,  columns  of,  70 

Schwann,  sheath  of,  38' 

Sciatic  nerve,  190 


INDEX. 


967 


Sciatic  neur^gia,  744 
Sciatica,  744 

Sclerosis,  amytrophic  lateral,  294 
course  of,  300 
diagnosis  of,  301 
duration   of,  300 
etiology  of,  296 
pathology  of,  295 
symptoms  of,  297 
treatment  of,  302 
arterial,  468    474 

pathology  of,  468 
symptoms  of,  469 
of  brain,  516 

combined,  diagnosis  of,  387 
etiology  of,  386 
history  of,  383 
pathology  of,  384 
symptoms  of,  386 
disseminated,  675 
insular,   675 
lateral,  337 

diagnosis  of,  341 
etiology  of,  338 
history  of,  337 
pathology  of,  339 
symptoms  of^  339 
talipes  in,  340 
treatment  of,  341 
multiple,  675 

diagnosis  of,  684 
etiology  of,  679 
frequency  of,  682 
pathology  of,  675 
prognosis  of,  684 
symptoms  of,  680 
treatment  of,  685 
Secondary  degeneration  in  spinal  cord, 

402 
Semilunar  canals,  665,  667 
Senile  dementia,  470 
neuritis,  258 
paraplegia,  415 
Sensation,  test  of,  108 
Sense  of  hearing,  662 
of  smell,  624 

anatomy  of,  133 
disturbance  of,  133 
hallucinations  of,  133 
of  taste,  133,  651,  668 
Sensory  area   of   cortex,   106 
impulse,  effects  of,  113 


Sensory  tracts,  114,  116 
in  brain,  112 
in  cerebral  axis,  114 
in  spinal  cord,  68 
Seventh  nerve,  655 
Shaking  palsy,  806 
Sheath  of  Mauthner,  38 

of  Schwann,  38 
Sherrington  '^  motor  areas,  92 
Silver  poisoning,  234 
Sixth  nerve,  642 
Skin,  glossy,  943 

hypertrophy  of,  946 
pigmentation  of,  944 
Skin  reflexes,  64 
Sleep,  disorders  of,  903 
Smell,  sense  of,  133,  624 

solar  plexus,  34 
Spasm  of  arm,  93 
of  arteries,  926 
of  face,  93 
hysterical,  881 
of  leg,  95 
in  neuritis,  46 
nodding,   786 
of  oesophagus,  671 
of  trunk,  95 
of  veins,  926 
vs.  tic,  778 
Spasmodic  torticollis,  794,  799 
Spinal  accessory  nerve,  671 
ankylosis,  452 
bloodvessels,  259 
cord,  anaemia  of,  392 
anatomy  of,  47 

aneurism    of    aorta    compress- 
ing, 449 
bloodvessels  of,  259 
changes  in  pernicious  anaemia, 

392 
compression  of,  433 
degeneration   of,  secondary,  402 
disease  of,  47 

paralysis  in,  48 

position    of    bed    in,    66, 

421 
symptoms  of,  48,  65 

anaesthesia  in,  67,  74, 

442 
ataxia  in,  77 
gait,  65 
pain  in,  67,  78 


968 


INDEX. 


Spinal  cord,  disease  of,   symptoms   of, 
parsBsthesia  in,  67 
paralysis,   48,   59 
reflexes,  61,  62 
spasm,   59 
trophic,  80 
vasomotor^    79 
division  of,  symptoms  of,  59 
functions  of,  47 
hyperaemia  of,  392 
injuries  of,  416 

diagnosis  of,  430 
etiology  of,  418 
pathology  of,  416 
prognosis  of,  430 
question    of    operation   in, 

431 
symptoms  of,     418 
treatment   of,  430 
lesions  of,  unilateral,  74 
motor  cells  of,  53 

functions  of,  55 
grouping  of,  54 
reflexes  of,  61,  64 
sensory  tracts  in,  68 
tumors  of,  440 

diagnosis  of,  446 
etiology  of,  444 
pathology  of,  440 
symptoms  of,  444 
treatment  of,  447 
epilepsy,  59 

fracture,  effects  of,  421 
hemorrhage,  261,  416 
irritation,  844 
meningitis,  acute,  457 

diagnosis  of,  460 
pathology  of,  457 
symptoms    of,    459 
treatment    of,   460 
chronic,  460 

diagnosis  of,  461 
pathology  of,  460 
symptoms  of,  461 
nerves,  47 
syphilis,  Erb's,  693 
Spine,  caries  of,  433 
Spondylose  rhizomelique,  452 
Sterno-mastoid    muscles,    paralysis    of, 

671 
Stereotyped  movements,  788 
Stomachic  vertigo,   901 


Stupor,  88 
Subconscious,  870 

Subcortical  lesions,  symptoms  of,  145 
Sulphide  of  carbon  poisoning,  223 
Suprascapular  nerve,  183 
Sydenham's  chorea,  778 
Symmetrical   gangrene,   932 
Sympathetic,  cervical  paralysis,  438 
ganglia,  34 

nervous  system,  34,  915 
Syphilis  of  nervous  system,  686 

apoplectic  attacks  in,  689 
diagnosis  of,  695 
endarteritis,  688 
frequency  of,  686 
pathology  of,  686 
symptoms  of,  689 
tests  for,  688,  695,  696 
treatment  of,  696 
various  forms  of,  687    ■ 
Syphilitic  endarteritis,  688 
exudations,  689 

cranial  nerve  palsy  due  to,  691 
effects  of,  690 
symptoms  of,  691 
cerebral,  692 
spinal,  693 
about  vertebrae,  439 
hyperplasia,  694 
diffuse,  694 
local,  695 
neurasthenia,   851 
neuritis,  255 
paraplegia,  401 
Syringomyelia,  320 
ana?sthesia  in,  75 
arthropathy  in,  331 
diagnosis  of,  335 
pathology  of,  320 
symptoms  of,  327 
theories  of  origin  of,  323 
treatment  of,  336 

TABES.     See  Locomotor  ataxia,  342 
dorsale  spasmodique.     See  Lateral 
sclerosis,   337 
talipes  in,  367 
Taehea  cerebrales,  927 
Tactile  sense,  tract  of,  115 
Talipes  in  lateral  sclerosis,  340 
in  neuritis,  202 
in  tabes.  367 


INDEX. 


969 


Taste,  sense  of,  133,  651,  668 
Tautomere  n^rones,  72 
Tegmentum  cruris  cerebri,  148 
Temperature  in  brain  disease,  90 

sense,  tract  of,  16 
Temporal  lobe,  126-133 

tumors  of,  593,  594 
Tendon  reflexes,  61 

in  apoplexy,  497 
exaggeration  of,  66 
Tenth  nerve,  668 
Terminals  of  neurones,  30 
T'jsticles,  neuralgia   of,  749 
Tests  of  muscular  sense,  109 

of  sensation,  108 
Tetany,  804 
Thermo-ansesthesia,  73 
Third  nerve,  638 

paralysis,  638 
Thomson's  disease,  802 
Thought    mechanism  of,  134 
Thrombosis,  cerebral,  476 

of  retinal  artery,  628 

of  venous  sinuses,  567 
Tic,  788 

de  salaam,  813 

douloureux,    733 

vs.   spasm,   788 
Tigroid,  22 
Tinnitus  aurium,  664 
Tongue,  hemiatrophy  of,  673 

nerves  of,  672 

paralysis  of,  672 
Torticollis,  672^  794 

spasmodic,  794 
Tortipelvis,  799 
Tract  of  muscular  sense,  113,  114,  115 

of  sensations  of  pain,  115 

of  sensations  of  touch,  115 
Trapezius  muscle,  paralysis  of,  671 
Traumatic  neurasthenia,  844 
Tremor,  812 
Triceps  reflex,  61 
Trigeminal  nerve,  649 

paralysis  of,  652 

neuralgia,  733 
Trismus,   652 
Trophic  disturbances,  theory  of,  79 

nerves,  43,  942 

neuroses,  942 

symptom,  42 

origin  of,  42 


Trophcedema,  945 
Trunk,  spasms  of,  95 
Tuberculosis  and  neuritis,  254 
Tumors    of    brain,    571.       See    Brain, 
tumors    of 
of  nerves,  193 

of    spinal    cord,   440.      See    Spinal 
Cord,  tumors  of 
Twelfth  nerve,  672 

ULCERATIONS    of    nervous    origin, 
951 
Ulnar  nerve,  187 
Uncinate  gyrus,  133 
Urine,   suppression   of,  hysterical,   886 

YAQAL  attacks,  601 
Vagus  nerve,  668 
Vascular  tone,  916 
Vaso-constrictors,  918 
Vaso-dilators,  919 
Vasomotor  diseases,  915 

nerves,   920 

reflexes,   922 

spasm,  925 

symptoms   in   neuritis,  43 

system^  916 

tracts,  923 
Veins,  spasm  of,  926 
Venous  sinuses  of  brain,  565 

thrombosis  of,  567 
VertebrfB,  carcinoma  of,  437 

fracture  of,  effects  of,  421 

sarcoma  of,  437 

syphilitic  exudation  about,  439 
Vertige  paralysant,  900 
Vertigo,   86,   398,  666 

ana3mic,  901 

auditory,  898 

cerebellar,  900 

due  to  intracranial  pressure,  901 

essential,   900 

laryngeal,  902 

muscular  sense,  900 

nasal,  902 

ocular,  900 

pontine,  899 

psychic,  902 

stomachic,  901 

toxic,   901 

vestibular,  899 
Vestibular  norvc,  665 


970 


INDEX. 


Vestibular  vertigo,  899 
Vibratory  sense,  364 
Vision^  disturbance  of,  120 
Visual  amnesia,  137 

aura^  124 

tract,  119 
Voice,  changes  in,  670 
Voltalini's  disease,  899 
Vomiting,  88 

hysterical,  886 

WASSEEMANN  test,  695 
Weber's  syndrome,  105 
Wernicke's    hemiopic    pupillary    reflex, 
628 


Wernicke's  polio-encephalitis,  538 
Word  blindness,  123,  137 

deafness,  126,  136 
Workj  its  effect  on  neurones,  32 
Wrisberg,  nerve  of,  656 
Writer's  cramp,  860 
Wryneck,  672,  794 


X 
Y 


ExlY    examination    in    tumor    of 
brain,   605 

A^^ING.   790 


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